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Kubo E, Haruhara K, Marumoto H, Sasaki T, Okabe M, Yokote S, Shimizu A, Ueda H, Tsuboi N, Yokoo T. Tonsillectomy in Immunoglobulin A vasculitis with nephritis: case series. CEN Case Rep 2025; 14:381-389. [PMID: 39961993 DOI: 10.1007/s13730-025-00975-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2024] [Accepted: 01/20/2025] [Indexed: 06/02/2025] Open
Abstract
There is no consensus-based treatment for adult-onset immunoglobulin A vasculitis with nephritis (IgAV nephritis). Tonsillectomy is a treatment option for primary IgA nephropathy, which has similar histopathological features and pathogenesis to IgAV nephritis. The present case series aimed to describe the clinical course of patients with IgAV nephritis who underwent tonsillectomy in our institution. Adult patients with biopsy-proven IgAV nephritis who received tonsillectomy from 2015 to 2022 were systematically reviewed at six hospitals in Japan. Hematuria, proteinuria and slope of the estimated glomerular filtration rate (eGFR) were evaluated before and after tonsillectomy. Patients with IgAV nephritis who underwent tonsillectomy was identified in 12 of 2626 kidney biopsies performed during the study period. The median observation periods before and after tonsillectomy were 20.7 and 48.6 months, respectively. The following drugs were administered concurrent with tonsillectomy: corticosteroids (n = 8), mizoribin (n = 1), and rituximab (n = 1). Three patients were not treated with corticosteroids or immunosuppressants. During post-tonsillectomy observation, 5 patients showed remission of hematuria. Of the 10 patients whose proteinuria was not at a remission level prior to tonsillectomy, 7 showed remission of proteinuria after tonsillectomy. The eGFR slope was attenuated in 9 patients after tonsillectomy relative to before tonsillectomy. This study suggests that some patients may benefit from tonsillectomy in the treatment of IgAV nephritis. The efficacy of tonsillectomy or combination therapy with immunosuppression for IgAV nephritis requires further case series to clarify the clinicopathologic picture of patients associated with a response to tonsillectomy.
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Affiliation(s)
- Eisuke Kubo
- Department of Internal Medicine, Division of Nephrology and Hypertension, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Kotaro Haruhara
- Department of Internal Medicine, Division of Nephrology and Hypertension, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan.
| | - Hirokazu Marumoto
- Department of Internal Medicine, Division of Nephrology and Hypertension, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Takaya Sasaki
- Department of Internal Medicine, Division of Nephrology and Hypertension, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Masahiro Okabe
- Department of Internal Medicine, Division of Nephrology and Hypertension, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Shinya Yokote
- Department of Internal Medicine, Division of Nephrology and Hypertension, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Akihiro Shimizu
- Department of Internal Medicine, Division of Nephrology and Hypertension, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Hiroyuki Ueda
- Department of Internal Medicine, Division of Nephrology and Hypertension, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Nobuo Tsuboi
- Department of Internal Medicine, Division of Nephrology and Hypertension, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan.
| | - Takashi Yokoo
- Department of Internal Medicine, Division of Nephrology and Hypertension, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
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Guo Z, Li S, Liu C, Zhu Z, Wang P, Yang Y, Du L. Immunoglobulin a vasculitis with central nervous system involvement: analysis of 10 cases. Clin Exp Med 2025; 25:145. [PMID: 40346320 PMCID: PMC12064608 DOI: 10.1007/s10238-025-01679-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2025] [Accepted: 04/09/2025] [Indexed: 05/11/2025]
Abstract
Immunoglobulin A vasculitis (IgAV) is a systemic inflammatory disease that affects small blood vessels. Central nervous system (CNS) involvement in IgAV is rare. This study analyzed the clinical characteristics of IgAV patients combined with CNS damage in children. Furthermore, the study made a comparison between the characteristics of IgAV patients with and without CNS damage, and initially explored the potential predictors for IgAV patients with CNS damage. A retrospective analysis was conducted on a cohort of 50 children diagnosed with IgAV and admitted to Beijing Children's Hospital from 2016 to 2019. The study encompassed a review of the clinical presentations, laboratory test results, imaging findings, therapeutic interventions, and prognoses of 10 children with IgAV who exhibited CNS involvement. These 10 cases were then compared with a group of 40 children with IgAV without CNS involvement. The prevalence of IgAV with CNS manifestations was 0.2%. The median age was 11.6 years, with a male-to-female ratio of 7:3. All CNS symptoms appeared after the purpuric rash. The mean period from IgAV onset to the development of neurological symptoms was 12.2 days (range: 1-27 days). Seizures were the most common neurological manifestation, with impaired consciousness and predominant convulsions. Other symptoms included headache, visual impairment, dysarthria, dyskinesia, and emotional irritation. The main abnormalities found on brain magnetic resonance imaging (MRI) were unilateral or bilateral abnormal focal signals, cortical and subcortical white matter edema, and thrombosis of the venous sinus. Glucocorticoid therapy and intravenous immunoglobulins were used to treat CNS damage caused by IgAV. All patients showed clinical improvement without recurrent neurological symptoms or sequelae. Statistically differences were identified in in terms of age, gastrointestinal damage, WBC count, NLR, ALB, C3 levels, and the CD4/CD8 ratio in IgAV patients with CNS damage when compared to those without CNS damage. Multivariable logistic regression analysis shows that age, NLR and C3 Levels are predictors of IgAV with CNS damage. CNS involvement in IgAV is a rare complication. Its clinical manifestations are diverse and vary in severity, and its diagnosis is exclusionary. Brain MRI is beneficial for diagnosis and follow-up. Steroid therapy is important for treating IgAV-associated CNS involvement. Age, NLR and C3 Levels are predictors of IgAV with CNS damage.
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Affiliation(s)
- Ziyun Guo
- Department of Chinese Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China
| | - Shaojing Li
- Department of Pediatrics, China Aerospace Science & Industry Corporation 731 Hospital, Beijing, 100074, China
| | - Chang Liu
- Department of Chinese Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China
| | - Zhongyi Zhu
- Department of Chinese Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China
| | - Panpan Wang
- Department of Pediatrics, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, 310007, China
| | - Yan Yang
- Department of Chinese Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China.
| | - Lina Du
- Department of Chinese Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China.
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Konstantopoulou A, Tsoliakos I, Berikopoulou MM, Kiorsavva A, Theochari M, Drosatou P, Messaritaki A, Dimopoulou D. A case-based review of IgA vasculitis complicated with gastrointestinal infections: insights from a norovirus-associated case in an adolescent. Rheumatol Int 2025; 45:114. [PMID: 40261399 DOI: 10.1007/s00296-025-05876-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2025] [Accepted: 04/10/2025] [Indexed: 04/24/2025]
Abstract
Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is a self-limited leukocytoclastic vasculitis targeting small-sized vessels. It is the most common vasculitis in children, but also targets adults who have usually worse prognosis. It typically presents with purpuric rash, arthritis, colicky abdominal pain and potential renal involvement. Gastrointestinal (GI) manifestations, although frequent, may be severe and occasionally complicated by infections. We present a 11-year-old male with a recent diagnosis of IgAV who was admitted with severe abdominal pain, hematemesis and hematochezia. Stool analysis identified Norovirus as the causative pathogen for the severe gastrointestinal symptoms. Treatment with proton pump inhibitors, high-dose corticosteroids and intravenous fluids resulted in symptoms resolution and clinical improvement. In addition, a case-based review of the literature was conducted to evaluate the prevalence of GI infections occurring after the development of IgAV that may result in severe complications or disease relapses. Thirty-five patients with a median age of 14 years were included in our study. Among these patients, 42.9% were diagnosed with a concomitant bacterial gastrointestinal pathogen, followed by 25.7% with viral gastroenteritis and 17.1% with parasitic gastrointestinal disease. Treatment was individualized, with 48.5% receiving corticosteroids and 8.6% receiving immunosuppressive therapy. This case and case-based review highlights the significance of careful management and monitoring of patients with IgAV complicated by infections, because severe complications can cause significant morbidity and mortality especially in adults.
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Affiliation(s)
- Argyro Konstantopoulou
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Ioannis Tsoliakos
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Maria M Berikopoulou
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Aikaterini Kiorsavva
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Maria Theochari
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Panagiota Drosatou
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Anna Messaritaki
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Dimitra Dimopoulou
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece.
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Kambara S, Nishio N, Sugiyama Y, Nishio Y, Takamoto Y, Kitai F, Takahashi Y, Hayashi N, Haruta K, Kondo M, Oike N, Miwa T, Watanabe N, Omori M, Kinoshita F, Furukawa T, Kawada JI, Kidokoro H, Sato Y, Takahashi Y, Nagoya Collaborative Clinical Research Team. Early discontinuation of steroid treatment in children with abdominal pain due to IgA vasculitis. Eur J Pediatr 2025; 184:279. [PMID: 40183803 PMCID: PMC11971167 DOI: 10.1007/s00431-025-06107-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Collaborators] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/06/2025] [Revised: 03/08/2025] [Accepted: 03/23/2025] [Indexed: 04/05/2025]
Abstract
This study aims to evaluate the impact of early steroid discontinuation on total dosage and outcomes in pediatric immunoglobulin A (IgA) vasculitis patients with uncontrolled abdominal pain. This retrospective cohort study included children younger than 16 years with newly diagnosed IgA vasculitis hospitalized for abdominal pain who received their first dose of steroids between April 1, 2013, and March 31, 2019, at 14 hospitals. Patients were divided into two groups: the standard (STD) group, which received steroid therapy for at least 8 consecutive days, and the early discontinuation attempt (EDA) group, which attempted discontinuation within 7 days. EDA was further divided into two subgroups: the early discontinuation (ED) group, which completed steroid treatment within a week, and the readministration (RA) group, which required readministration. Total steroid dosage, duration of therapy, hospital stay, and complications were compared. A total of 272 patients were analyzed: STD (n = 190) and EDA (n = 82). There were no significant differences in baseline characteristics. EDA had a shorter hospital stay (8.5 vs. 15.0 days, p < 0.01), fewer total steroid days (6 vs. 17.5 days, p < 0.01), and lower total steroid dosage (5.4 mg/kg vs. 15.4 mg/kg, p < 0.01) compared to STD, with no significant differences in complications. Among EDA patients, 22 (27%) required steroid readministration due to symptom recurrence; however, symptoms resolved in all RA patients, with lower total steroid dosage and duration compared to STD, without prolonging hospital stay. Conclusion: Discontinuing steroids within 7 days for abdominal pain in children with IgA vasculitis reduces total steroid dosage without increasing complications, even with occasional readministration. Clinical trial registration: Approval no. 2019-0394.
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Affiliation(s)
- Sumika Kambara
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Nobuhiro Nishio
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.
- Center for Advanced Medicine and Clinical Research, Department of Advanced Medicine, Nagoya University Hospital, 65 Tsurumai-Cho, Showa Ward, Nagoya City, Aichi Prefecture, 466-8560, Japan.
| | - Yuichiro Sugiyama
- Department of Pediatrics, Japanese Red Cross Aichi Medical Center Nagoya Daiichi Hospital, Nagoya, Japan
| | - Yosuke Nishio
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Yukina Takamoto
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Fumie Kitai
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Yuma Takahashi
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Nozomi Hayashi
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Kazunori Haruta
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Maki Kondo
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Naoko Oike
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Takeshi Miwa
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | | | - Marei Omori
- Department of Pediatrics, Hekinan Municipal Hospital, Hekinan, Aichi, Japan
| | - Fumie Kinoshita
- Center for Advanced Medicine and Clinical Research, Department of Advanced Medicine, Nagoya University Hospital, 65 Tsurumai-Cho, Showa Ward, Nagoya City, Aichi Prefecture, 466-8560, Japan
- Data Coordinating Center, Department of Advanced Medicine, Nagoya University Hospital, Nagoya, Japan
| | - Taiki Furukawa
- Medical IT Center, Nagoya University Hospital, Nagoya, Japan
| | - Jun-Ichi Kawada
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Hiroyuki Kidokoro
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Yoshiaki Sato
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
- Division of Neonatology, Center for Maternal-Neonatal Care, Nagoya University Hospital, Nagoya, Japan
| | - Yoshiyuki Takahashi
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
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Collaborators
Kazuto Ueda, Makoto Oshiro, Atsushi Tashiro, Kazuki Yamamori, Motohiro Shibata, Shinji Hasegawa, Naoko Nishimura, Masashi Morishita, Michio Suzuki, Tetsuo Kubota, Noriko Nagai, Osamu Shinohara, Satoru Doi, Mitsuharu Kajita, Shinya Hara, Takashi Kawabe, Shin Hoshino,
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Froberg PW, Ruml A, Fernandez JK. Henoch-Schönlein Purpura Without Proven Immunoglobin A Deposition: A Diagnostic Distinction. Cureus 2025; 17:e82312. [PMID: 40376363 PMCID: PMC12080952 DOI: 10.7759/cureus.82312] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/14/2025] [Indexed: 05/18/2025] Open
Abstract
Henoch-Schönlein purpura (HSP), or immunoglobulin A (IgA) vasculitis (IgAV), is a small vessel vasculitis that is most commonly seen in children; it is classically characterized by IgA deposition within the renal mesangium, resulting in a wide range of symptoms: palpable purpura, arthralgia, gastrointestinal symptoms, renal involvement, and, in severe cases, pulmonary complications or intussusception. Diagnosis relies upon clinical symptoms, histopathology, and direct immunofluorescence (DIF) testing to differentiate HSP from other vasculitides. DIF typically reveals IgA deposits; however, negative DIF findings do not rule out the diagnosis, which indicates the need for an adaptable diagnostic approach. Groups such as the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) categorize positive IgA as supportive data for an HSP diagnosis but not as a necessity. We present the case of a 14-year-old male with progressive ascending palpable purpura, significant abdominal pain, and lower extremity edema. Histopathological analysis of the skin biopsy confirmed leukocytoclastic vasculitis (LCV); however, his DIF was negative for IgG, IgA, IgM, C3, and fibrinogen deposits. At the time of biopsy, his workup was significant for an isolated elevated alanine aminotransferase (ALT), but urinalysis and renal function testing were unremarkable. Despite a negative DIF, the patient's findings were clinically consistent with HSP, and hence, a diagnosis was made. He was started on a prednisone taper and supportive care for management. After initial improvement, he experienced a flare warranting an additional course of steroids, which improved his symptoms. This report underscores the diagnostic challenges associated with HSP, particularly with negative DIF. While DIF serves as a useful tool in the classification of certain vasculitides, its sensitivity is influenced by the biopsy site, lesion age, and degradation of immune complexes. Providers should maintain a high index of suspicion and carefully consider histopathologic findings and laboratory data when diagnosing HSP. Further research is needed to refine the diagnostic applicability of DIF and its association with disease severity and systemic involvement.
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Yan L, Shen J, Liu L, Yang M, Wang S. IgA vasculitis induced by tumor necrosis factor-α antagonists: clinical features, diagnosis and management. Arch Dermatol Res 2025; 317:445. [PMID: 39976811 DOI: 10.1007/s00403-025-03965-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2024] [Revised: 01/22/2025] [Accepted: 02/03/2025] [Indexed: 05/10/2025]
Abstract
INTRODUCTION Anti-TNF therapies are commonly employed in the treatment of autoimmune disorders, yet they are associated with a rare side effect known as IgA vasculitis (IgAV), whose clinical presentation remains poorly understood. This study aims to clarify the features of IgAV linked to anti-TNF treatments to aid in prompt recognition and management. METHODS Case reports on TNF-α-antagonist-associated IgAV dated up to February 29, 2024, were retrieved for retrospective analysis. RESULTS A total of 35 cases from 30 publications were identified. The average age of patients was 36 years (range 11 to 69), with 31.4% being pediatric cases. The primary conditions treated were Crohn's disease (45.7%) and ulcerative colitis (22.9%). Infliximab (42.9%) and adalimumab (37.1%) were the most frequently used agents. The onset of IgAV after initiating anti-TNF therapy occurred at a median of 10 months (range 1 day to 11 years). Clinical symptoms predominantly involved the skin (97.1%), kidneys (68.6%), joints (57.1%), and gastrointestinal tract (40.0%). Renal failure developed in 11.4% of patients. Histopathology revealed leukocytoclastic vasculitis in the skin and mainly proliferative nephritis in renal biopsies, with significant IgA deposition observed. Most patients (80.0%) ceased anti-TNF treatment, and the majority received corticosteroids (96.2%) and dapsone (15.4%) as part of their treatment. Remission was achieved in 34 patients, while one patient worsened. Among the 14 patients who restarted anti-TNF therapy, 9 experienced a recurrence of IgAV. CONCLUSION IgAV associated with anti-TNF therapy may emerge months into treatment and can lead to severe renal complications necessitating ongoing surveillance. Halting anti-TNF therapy is imperative, but the decision to resume treatment must be weighed carefully against the risk of primary disease exacerbation and IgAV recurrence.
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Affiliation(s)
- Lu Yan
- Department of Pediatrics, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China
- Hunan Clinical Research Center of Pediatric Cancer, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China
| | - Jie Shen
- Department of Pediatrics, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China
- Hunan Clinical Research Center of Pediatric Cancer, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China
| | - Lin Liu
- Department of Pediatrics, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China
- Hunan Clinical Research Center of Pediatric Cancer, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China
| | - Minghua Yang
- Department of Pediatrics, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China.
- Hunan Clinical Research Center of Pediatric Cancer, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China.
| | - Shengfeng Wang
- Department of Pediatrics, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China.
- Department of Pharmacy, The Third Xiangya Hospital, Central South University, Changsha, Hunan, 410013, China.
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Oprițescu S, Nițescu GV, Golumbeanu M, Boghițoiu D, Ioniță EI, Ușurelu DA, Lucaci C, Negoiță A, Moroșan E. The Impact of Infectious Diseases on Clinical Characteristics and Immunological Correlations in Pediatric Henoch-Schönlein Purpura: A Five-Year Retrospective Study. Biomedicines 2025; 13:113. [PMID: 39857697 PMCID: PMC11762163 DOI: 10.3390/biomedicines13010113] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2024] [Revised: 12/31/2024] [Accepted: 01/04/2025] [Indexed: 01/27/2025] Open
Abstract
Background/Objectives: Immunoglobulin A (IgA) vasculitis (IgAV), classically known as Henoch-Schönlein purpura (HSP), is a type of nonthrombocytopenic small-vessel vasculitis. HSP is the most frequent kind of systemic vasculitis in children, characterized by purpura, arthritis or arthralgia, gastrointestinal pain, and kidney dysfunction. The aim of our research was to investigate and observe the clinical characteristics of children diagnosed with HSP and to explore the correlation between infectious diseases and HSP. Furthermore, this retrospective study considered other factors, such as demographic characteristics (sex, area/environment, and age), and their effect on the pediatric population diagnosed with HSP. Methods: To answer this question, we conducted a five-year hospital-based retrospective study that included 144 hospitalized children who were diagnosed with HSP during hospitalization. Measurements of immunological panels (IgA, IgM, IgG, and IgE), C3, C4, C-reactive protein, fibrinogen, and hematite sedimentation rate (VSH) determined using blood samples revealed that there is a strong correlation between the elements of the immunological panel and the HSP manifestations. Results: Additionally, elevated IgG and normal IgA serum levels in pediatric HSP patients are strongly associated with infectious diseases. Conclusions: Notably, patients with infectious diseases exhibited high IgG and normal IgA serum levels post-treatment and a higher risk of relapses.
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Affiliation(s)
- Sînziana Oprițescu
- Discipline of Clinical Laboratory and Food Safety, Faculty of Pharmacy, “Carol Davila” University of Medicine and Pharmacy, 6 Traian Vuia Street, 020945 Bucharest, Romania; (S.O.); (E.M.)
| | - Gabriela Viorela Nițescu
- Discipline of Pediatrics, Faculty of Dentistry, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
- “Grigore Alexandrescu” Clinical Emergency Hospital for Children, 017443 Bucharest, Romania
| | - Mihaela Golumbeanu
- “Grigore Alexandrescu” Clinical Emergency Hospital for Children, 017443 Bucharest, Romania
| | - Dora Boghițoiu
- Discipline of Pediatrics, Faculty of Dentistry, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
- “Grigore Alexandrescu” Clinical Emergency Hospital for Children, 017443 Bucharest, Romania
| | - Elena Iuliana Ioniță
- Discipline of Pharmacognosy, Phytochemistry and Phytotherapy, Faculty of Pharmacy, “Carol Davila”, University of Medicine and Pharmacy, 6 Traian Vuia Street, 020945 Bucharest, Romania
| | - Diana-Andreea Ușurelu
- Discipline of Pediatrics, Faculty of Dentistry, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
- “Grigore Alexandrescu” Clinical Emergency Hospital for Children, 017443 Bucharest, Romania
| | - Cristian Lucaci
- Discipline of Pediatrics, Faculty of Dentistry, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
- “Grigore Alexandrescu” Clinical Emergency Hospital for Children, 017443 Bucharest, Romania
| | - Adriana Negoiță
- Discipline of Pediatrics, Faculty of Dentistry, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
- “Grigore Alexandrescu” Clinical Emergency Hospital for Children, 017443 Bucharest, Romania
| | - Elena Moroșan
- Discipline of Clinical Laboratory and Food Safety, Faculty of Pharmacy, “Carol Davila” University of Medicine and Pharmacy, 6 Traian Vuia Street, 020945 Bucharest, Romania; (S.O.); (E.M.)
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Ebadinejad A, Silver E, O'Sullivan DM, Dar W, Morgan G, Emmanuel B, Cobar JP, Ye X, Singh JU, Kent R, Tremaglio J, Serrano OK. Outcomes in Patients With Henoch-Schönlein Purpura After Kidney Transplantation: A Propensity Score Matched Study. Nephrology (Carlton) 2025; 30:e14431. [PMID: 39821923 DOI: 10.1111/nep.14431] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/23/2024] [Revised: 11/22/2024] [Accepted: 01/01/2025] [Indexed: 01/19/2025]
Abstract
AIM Henoch-Schönlein purpura (HSP) nephritis leads to end-stage renal disease (ESRD) in upto 3% of cases, necessitating kidney transplantation (KT). This study compared graft and patient survival outcomes between HSP and non-HSP KT recipients and identified factors associated with HSP recurrence. METHODS Data from the Scientific Registry of Transplant Recipients (SRTR) were analysed for adult and paediatric KT patients listed between January 2005 and April 2021. HSP recipients were extracted from the database and non-HSP recipients were selected and propensity-matched 3:1 based on demographic factors. RESULTS A total of 371 KT recipients with HSP were matched to 1113 non-HSP recipients. When stratified by age, adult and paediatric HSP patients showed similar death-censored graft survival (DCGS) at 5 years compared to their non-HSP counterparts. Furthermore, paediatric patients with HSP had comparable DCGS to adult HSP patients at 5 years (86.5% vs. 88.3%, p = 0.221). Amongst HSP recipients, 27.2% experienced recurrence, with higher rates in adults (29.7%) compared to children (13.0%). Recurrent HSP was associated with increased graft failure and mortality. Regression analysis showed that older age (OR [95% CI]: 1.018 (1.001-1.035), p = 0.037) was associated with a higher risk of recurrence, while a higher BMI (0.95 [0.91-0.99], p = 0.020) was linked to a lower risk. CONCLUSION In a contemporary cohort of HSP KT patients, graft survival was comparable between HSP and matched non-HSP patients in both adult and paediatric groups. However, graft loss was more frequent in HSP patients who experienced disease recurrence.
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Affiliation(s)
- Amir Ebadinejad
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
| | - Elizabeth Silver
- Department of Surgery, University of Connecticut School of Medicine, Farmington, Connecticut, USA
| | - David M O'Sullivan
- Department of Research Administration, Hartford HealthCare, Hartford, Connecticut, USA
| | - Wasim Dar
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
- Department of Surgery, University of Connecticut School of Medicine, Farmington, Connecticut, USA
| | - Glyn Morgan
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
- Department of Surgery, University of Connecticut School of Medicine, Farmington, Connecticut, USA
| | - Bishoy Emmanuel
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
- Department of Surgery, University of Connecticut School of Medicine, Farmington, Connecticut, USA
| | - Juan P Cobar
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
| | - Xiaoyi Ye
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
| | - Joseph U Singh
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
| | - Rebecca Kent
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
| | - Joseph Tremaglio
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
| | - Oscar K Serrano
- Transplant & Comprehensive Liver Center, Hartford, Connecticut, USA
- Department of Surgery, University of Connecticut School of Medicine, Farmington, Connecticut, USA
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9
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Oni L, Platt C, Marlais M, McCann L, Barakat F, Hesseling M, Cottis H, Protheroe S, Haigh G, Nott K, Marro J, King E, Kelly J, Sussens J, Mulvaney S, Whitby T, Morgan I, Sharma A, Al-Jayyousi R, Cheung CK, Ng C, Lander AD, Simmons W, Melling C, Grandison R, Treitl L, Salama AD, Dudley J. National recommendations for the management of children and young people with IgA vasculitis: a best available evidence, group agreement-based approach. Arch Dis Child 2024; 110:67-76. [PMID: 39379139 PMCID: PMC11671997 DOI: 10.1136/archdischild-2024-327364] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/24/2024] [Accepted: 09/18/2024] [Indexed: 10/10/2024]
Abstract
OBJECTIVE IgA vasculitis (IgAV) is the most frequently experienced subtype of vasculitis seen in children. Most children fully recover, however, complications including chronic kidney disease are recognised. The aim of this project was to use a best available evidence, group agreement, based approach to develop national recommendations for the initial management of IgAV and its associated complications. METHODS A fully representative multiprofessional guideline development group (GDG), consisting of 28 members, was formed and met monthly. Graded recommendations were generated using nationally accredited methods, which included a predefined scope, open consultation, systematic literature review, evidence appraisal, review of national or international guidelines and a period of open consultation. Audit measures and research priorities were incorporated. RESULTS The IgAV GDG met over a 14-month period. A total of 82 papers were relevant for evidence synthesis. For the initial management, four topic areas were identified with five key questions generating six graded recommendations related to classification, specialist referral and musculoskeletal involvement. For the associated complications, five topic areas with 12 key questions generated 15 graded recommendations covering nephritis, gastrointestinal and testicular involvement, atypical disease and follow-up. Open consultation feedback was incorporated. The guidelines were endorsed by the UK Kidney Association and Royal College of Paediatrics and Child Health and are available online. CONCLUSION Despite IgAV being a rare disease with limited evidence, a national standardised approach to the clinical management for children and young people has been achieved. This should unite approaches to care and act as a foundation for improvement.
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Affiliation(s)
- Louise Oni
- Department of Women’s and Children’s Health, University of Liverpool, Liverpool, UK
- Department of Paediatric Nephrology, Alder Hey Children’s Hospital, Liverpool
| | - Caroline Platt
- Bristol Renal Unit, Bristol Royal Hospital for Children, Bristol, UK
| | - Matko Marlais
- Department of Paediatric Nephrology, Great Ormond Street Hospital, London, UK
| | - Liza McCann
- Department of Paediatric Rheumatology, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK
| | - Farah Barakat
- Kings College Hospital NHS Foundation Trust, London, UK
| | - Markus Hesseling
- Department of Paediatrics, Children’s health Ireland, Dublin, Ireland
| | - Hannah Cottis
- Department of Paediatrics, Royal Devon University Hospital, Devon, UK
| | - Sue Protheroe
- Department of Paediatric Gastroenterology, Birmingham Children’s Hospital, Birmingham, UK
| | - Gabrielle Haigh
- Department of Paediatrics, Betsi Cadwaladr Health Board, Wales, UK
| | - Kerstin Nott
- Department of Paediatric Rheumatology, Southampton Children’s Hospital, Southampton, UK
| | - Julien Marro
- University of Liverpool Medical School, Liverpool, UK
| | | | - Jane Kelly
- General Practice, Minchinhampton Surgery, Gloucestershire, UK
| | - Jill Sussens
- Nottingham Children's Hospital, Nottingham University Hospitals NHS Trust, Nottingham, UK
| | - Shirley Mulvaney
- Department of Paediatric Emergency Medicine, Alder Hey Children’s Hospital, Liverpool, UK
| | - Thomas Whitby
- General Paediatrics, Alder Hey Children's Hospital, Liverpool, Merseyside, UK
| | - Iona Morgan
- Department of Paediatrics, Royal Hospital for Children, Glasgow, UK
| | - Amita Sharma
- Department of Paediatrics, Royal Hospital for Children, Glasgow, UK
| | | | | | | | | | - William Simmons
- Department of Paediatric Pathology, Alder Hey Children’s Hospital, Liverpool, UK
| | - Charlotte Melling
- Department of Paediatric Surgery, Alder Hey Children’s Hospital, Liverpool, UK
| | | | | | - Alan D Salama
- Department of Renal Medicine, UCL Centre for Kidney and Bladder Health, London, UK
| | - Jan Dudley
- Bristol Renal Unit, Bristol Royal Hospital for Children, Bristol, UK
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10
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Gong YQ, Han L, Zhang JY, Yu J, Wu N, Hu WP, Xu Z, Liu W, Huang WF. Abdominal imaging and endoscopic characteristics of adult abdominal IgA vasculitis: a multicenter retrospective study. Ann Med 2024; 56:2408467. [PMID: 39324401 PMCID: PMC11429444 DOI: 10.1080/07853890.2024.2408467] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/27/2024] [Revised: 06/27/2024] [Accepted: 07/03/2024] [Indexed: 09/27/2024] Open
Abstract
BACKGROUND IgA vasculitis (IgAV), previously known as Henoch-Schönlein purpura, is an IgA-mediated systemic small vessel vasculitis that tends to be more severe in adults than in children. Early diagnosis of IgAV involving the gastrointestinal tract remains difficult, especially in patients who present with gastrointestinal symptoms before purpura. This study aims to systematically analyze the abdominal imaging and endoscopic features of adult patients with abdominal IgAV, providing assistance to clinicians in the early recognition of this condition. PATIENTS AND METHODS This multicenter retrospective study was conducted in three large tertiary hospitals in China from January 2017 to January 2024. A total of 108 adult patients with abdominal IgAV, who had complete abdominal imaging and/or endoscopy results, were enrolled. The clinical manifestations, abdominal imaging findings, endoscopic characteristics, and serological indicators of the patients were analyzed. RESULTS The median age of the patients was 40 years (IQR: 26-55), with a male-to-female ratio of 2:1. Acute abdominal pain was the most common presenting symptom (100 patients, 92.59%). Bowel wall thickening was the most frequent finding on abdominal imaging (50/86 patients, 58.14%). Gastrointestinal endoscopy showed findings of congestion and erosion (32/67 patients, 47.76%), and erosion with ulcers (21/67 patients, 31.34%). Among patients with both imaging and endoscopic results, the duodenum (28/51 patients, 54.90%) and ileum (28/51 patients, 54.90%) were the most commonly affected sites. Laboratory findings revealed elevated white blood cell count (WBC), neutrophil-to-lymphocyte ratio (NLR), C-reactive protein (CRP), D-dimer and fibrinogen levels, along with decreased albumin level. Comparing patients with gastrointestinal symptoms versus purpura as the initial symptom, those with gastrointestinal symptoms had higher levels of WBC (p < 0.05) and NLR (p < 0.01). CONCLUSIONS The most common symptom in adult abdominal IgAV patients is acute abdominal pain. In the early stage of the disease, most patients exhibit elevated levels of WBC, NLR, CRP, D-dimer, and fibrinogen, along with decreased albumin level. The duodenum and ileum are the most commonly affected sites. By integrating these findings, clinicians can identify abdominal IgAV patients earlier and more accurately.
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Affiliation(s)
- Yu-Qing Gong
- Department of Gastroenterology and Hepatology, The First Affiliated Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Lin Han
- Department of Gastroenterology, Zhangzhou Affiliated Hospital of Fujian Medical University, Zhangzhou, China
| | - Jin-Yan Zhang
- Department of Gastroenterology and Hepatology, The First Affiliated Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
- The School of Clinical Medicine, Fujian Medical University, Fuzhou, China
| | - Juan Yu
- Department of Gastroenterology and Hepatology, The First Affiliated Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Na Wu
- Department of Gastroenterology and Hepatology, The First Affiliated Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Wei-Ping Hu
- Department of Nephrology, The First Affiliated Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Zhong Xu
- Department of Gastroenterology and Hepatology, The First Affiliated Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
| | - Wei Liu
- Department of Gastroenterology, Yichang Central People’s Hospital, Yichang, China
- Institute of Digestive Disease, China Three Gorges University, Yichang, China
| | - Wei-Feng Huang
- Department of Gastroenterology and Hepatology, The First Affiliated Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China
- The School of Clinical Medicine, Fujian Medical University, Fuzhou, China
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11
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Hanawa Y, Namba H. Metacarpophalangeal joint arthritis in IgA vasculitits. Oxf Med Case Reports 2024; 2024:omae157. [PMID: 39734685 PMCID: PMC11682482 DOI: 10.1093/omcr/omae157] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2024] [Revised: 08/25/2024] [Accepted: 10/08/2024] [Indexed: 12/31/2024] Open
Affiliation(s)
- Yamato Hanawa
- Department of Pediatrics, Jikei University School of Medicine, Tokyo, Japan
- Department of Pediatrics, Jikei University Kashiwa Hospital, Chiba, Japan
| | - Hiroyuki Namba
- Department of Pediatrics, Jikei University School of Medicine, Tokyo, Japan
- Department of Pediatrics, Jikei University Kashiwa Hospital, Chiba, Japan
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12
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Öksel B, Şahin N, Sönmez HE. Exploring the predictive factors in the gastrointestinal involvement of patients with immunoglobulin A vasculitis. Turk J Pediatr 2024; 66:599-607. [PMID: 39582453 DOI: 10.24953/turkjpediatr.2024.4797] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/31/2024] [Accepted: 09/30/2024] [Indexed: 11/26/2024]
Abstract
BACKGROUND Immunoglobulin A vasculitis (IgAV), the most common systemic vasculitis in children, typically presents with gastrointestinal (GI) symptoms in about half of cases. This study aimed to analyze the clinical and laboratory findings of patients with IgAV regarding GI involvement. METHODS We compared the GI involvement data of the patients diagnosed with IgAV. RESULTS Of the 210 patients (60.5% female and 39.5% male), 101 had GI involvement, with abdominal pain being the predominant symptom (n=98). White blood cell, neutrophil, monocyte, and platelet counts, C-reactive protein, neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), monocyte-to-lymphocyte ratio (MLR), systemic immune-inflammation index (SII), and systemic inflammation response index (SIRI) were significantly elevated in patients with GI involvement (p<0.001, p<0.001, p=0.01, p=0.005, p=0.002, p<0.001, p=0.03, p=0.001, p<0.001, p<0.001, respectively). The cutoff values for SII (>1035.7), SIRI (>1.65), NLR (>2.73), and MLR (>0.28) were determined, yielding respective sensitivities of 46%, 59%, 47%, and 53%, specificities of 83.1%, 69.1%, 81.3%, and 71.9%. Corresponding areas under the curve were 0.658, 0.668, 0.649, and 0.634, respectively (all p<0.001). CONCLUSION Although IgAV is a self-limiting disease, GI involvement can lead to serious consequences. Systemic inflammatory indices such as SII and SIRI may be indicative in identifying patients with GI involvement.
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Affiliation(s)
- Betül Öksel
- Department of Pediatrics, Department of Pediatric Rheumatology, Faculty of Medicine, Kocaeli University, Kocaeli, Türkiye
| | - Nihal Şahin
- Department of Pediatrics, Department of Pediatric Rheumatology, Faculty of Medicine, Kocaeli University, Kocaeli, Türkiye
| | - Hafize Emine Sönmez
- Department of Pediatrics, Department of Pediatric Rheumatology, Faculty of Medicine, Kocaeli University, Kocaeli, Türkiye
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13
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Banbury S, Milgraum DM, Lewis DJ, Queenan M, Treat JR. Post-streptococcal small-vessel vasculitis in a 16-month-old with associated intussusception and hypertension: Henoch Schönlein purpura? Pediatr Dermatol 2024; 41:929-931. [PMID: 38631675 DOI: 10.1111/pde.15599] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/02/2023] [Accepted: 02/24/2024] [Indexed: 04/19/2024]
Abstract
Henoch Schönlein purpura (HSP), also known as IgA vasculitis, is a systemic small-vessel vasculitis typically occurring in children 3-15 years of age, with peak incidence at 4-6 years. It is characterized by a constellation of symptoms including palpable purpura, arthralgias or arthritis, abdominal pain including intussusception, and renal involvement. We report a patient with these clinical findings whose IgA immunofluorescence was negative but with a presumptive diagnosis of HSP at 16 months of age, significantly younger than the classic population. This condition rarely affects this age group, and we highlight the importance of considering vasculitis in children of all ages, as a failure to diagnose could lead to insufficient long-term monitoring, particularly regarding renal function.
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Affiliation(s)
- Sara Banbury
- Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - David M Milgraum
- Section of Pediatric Dermatology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - Daniel J Lewis
- Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Maria Queenan
- Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - James R Treat
- Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
- Section of Pediatric Dermatology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
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14
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Cui S, Liu L, Zhang F. The Clinical Effect and Safety of Dihuang Decoction in Henoch-Schönlein Purpura Compared With Different Traditional Programs: A Network Meta-Analysis. Cureus 2024; 16:e64457. [PMID: 39007015 PMCID: PMC11245892 DOI: 10.7759/cureus.64457] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/13/2024] [Indexed: 07/16/2024] Open
Abstract
This systematic review aims to evaluate the therapeutic efficacy of Dihuang decoction (DD), anti-inflammatory drugs (AIDs), blood circulation improvement drugs (BCIDs), and conventional therapy (CT) in the management of Henoch-Schönlein purpura (HSP) and to establish their comparative effectiveness rankings. Using the Population, Intervention, Comparison, Outcome, Study (PICOS) design framework, we developed a detailed search strategy. The literature search included databases such as PubMed, Embase, Cochrane Library, China National Knowledge Infrastructure, Wanfang Data, Weipu Journal Data, and the Chinese Biomedical Database, covering studies published up to June 2024. We included randomized controlled trials that featured the DD as the experimental intervention, with three remaining treatments as comparators. Our analysis encompassed 63 studies with 5,435 participants, divided into 2,817 in the experimental group and 2,618 in the control group. The network meta-analysis suggested that the DD potentially surpasses AIDs, BCIDs, and CT in the management of HSP. This conclusion is supported by its superior SUCRA (Surface Under the Cumulative Ranking) scores across various measures, including the overall effective rate of medication, time to relief or disappearance of the rash, incidence of adverse reactions, time to relief or disappearance of abdominal pain, time to relief or disappearance of arthritic swelling or pain, IgA levels, and the relapse rate within six months (SUCRA scores: 100.0%, 88.3%, 79.8%, 94.4%, 99.9%, 88.3%, and 95.4%, respectively). In terms of overall effectiveness rate, the SUCRA efficacy rankings are as follows: DD > AIDs > AIDs+BCID > BCID > CT. Regarding rash relief and regression time, the SUCRA efficacy rankings are as follows: DD > CT > AIDs+BCID > AIDs. For the incidence rate of adverse reactions, the SUCRA efficacy rankings are as follows: DD > CT > AIDs > BCID > AIDs+BCID. For the relief and disappearance of abdominal pain, the SUCRA efficacy rankings are as follows: DD > CT > AIDs+BCID > AIDs. In terms of relief and disappearance of joint swelling and pain, the SUCRA efficacy rankings are as follows: DD > AIDs+BCID > AIDs. Regarding IgA changes, the SUCRA efficacy rankings are as follows: DD > CT > BCID > AIDs+BCID > AIDs. For the six-month recurrence rate, the SUCRA efficacy rankings are as follows: DD > AIDs > AIDs+BCID > CT. The DD appears to be a more effective alternative for treating HSP compared to AIDs, BCIDs, and CT. We hope that this study will provide better assistance to clinical practice.
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Affiliation(s)
- Shifang Cui
- Traditional Chinese Internal Medicine, School of Basic Medical Sciences, Heilongjiang University of Chinese Medicine, Harbin, CHN
| | - Lin Liu
- Traditional Chinese Internal Medicine, School of Basic Medical Sciences, Heilongjiang University of Chinese Medicine, Harbin, CHN
| | - Fuli Zhang
- Traditional Chinese Internal Medicine, School of Basic Medical Sciences, Heilongjiang University of Chinese Medicine, Harbin, CHN
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15
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Ahn JG. Overview of childhood vasculitis. JOURNAL OF RHEUMATIC DISEASES 2024; 31:135-142. [PMID: 38957367 PMCID: PMC11215247 DOI: 10.4078/jrd.2024.0045] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/08/2024] [Revised: 05/13/2024] [Accepted: 05/29/2024] [Indexed: 07/04/2024]
Abstract
Pediatric vasculitis and adult vasculitis differ in several aspects. While both involve inflammation of blood vessels, pediatric vasculitis tends to present with distinct clinical features and may involve different types of blood vessels compared to adult vasculitis. Despite its relatively rare occurrence compared to adult vasculitis, pediatric vasculitis warrants careful attention due to its potential for profound and diverse clinical manifestations, ranging from mild cutaneous symptoms to life-threatening systemic complications. Childhood vasculitis should be suspected in children who present symptoms attributable to systemic inflammation and complications arising from multi-organ dysfunction. However, achieving a diagnosis necessitates thorough exclusion of alternative conditions manifesting similar symptoms and findings. Hence, children suspected of vasculitis should undergo meticulous history-taking, comprehensive physical examination, and requisite laboratory investigations, imaging studies, and sometimes tissue biopsies to elucidate the diagnosis. Early detection and treatment of childhood vasculitis are crucial, as the condition can affect various organs and potentially lead to life-threatening complications or long-term sequelae in adulthood if left untreated. This review aimed to provide an exhaustive overview of childhood vasculitis, outlining its epidemiology, classification, clinical presentation, diagnostic modalities, therapeutic strategies and outcome.
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Affiliation(s)
- Jong Gyun Ahn
- Department of Pediatrics, Severance Children’s Hospital, Seoul, Korea
- Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Korea
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16
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Ahn J, Shin S, Lee GC, Han BE, Lee E, Ha EK, Shin J, Lee WS, Kim JH, Han MY. Unraveling the link between atopic dermatitis and autoimmune diseases in children: Insights from a large-scale cohort study with 15-year follow-up and shared gene ontology analysis. Allergol Int 2024; 73:243-254. [PMID: 38238236 DOI: 10.1016/j.alit.2023.12.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2023] [Revised: 12/11/2023] [Accepted: 12/13/2023] [Indexed: 04/02/2024] Open
Abstract
BACKGROUND Atopic dermatitis and autoimmune diseases are highly heritable conditions that may co-occur from an early age. METHODS The primary study is a national administrative cohort study involving 499,428 children born in 2002, tracked until 2017. Atopic dermatitis was defined as five or more principal diagnoses of atopic dermatitis and two or more topical steroid prescriptions. We estimated the risks for the occurrence of 41 autoimmune diseases, controlling for risk factors. In addition, we sourced a gene library from the National Library of Medicine to conduct a comprehensive gene ontology. We used Gene Weaver to identify gene set similarity and clustering, and used GeneMania to generate a network for shared genes. RESULTS Exposed and unexposed groups included 39,832 and 159,328 children, respectively. During a mean follow-up of 12 years, the exposed group had an increased risk of autoimmune disease (hazard ratio, 1.27 [95 % confidence interval, 1.23-1.32]) compared to the unexposed group. The hazard ratios of autoimmune illnesses consistently increased with two- and five years lag times and alternative atopic dermatitis definitions. Shared genes between atopic dermatitis and autoimmune diseases were associated with comorbidities such as asthma, bronchiolitis, and specific infections. Genetic interactions of these shared genes revealed clustering in Th1, Th2, Th17, and non-classifiable pathways. CONCLUSIONS Atopic dermatitis was significantly associated with an increased risk of subsequent autoimmune disease. we identified the genetically associated disease in atopic dermatitis patients comorbid with autoimmune disease and demonstrated a genetic network between atopic dermatitis and autoimmune diseases.
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Affiliation(s)
- Jungho Ahn
- Department of Biochemistry, Research Institute for Basic Medical Science, CHA University School of Medicine, Seongnam, South Korea
| | - Seungyong Shin
- CHA Bundang Medical Center, CHA University School of Medicine, Seongnam, South Korea
| | - Gi Chun Lee
- Department of Computer Science and Engineering, College of Engineering, Konkuk University, Seoul, South Korea
| | - Bo Eun Han
- Department of Software, Sejong University, Seoul, South Korea; Department of Pediatrics, CHA Bundang Medical Center, CHA University School of Medicine, Seongnam, South Korea
| | - Eun Lee
- Department of Pediatrics, Chonnam National University Hospital, Chonnam National University Medical School, Gwangju, South Korea
| | - Eun Kyo Ha
- Department of Pediatrics, Hallym University Kangnam Sacred Heart Hospital, Seoul, South Korea
| | - Jeewon Shin
- Department of Pediatrics, CHA Bundang Medical Center, CHA University School of Medicine, Seongnam, South Korea
| | - Won Seok Lee
- Department of Pediatrics, CHA Ilsan Medical Center, CHA University, Goyang, South Korea
| | - Ju Hee Kim
- Department of Pediatrics, Kyung Hee University Medical Center, Kyung Hee University, Seoul, South Korea.
| | - Man Yong Han
- Department of Pediatrics, CHA Bundang Medical Center, CHA University School of Medicine, Seongnam, South Korea.
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17
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Miki H, Tsuboi H, Kawashima F, Sugita T, Nishiyama T, Kuroda Y, Sawabe T, Uematsu N, Terasaki M, Kitada A, Honda F, Ohyama A, Yagishita M, Asashima H, Hagiwara S, Kondo Y, Matsumoto I. Multidrug-resistant IgA Vasculitis with Gastrointestinal Symptoms Successfully Treated with Intravenous Cyclophosphamide and Maintained with Mycophenolate Mofetil. Intern Med 2024; 63:743-747. [PMID: 37468247 PMCID: PMC10982010 DOI: 10.2169/internalmedicine.1990-23] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/20/2023] [Accepted: 06/06/2023] [Indexed: 07/21/2023] Open
Abstract
We present the case of a 17-year-old woman with IgA vasculitis (IgAV) who presented with relapsing gastrointestinal (GI) symptoms that were refractory to glucocorticoid and combination therapy with cyclosporine A, azathioprine or mycophenolate mofetil (MMF). The patient responded well to remission induction with intravenous cyclophosphamide (IVCY) and was successfully maintained with MMF. Remission induction with IVCY followed by maintenance therapy with MMF was effective in a patient with multidrug-resistant IgAV with GI lesions.
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Affiliation(s)
- Haruka Miki
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Hiroto Tsuboi
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Fumina Kawashima
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Toshiki Sugita
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Taihei Nishiyama
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Yuki Kuroda
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Tomonori Sawabe
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Nana Uematsu
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Mayu Terasaki
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Ayako Kitada
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Fumika Honda
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Ayako Ohyama
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Mizuki Yagishita
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Hiromitsu Asashima
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Shinya Hagiwara
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Yuya Kondo
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
| | - Isao Matsumoto
- Department of Rheumatology, Institute of Medicine, University of Tsukuba, Japan
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18
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Filleron A, Cezar R, Fila M, Protsenko N, Van Den Hende K, Jeziorski E, Occean B, Chevallier T, Corbeau P, Tran TA. Regulatory T and B cells in pediatric Henoch-Schönlein purpura: friends or foes? Arthritis Res Ther 2024; 26:52. [PMID: 38365843 PMCID: PMC10870453 DOI: 10.1186/s13075-024-03278-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/04/2023] [Accepted: 01/25/2024] [Indexed: 02/18/2024] Open
Abstract
BACKGROUND AND OBJECTIVES Henoch-Schönlein purpura (HSP) is the most common immunoglobulin A-mediated systemic vasculitis in childhood. We studied immune dysregulation in HSP by analyzing regulatory T (Treg), T helper 3 (Th3), and regulatory B cell (Breg) subpopulations that might intervene in immune activation, IgA production, and HSP clinical manifestations. METHODS This prospective study included 3 groups of children: 30 HSP on acute phase, 30 HSP on remission, and 40 healthy controls (HCs) matched on age. Treg, Breg, and Th3 were analyzed by flow cytometry. Serum immunoglobulin and cytokine levels were quantified by ELISA and Luminex. RESULTS Treg frequencies were higher in acute HSP than in remitting HSP and HCs (6.53% [4.24; 9.21] vs. 4.33% [3.6; 5.66], p = 0.002, and vs. 4.45% [3.01; 6.6], p = 0.003, respectively). Activated Th3 cells (FoxP3 + Th3 cells) tend to be more abundant in HSP than in HCs (78.43% [50.62; 80.84] vs. 43.30% [40.20; 49.32], p = 0.135). Serum IgA, IL-17, and latency-associated peptide (a marker of the anti-inflammatory cytokine TGF-beta production) were significantly and inflammatory cytokines TNF-alpha, IL-1-beta, and IL-6 were non-significantly higher in HSP than HCs. Bregs were identical between the groups, but, in patients with renal impairment, Breg percentage was lower compared to those without. Treg removal in PBMC culture resulted in an increase in IgA production in HSP proving a negative regulatory role of Tregs on IgA production. CONCLUSIONS In pediatric HSP, immune activation persists in spite of an increase in Th3 and Tregs. Th3 could be involved in IgA hyperproduction, inefficiently downregulated by Tregs. Lack of Bregs appears linked to renal impairment.
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Affiliation(s)
- Anne Filleron
- IRMB, Montpellier University, INSERM U1183, Montpellier, France
- Department of Pediatrics, Nîmes University Hospital, Montpellier University, Service de Pédiatrie, Place du Pr R. Debré, 30029, Nîmes Cedex 9, France
| | - Renaud Cezar
- IRMB, Montpellier University, INSERM U1183, Montpellier, France
- Department of Immunology, Nîmes University Hospital, Montpellier University, Nîmes, France
| | - Marc Fila
- Department of Pediatric Nephrology, Montpellier University Hospital, Montpellier University, Montpellier, France
| | - Nastassja Protsenko
- Department of Pediatrics, Nîmes University Hospital, Montpellier University, Service de Pédiatrie, Place du Pr R. Debré, 30029, Nîmes Cedex 9, France
| | - Kathleen Van Den Hende
- Department of Pediatrics, Nîmes University Hospital, Montpellier University, Service de Pédiatrie, Place du Pr R. Debré, 30029, Nîmes Cedex 9, France
| | - Eric Jeziorski
- Department of Pediatric Infectious Diseases, Montpellier University Hospital, Univ Montpellier, INSERM, EFS, Univ Antilles, Montpellier, France
| | - Bob Occean
- Department of Epidemiology, Medical Statistics and Public Health, Nîmes University Hospital, Montpellier University, Nîmes, France
| | - Thierry Chevallier
- Department of Epidemiology, Medical Statistics and Public Health, Nîmes University Hospital, Montpellier University, Nîmes, France
- UMR 1302 Desbrest Institute of Epidemiology and Public Health, INSERM, University of Montpellier, Montpellier, France
| | - Pierre Corbeau
- Department of Immunology, Nîmes University Hospital, Montpellier University, Nîmes, France
- Institute of Human Genetics, CNRS UMR9002, Montpellier University, Montpellier, France
| | - Tu Anh Tran
- IRMB, Montpellier University, INSERM U1183, Montpellier, France.
- Department of Pediatrics, Nîmes University Hospital, Montpellier University, Service de Pédiatrie, Place du Pr R. Debré, 30029, Nîmes Cedex 9, France.
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19
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Di Vincenzo F, Ennas S, Pizzoferrato M, Bibbò S, Porcari S, Ianiro G, Cammarota G. Henoch-schonlein purpura following exposure to SARS-CoV2 vaccine or infection: a systematic review and a case report. Intern Emerg Med 2024; 19:13-37. [PMID: 37500944 PMCID: PMC10827835 DOI: 10.1007/s11739-023-03366-w] [Citation(s) in RCA: 4] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/27/2023] [Accepted: 07/03/2023] [Indexed: 07/29/2023]
Abstract
BACKGROUND Henoch-Schonlein purpura (HSP) is an IgA-mediated systemic small-vessel vasculitis (IgAV) that typically presents with a variable tetrad of symptoms. HSP if often preceded by respiratory tract infections, vaccinations, drugs or malignancies. During the recent COVID-19 pandemic multiples cases of HSP have been described after both infection and vaccination for SARS-CoV2. This study aims to perform a systematic review of literature and describe an additional complicated case of de-novo HSP appeared after the administration of the third dose of a mRNA-SARS-CoV2 vaccination. METHODS Electronic bibliographic research was performed to identify all the original reports describing cases of de-novo HSP or IgAV appeared after respiratory infection or vaccine administration for SARS-CoV2. We included all case series or case reports of patients who respected our inclusion and exclusion criteria. RESULTS Thirty-eight publications met our pre-defined inclusion criteria, for an overall number of 44 patients. All patients presented with palpable purpura variable associated with arthralgia, abdominal pain or renal involvement. Increased levels of inflammation markers, mild leukocytosis and elevated D-dimer were the most common laboratory findings. Up to 50% of patients presented proteinuria and/or hematuria. Almost all skin biopsies showed leukocytoclastic vasculitis, with IgA deposits at direct immunofluorescence in more than 50% of cases. CONCLUSIONS Our results suggest that the immune response elicited by SARS-CoV2 vaccine or infection could play a role in the development of HSP. Current research suggests a possible role of IgA in immune hyperactivation, highlighted by early seroconversion to IgA found in some COVID-19 patients who develop IgA vasculitis.
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Affiliation(s)
- Federica Di Vincenzo
- UOC di Gastroenterologia, Dipartimento di Scienze Mediche e Chirurgiche, Fondazione Policlinico Universitario A. Gemelli IRCCS, L. go A. Gemelli 8, Roma, Italia
- Dipartimento di Medicina e Chirurgia Traslazionale, Università Cattolica del Sacro Cuore, L. go F. Vito 1, Roma, Italia
| | - Sara Ennas
- UOC di Gastroenterologia, Dipartimento di Scienze Mediche e Chirurgiche, Fondazione Policlinico Universitario A. Gemelli IRCCS, L. go A. Gemelli 8, Roma, Italia
| | - Marco Pizzoferrato
- UOC di Gastroenterologia, Dipartimento di Scienze Mediche e Chirurgiche, Fondazione Policlinico Universitario A. Gemelli IRCCS, L. go A. Gemelli 8, Roma, Italia.
| | - Stefano Bibbò
- UOC di Gastroenterologia, Dipartimento di Scienze Mediche e Chirurgiche, Fondazione Policlinico Universitario A. Gemelli IRCCS, L. go A. Gemelli 8, Roma, Italia
| | - Serena Porcari
- UOC di Gastroenterologia, Dipartimento di Scienze Mediche e Chirurgiche, Fondazione Policlinico Universitario A. Gemelli IRCCS, L. go A. Gemelli 8, Roma, Italia
| | - Gianluca Ianiro
- UOC di Gastroenterologia, Dipartimento di Scienze Mediche e Chirurgiche, Fondazione Policlinico Universitario A. Gemelli IRCCS, L. go A. Gemelli 8, Roma, Italia
- Dipartimento di Medicina e Chirurgia Traslazionale, Università Cattolica del Sacro Cuore, L. go F. Vito 1, Roma, Italia
| | - Giovanni Cammarota
- UOC di Gastroenterologia, Dipartimento di Scienze Mediche e Chirurgiche, Fondazione Policlinico Universitario A. Gemelli IRCCS, L. go A. Gemelli 8, Roma, Italia
- Dipartimento di Medicina e Chirurgia Traslazionale, Università Cattolica del Sacro Cuore, L. go F. Vito 1, Roma, Italia
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20
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Kocic M, Rasic P, Marusic V, Prokic D, Savic D, Milickovic M, Kitic I, Mijovic T, Sarajlija A. Age-specific causes of upper gastrointestinal bleeding in children. World J Gastroenterol 2023; 29:6095-6110. [PMID: 38186684 PMCID: PMC10768410 DOI: 10.3748/wjg.v29.i47.6095] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/25/2023] [Revised: 11/05/2023] [Accepted: 12/01/2023] [Indexed: 12/19/2023] Open
Abstract
The etiology of upper gastrointestinal bleeding (UGIB) varies by age, from newborns to adolescents, with some of the causes overlapping between age groups. While particular causes such as vitamin K deficiency and cow's milk protein allergy are limited to specific age groups, occurring only in neonates and infants, others such as erosive esophagitis and gastritis may be identified at all ages. Furthermore, the incidence of UGIB is variable throughout the world and in different hospital settings. In North America and Europe, most UGIBs are non-variceal, associated with erosive esophagitis, gastritis, and gastric and duodenal ulcers. In recent years, the most common causes in some Middle Eastern and Far Eastern countries are becoming similar to those in Western countries. However, variceal bleeding still predominates in certain parts of the world, especially in South Asia. The most severe hemorrhage arises from variceal bleeding, peptic ulceration, and disseminated intravascular coagulation. Hematemesis is a credible indicator of a UGI source of bleeding in the majority of patients. Being familiar with the most likely UGIB causes in specific ages and geographic areas is especially important for adequate orientation in clinical settings, the use of proper diagnostic tests, and rapid initiation of the therapy. The fundamental approach to the management of UGIB includes an immediate assessment of severity, detecting possible causes, and providing hemodynamic stability, followed by early endoscopy. Unusual UGIB causes must always be considered when establishing a diagnosis in the pediatric population because some of them are unique to children. Endoscopic techniques are of significant diagnostic value, and combined with medicaments, may be used for the management of acute bleeding. Finally, surgical treatment is reserved for the most severe bleeding.
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Affiliation(s)
- Marija Kocic
- Department of Gastroenterology, Hepatology and Nutrition, Mother and Child Health Care Institute of Serbia “Dr. Vukan Cupic”, Belgrade 11000, Serbia
| | - Petar Rasic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia “Dr. Vukan Cupic”, Belgrade 11000, Serbia
| | - Vuk Marusic
- Institute of Epidemiology, Faculty of Medicine, University of Belgrade, Belgrade 11000, Serbia
| | - Dragan Prokic
- Department of Gastroenterology, Hepatology and Nutrition, Mother and Child Health Care Institute of Serbia “Dr. Vukan Cupic”, Belgrade 11000, Serbia
- Faculty of Medicine, University of Belgrade, Belgrade 11000, Serbia
| | - Djordje Savic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia “Dr. Vukan Cupic”, Belgrade 11000, Serbia
- Faculty of Medicine, University of Belgrade, Belgrade 11000, Serbia
| | - Maja Milickovic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia “Dr. Vukan Cupic”, Belgrade 11000, Serbia
- Faculty of Medicine, University of Belgrade, Belgrade 11000, Serbia
| | - Ivana Kitic
- Department of Gastroenterology, Hepatology and Nutrition, Mother and Child Health Care Institute of Serbia “Dr. Vukan Cupic”, Belgrade 11000, Serbia
- Faculty of Medicine, University of Belgrade, Belgrade 11000, Serbia
| | - Tanja Mijovic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia “Dr. Vukan Cupic”, Belgrade 11000, Serbia
| | - Adrijan Sarajlija
- Faculty of Medicine, University of Belgrade, Belgrade 11000, Serbia
- Pediatric Day Care Hospital Department, Mother and Child Health Care Institute of Serbia “Dr. Vukan Cupic”, Belgrade 11000, Serbia
- Faculty of Medicine, University of Eastern Sarajevo, Foča 73300, Bosnia and Herzegovina
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21
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Levanon S, Gotloib V, Kraus Y, Novofastovski I, Brikman S, Fawaz A, Egbaria M, Butbul Aviel Y, Balbir-Gurman A, Mader R, Bieber A. IgA vasculitis in adults, pediatrics and non-vasculitic IgA nephropathy, retrospective analysis from 2 centers. Medicine (Baltimore) 2023; 102:e36521. [PMID: 38115301 PMCID: PMC10727533 DOI: 10.1097/md.0000000000036521] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/26/2023] [Revised: 11/03/2023] [Accepted: 11/13/2023] [Indexed: 12/21/2023] Open
Abstract
Renal involvement represents the major long-term morbidity associated with IgA vasculitis (IgAV). Our aim was to evaluate clinical characteristics and long-term renal outcomes of IgAV in pediatrics and adults comparing to IgA nephropathy (IgAN). Our retrospective study included children and adults with IgAV and IgAN patients, admitted in a 13-year period (2007-2019) to rheumatology clinics and in hospital pediatric and internal medicine departments. We compared frequencies of clinical manifestations, laboratory findings, treatments, long-term outcomes at 1 year follow-up, including all-cause mortality and dialysis until the end of follow-up time. A total of 60 adult IgAV, 60 pediatric IgAV and 45 IgAN patients were evaluated. Adult IgAV patients were significantly older than IgAN patients (53.1 ± 17.4 years vs 45.1 ± 15.7 years respectively, P = .02) and had significantly higher rates of cardiovascular comorbidities. The risk and time to dialysis were similar among IgAN and adult IgAV groups. Yet, overall mortality at long term follow up was higher in IgAV adult group compared to IgAN. No dialysis or renal transplantation were reported in pediatric IgAV patients. IgAV and IgAN adult patients were comparable regarding risk of end stage renal disease. Of note, high mortality rates were observed among adult IgAV group.
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Affiliation(s)
| | - Vera Gotloib
- Pediatric Rheumatology Service, Emek Medical Center, Afula, Israel
| | | | | | - Shay Brikman
- Rheumatology, Emek Medical Center, Afula, Israel
- Rappaport Faculty of Medicine, Technion, Haifa, Israel
| | | | | | - Yonatan Butbul Aviel
- Rappaport Faculty of Medicine, Technion, Haifa, Israel
- The Ruth Rappaport Children’s Hospital, Pediatric Rheumatology Service, Haifa, Israel
| | - Alexandra Balbir-Gurman
- Rappaport Faculty of Medicine, Technion, Haifa, Israel
- Rambam Health Care Campus, Rheumatology, Haifa, Israel
| | - Reuven Mader
- Rheumatology, Emek Medical Center, Afula, Israel
- Rappaport Faculty of Medicine, Technion, Haifa, Israel
| | - Amir Bieber
- Rheumatology, Emek Medical Center, Afula, Israel
- Rappaport Faculty of Medicine, Technion, Haifa, Israel
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22
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Sunar Yayla EN, Bakkaloğlu SA. Does age at disease onset affect the clinical presentation and outcome in children with immunoglobulin A vasculitis? Arch Rheumatol 2023; 38:633-641. [PMID: 38125056 PMCID: PMC10728748 DOI: 10.46497/archrheumatol.2023.9914] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2022] [Accepted: 02/09/2023] [Indexed: 12/23/2023] Open
Abstract
Objectives The study aimed to determine whether there is a relationship between the age at diagnosis and the clinical, laboratory, and prognostic features in pediatric immunoglobulin A vasculitis (IgAV) patients. Patients and methods In this study, 539 pediatric IgAV patients (298 males, 241 females; mean age: 7.74±3.36 years; range, 1 to 17.8 years) were retrospectively evaluated between January 2005 and July 2020. The relationship between clinical findings and age at diagnosis was analyzed by univariate logistic regression analysis. Factors associated with renal involvement, steroid-dependent or refractory disease, and recurrence were examined. Results The median age of diagnosis was 7.1 (1-17.8) years in all patients. At the time of admission, purpura, abdominal pain, and arthritis were the most common clinical findings. At the time of diagnosis, there was a positive association between age and purpura and an inverse association with the presence of arthritis. There were associations between renal involvement and age at diagnosis (odds ratio=1.22, 95% confidence interval 1.13-1.31, p<0.001), follow-up time (p<0.001), no history of previous infection (p<0.001), and presence of gastrointestinal (GI) involvement (p=0.003). Significant relationships were found between the age at diagnosis, follow-up time, GI involvement, renal involvement, scrotal involvement, the C-reactive protein value at the time of diagnosis, and the presence of steroid-dependent disease. An association was found between recurrence and GI involvement. All refractory patients had renal involvement. Age at diagnosis (p<0.001) and follow-up time (p<0.001) was found to be associated with refractory disease. Conclusion Age at diagnosis and follow-up time may be associated with renal involvement and refractory and steroid-dependent disease in IgAV. In addition, there may be a relationship between steroid-dependent disease and renal, GI, and scrotal involvement and between GI involvement and recurrence.
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Affiliation(s)
- Emine Nur Sunar Yayla
- Department of Pediatrics, Division of Pediatric Rheumatology, Gazi University Faculty of Medicine, Ankara, Türkiye
| | - Sevcan A. Bakkaloğlu
- Department of Pediatrics, Division of Pediatric Rheumatology, Gazi University Faculty of Medicine, Ankara, Türkiye
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23
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Williams CEC, Lamond M, Marro J, Chetwynd AJ, Oni L. A narrative review of potential drug treatments for nephritis in children with IgA vasculitis (HSP). Clin Rheumatol 2023; 42:3189-3200. [PMID: 37755547 PMCID: PMC10640478 DOI: 10.1007/s10067-023-06781-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/19/2023] [Revised: 09/14/2023] [Accepted: 09/15/2023] [Indexed: 09/28/2023]
Abstract
Immunoglobulin A (IgA) vasculitis (IgAV, also known as Henoch-Schoenlein purpura, HSP) is the most common vasculitis of childhood. It usually presents with a simple, self-limiting disease course; however, a small subset of patients may develop kidney involvement (IgAV-N) which occurs 4-12 weeks after disease onset and is the biggest contributor to long-term morbidity. Treatment currently targets patients with established kidney involvement; however; there is a desire to work towards early prevention of inflammation during the window of opportunity between disease presentation and onset of significant nephritis. There are no clinical trials evaluating drugs which may prevent or halt the progression of nephritis in children with IgAV apart from the early use of corticosteroids which have no benefit. This article summarises the latest scientific evidence and clinical trials that support potential therapeutic targets for IgAV-N that are currently being developed based on the evolving understanding of the pathophysiology of IgAV-N. These span the mucosal immunity, B-cell and T-cell modulation, RAAS inhibition, and regulation of complement pathways, amongst others. Novel drugs that may be considered for use in early nephritis include TRF-budesonide; B-cell inhibiting agents including belimumab, telitacicept, blisibimod, VIS649, and BION-1301; B-cell depleting agents such as rituximab, ofatumumab, and bortezomib; sparsentan; angiotensin converting enzyme inhibitors (ACE-Is); and complement pathway inhibitors including avacopan, iptacopan, and narsoplimab. Further clinical trials, as well as pre-clinical scientific studies, are needed to identify mechanistic pathways as there may be an opportunity to prevent nephritis in this condition. Key Points • Kidney involvement is the main cause of long-term morbidity and mortality in IgA vasculitis despite the current treatment recommendations. • The evolving understanding of the pathophysiology of IgA vasculitis is allowing exploration of novel treatment options which target underlying immune pathways. • Novel treatments currently being trialled in IgA nephropathy may have benefit in IgA vasculitis due to the similarities in the underlying pathophysiology, such as TRF-budesonide, B-cell modulators, and complement inhibitors. • Further studies, including clinical trials of novel drugs, are urgently needed to improve the long-term outcomes for children with IgA vasculitis nephritis.
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Affiliation(s)
- Chloe E C Williams
- Royal Liverpool and Broadgreen University Hospital Trusts, Liverpool, UK
- Department of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK
| | - Megan Lamond
- Department of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK
| | - Julien Marro
- School of Medicine, University of Liverpool, Liverpool, UK
| | - Andrew J Chetwynd
- Department of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK
- Centre for Proteome Research, Institute of Systems, Molecular and Integrative Biology, University of Liverpool, Liverpool, UK
| | - Louise Oni
- Department of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK.
- Department of Paediatric Nephrology, Institute in the Park Building, University of Liverpool, Alder Hey Children's NHS Foundation Trust Hospital, Eaton Road, Liverpool, L12 2AP, UK.
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24
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Mukanhaire L, Ren X, Liu G, Wang T, Kasumba YY, Zhou X, Peng H. Recurrence of Henoch Schoenlein Purpura Nephritis in Children: A Retrospective Study. Heliyon 2023; 9:e22501. [PMID: 38034624 PMCID: PMC10687055 DOI: 10.1016/j.heliyon.2023.e22501] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/08/2023] [Revised: 11/11/2023] [Accepted: 11/14/2023] [Indexed: 12/02/2023] Open
Abstract
The aim of the study was to identify predictive patient characteristics for Henoch Schoenlein Purpura (HSPN) relapse in childhood HSPN. One hundred and thirty-five Chinese children with HSPN were enrolled in this study, mean age 10.25 ± 3.39 years. The pathology of HSPN was according to the International Study of Kidney Disease in Children criteria.(ISKDC); ISKDC II(mesangial proliferation (MP)) AND ISKDC III (MP with <50 % crescents).Recurrence of HSPN was observed in 66.3 % patients; male to female ratio (2:1)Statistically significant correlation existed between biopsy grade(p < 0.001), gender(p < 0.001),age ranges(p = 0.002) and treatment regimen (p < 0.001)in the frequency of recurrent HSPN episodes. We identified some significant predictors for HSPN relapse such as the severity of HSPN, adjunctive therapies administered to these patients,and close attention should be paid in patients between the ages 7 and 12 years old. In addition, the use of mycophenolate mofetil as an adjunctive therapy in the treatment of HSPN may reduce the frequency of HSPN relapse episodes in children.
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Affiliation(s)
- Lydia Mukanhaire
- School of Basic Medicine and Clinical Pharmacy, China Pharmaceutical University, Nanjing, Jiangsu, 211198, China
- Department of Pediatrics, Sir Run Run Hospital, Nanjing Medical University, Nanjing, Jiangsu Province, 211166, China
- Department of Pediatrics, Nanjing Drum Tower Hospital, Nanjing, Jiangsu Province, 210008, China
| | - Xianguo Ren
- Department of Pediatrics, Sir Run Run Hospital, Nanjing Medical University, Nanjing, Jiangsu Province, 211166, China
| | - Guangling Liu
- Department of Pediatrics, Nanjing Drum Tower Hospital, Nanjing, Jiangsu Province, 210008, China
| | - Ting Wang
- Department of Pediatrics, Sir Run Run Hospital, Nanjing Medical University, Nanjing, Jiangsu Province, 211166, China
| | - Yeukai Y. Kasumba
- School of Basic Medicine and Clinical Pharmacy, China Pharmaceutical University, Nanjing, Jiangsu, 211198, China
| | - Xiaohui Zhou
- School of Basic Medicine and Clinical Pharmacy, China Pharmaceutical University, Nanjing, Jiangsu, 211198, China
| | - Hongjun Peng
- Department of Pediatrics, Nanjing Drum Tower Hospital, Nanjing, Jiangsu Province, 210008, China
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25
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Rosenberg S, Sweetser P, Ismail L. Vomiting and Abdominal Pain in a 9-year-old Boy. Pediatr Rev 2023; 44:S103-S105. [PMID: 37777227 DOI: 10.1542/pir.2021-005385] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/02/2023]
Affiliation(s)
- Sedona Rosenberg
- University of Virginia School of Medicine, Department of Ophthalmology, Charlottesville, VA
| | - Peter Sweetser
- George Washington University School of Medicine and Health Sciences, Washington, DC
| | - Lana Ismail
- Children's National Hospital, Division of Hospital Medicine, Washington, DC
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26
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Keskinyan VS, Lattanza B, Reid-Adam J. Glomerulonephritis. Pediatr Rev 2023; 44:498-512. [PMID: 37653138 DOI: 10.1542/pir.2021-005259] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 09/02/2023]
Abstract
Glomerulonephritis (GN) encompasses several disorders that cause glomerular inflammation and injury through an interplay of immune-mediated mechanisms, host characteristics, and environmental triggers, such as infections. GN can manifest solely in the kidney or in the setting of a systemic illness, and presentation can range from chronic and relatively asymptomatic hematuria to fulminant renal failure. Classic acute GN is characterized by hematuria, edema, and hypertension, the latter 2 of which are the consequence of sodium and water retention in the setting of renal impairment. Although presenting signs and symptoms and a compatible clinical history can suggest GN, serologic and urinary testing can further refine the differential diagnosis, and renal biopsy can be used for definitive diagnosis. Treatment of GN can include supportive care, renin-angiotensin-aldosterone system blockade, immunomodulatory therapy, and renal transplant. Prognosis is largely dependent on the underlying cause of GN and can vary from a self-limited course to chronic kidney disease. This review focuses on lupus nephritis, IgA nephropathy, IgA vasculitis, and postinfectious GN.
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27
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Marro J, Williams C, Pain CE, Oni L. A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children. Pediatr Rheumatol Online J 2023; 21:85. [PMID: 37580746 PMCID: PMC10424434 DOI: 10.1186/s12969-023-00872-1] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/01/2023] [Accepted: 08/02/2023] [Indexed: 08/16/2023] Open
Abstract
BACKGROUND IgA vasculitis (IgAV) is a small vessel vasculitis that is more common in childhood. Very limited evidence exists on patients who experience an atypical disease course. The aim of this study was to describe a cohort of children diagnosed with recurrent or persisting IgAV to identify any themes associated with their disease course and areas of unmet needs. METHODS A single centre retrospective study of children diagnosed with recurrent or persisting IgAV at Alder Hey Children's Hospital (Liverpool, UK). Clinical data, including features at presentation and during follow up, potential triggers, abnormal laboratory and histology results, treatment and outcome at last clinical review were retrospectively collected. Key themes were identified. RESULTS A total of 13 children met the inclusion criteria (recurrent disease, n = 4; persisting disease, n = 9). Median age at first presentation was 10.2 years [2.6-15.5], female:male ratio 1.2:1. Children in the atypical cohort were significantly older than a larger cohort of children who followed a non-complicated disease course (median age 5.5 years (range [0.6-16.7], p = 0.003)). All children re-presented with a purpuric rash (either recurring or persisting), accompanied by joint involvement in 92% of patients (12/13). Disease-modifying anti-rheumatic drugs (DMARDs) were used in 8/13 (62%) children. The median time from first presentation to diagnosis of atypical disease was 18.4 months [5.3-150.8] and the time from first presentation to treatment was 24.1 months [1.8-95.4]. Use of corticosteroids was significantly higher in children with renal involvement (p = 0.026). During follow up, 8/13 (62%) children were admitted at least once, whilst 10/13 (77%) had re-presented at least once to the emergency department. Five (38%) children were referred to psychology services and 7 (54%) children reported feelings of frustration. CONCLUSIONS This series describes some characteristics of a small cohort of children with atypical IgAV. It also identifies unmet needs in children with atypical IgAV, which includes delays in diagnosis and lengthy waits for treatment, lack of high-quality evidence regarding treatment choices and a high unrecognised disease burden. Further research is needed to study this subgroup of children as evidence is lacking.
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Affiliation(s)
- Julien Marro
- Department of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK
| | - Chloe Williams
- Royal Liverpool and Broadgreeen University Hospital Trusts, Liverpool, UK
| | - Clare E Pain
- Department of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK
- Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK
| | - Louise Oni
- Department of Women's and Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK.
- Department of Paediatric Nephrology, University of Liverpool Alder Hey Children's NHS Foundation Trust Hospital, Institute in the Park Building, Eaton Road, Liverpool, L12 2AP, UK.
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28
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YILDIRIM S, ERGÜVEN M. Reporting the clinical spectrum of children with IgAV in a retrospective 24-year cohort: Influences of age and sex on clinical presentation. Turk J Med Sci 2023; 53:1339-1347. [PMID: 38813037 PMCID: PMC10763743 DOI: 10.55730/1300-0144.5700] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2022] [Revised: 10/26/2023] [Accepted: 08/11/2023] [Indexed: 05/31/2024] Open
Abstract
Background/aim Immunoglobulin A vasculitis (IgAV) is one of the most common types of vasculitis in children. The aims of this study were to investigate the clinical characteristics of the disease, and the effects of age and sex on the clinical course in children with IgAV. Materials and methods This was a retrospective study including pediatric patients diagnosed with IgAV who attended follow-ups at the pediatric rheumatology department of a tertiary healthcare institution between January 1997 and December 2020. The patients were grouped and compared according to sex and age at diagnosis (<7 years vs. ≥7 years). Results The study included 709 children with IgAV, 392 (55.3%) of whom were male. The mean age at diagnosis was 7.9 ± 3.2 years. The most common disease onset season was autumn (31.2%). Upper respiratory infections (27.8%) were the most common predisposing factors. Gastrointestinal system (GIS), joint, and renal involvement were observed in 52.8%, 47.5%, and 17.5% of patients, respectively. Renal involvement, GIS involvement, and disease relapse were significantly more common among those diagnosed after 7 years of age compared to those diagnosed before the age of 7 (p < 0.001, p = 0.033, and p < 0.001, respectively). Scrotal involvement and subcutaneous edema were more common among those diagnosed at younger than 7 years compared to those aged ≥7 years at diagnosis (p < 0.001 and p = 0.016, respectively). GIS involvement was more frequently seen in males compared to females (p = 0.046). Conclusion It was demonstrated that being ≥7 years of age at diagnosis or being a male were associated with higher likelihood of renal and GIS involvement in children with IgAV.
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Affiliation(s)
- Sema YILDIRIM
- Department of Pediatric, Göztepe Prof. Dr. Süleyman Yalçın City Hospital, İstanbul,
Turkiye
| | - Müferet ERGÜVEN
- Department of Pediatric Rheumatology, Faculty of Medicine, Düzce University, Düzce,
Turkiye
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Horváth O, Szabó AJ, Reusz GS. How to define and assess the clinically significant causes of hematuria in childhood. Pediatr Nephrol 2023; 38:2549-2562. [PMID: 36260163 PMCID: PMC9580432 DOI: 10.1007/s00467-022-05746-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/15/2022] [Revised: 08/27/2022] [Accepted: 09/06/2022] [Indexed: 11/28/2022]
Abstract
Given the wide diversity of causes of hematuria, ranging from simple urinary tract infections with rapid recovery to severe glomerulonephritis with fast decline in kidney function, it is essential to recognize the underlying disease. The first objective of the assessment is to determine whether the cause of the hematuria is medically significant. The combination of hematuria with proteinuria, the presence of hypertension, or worsening kidney function can represent signs of progressive kidney disease. Differentiating the various causes of hematuria is often simple and obvious based on the clinical signs and gross appearance of the urine. However, in some instances, additional non-invasive investigations, such as ultrasound imaging, urinary red cell morphology, measurement of calcium and other solutes in the urine, evaluation of kidney function, and protein excretion, are needed to elucidate the nature of the hematuria. Taking a detailed family history can help in establishing the underlying cause in cases of familial hematuria. On the other hand, the decision to perform a kidney biopsy in children with asymptomatic hematuria remains a challenging issue for clinicians. Ultimately, the frequency of diagnosis of glomerular involvement causing hematuria may depend on the threshold for performing a kidney biopsy. The following review will focus on the diagnostics of hematuria, starting with difficulties regarding its definition, followed by various means to differentiate between urinary, glomerular, and other causes, and finally reviewing the most common diseases that, due to their frequency or their effect on kidney function, present a diagnostic challenge in everyday practice.
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Affiliation(s)
- Orsolya Horváth
- 1st Department of Pediatrics, Semmelweis University, 53-54 Bókay János Street, Budapest, 1083, Hungary
| | - Attila J Szabó
- 1st Department of Pediatrics, Semmelweis University, 53-54 Bókay János Street, Budapest, 1083, Hungary
| | - George S Reusz
- 1st Department of Pediatrics, Semmelweis University, 53-54 Bókay János Street, Budapest, 1083, Hungary.
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Cao T, Yang HM, Huang J, Hu Y. Risk factors associated with recurrence of Henoch-Schonlein purpura: a retrospective study. Front Pediatr 2023; 11:1164099. [PMID: 37377759 PMCID: PMC10291609 DOI: 10.3389/fped.2023.1164099] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/12/2023] [Accepted: 05/18/2023] [Indexed: 06/29/2023] Open
Abstract
Background Recurrence is considered a vital problem for assessing the prognosis of Henoch-Schonlein purpura (HSP). The objective of this study was to evaluate factors affecting the recurrence in children with HSP. Methods We retrospectively reviewed records of 368 patients under the age of 16 years diagnosed with HSP from October 2019 to December 2020 in Beijing Children's Hospital. Patients were divided into a non-recurrence group and a recurrence group according to whether there was a recurrence. Incidence of manifestation, possible cause, age, and treatment were retrospectively analyzed. Univariate and multivariate logistic regression analyses were used to determine the risk factors of recurrence in HSP. Results Percentages of patients were 65.2% for the non-recurrence group and 34.8% for the recurrence group. The percentage of patients with renal involvement was significantly higher in the recurrence group (40.6%) than in the non-recurrence group (26.3%). Respiratory tract infection was the most frequent trigger: 67.5% in the non-recurrence group and 66.4% in the recurrence group. Recurrence was more likely to occur in patients aged >6 years (53.3% vs. 71.9%). Logistic regression analysis revealed that hematuria plus proteinuria was an independent risk factor for the recurrence of HSP. Conversely, animal protein, exercise restriction, and age ≤6 years were independent favorable factors for the non-recurrence of HSP. Conclusion These results suggest that organ involvement, exercise, and diet management during the initial episode of HSP should be strictly monitored for children with HSP. Adequate clinical intervention for these risk factors may limit or prevent HSP recurrence. Moreover, renal involvement is associated with the long-term prognosis of HSP.
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Yu S, Feng W, Wang Y, Zhao M, Tu Y, Guo Z. Serum total bile acid levels assist in the prediction of acute intussusception with abdominal type Henoch-Schonlein purpura in children. Front Pediatr 2023; 11:1183470. [PMID: 37342527 PMCID: PMC10277492 DOI: 10.3389/fped.2023.1183470] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/10/2023] [Accepted: 05/23/2023] [Indexed: 06/23/2023] Open
Abstract
Background The severe acute abdomen associated with Henoch-Schonlein purpura (HSP) is an acute intussusception (AI). There is no reliable specific marker for AI with abdominal-type HSP. The serum total bile acid (TBA) level is a new prognostic marker associated with the severity of intestinal inflammation. The purpose of this study was to identify the prognostic value of serum TBA levels for the diagnosis of AI in children with abdominal-type HSP. Methods A retrospective study of 708 patients with abdominal-type HSP was conducted, with demographic data, clinical symptoms, hepatic function index, immune function markers, and clinical outcomes assessed. Patients were divided into two groups: HSP (613 patients) and HSP with AI (95 patients). The data were analysed using SPSS 22.0. Results Of the 708 patients, the serum TBA levels were higher in the HSP with AI group than in the HSP group (P < 0.05). Logistic regression analysis showed that vomiting (OR = 396.492, 95% CI = 14.93-10,529.67, P < 0.001), haematochezia (OR = 87.436, 95% CI = 5.944-1,286.214, P = 0.001), TBA (OR = 16.287, 95% CI = 4.83-54.922, P < 0.001), and D-dimer (OR = 5.987, 95% CI = 1.892-15.834, P = 0.003) were independent risk factors for abdominal-type HSP with AI. Receiver operating characteristic (ROC) curve analysis showed that the optimal cut-off serum TBA value (sensitivity = 91.58%, specificity = 84.67%, AUC = 93.6524%) was >3 μmol/L for predicting AI in children with abdominal-type HSP. In this group of HSP patients with AI, a serum TBA level ≥6.98 μmol/L was significantly associated with an increased incidence of operative treatment (51.85% vs. 75.61%, P = 0.0181), intestinal necrosis (9.26% vs. 29.27%, P = 0.0117), and length of hospital stay [15.76 ± 5.31 vs. 10.98 ± 2.83 (days), P < 0.0001]. Conclusion In children with HSP and AI, the serum TBA level was significantly higher. A novel but promising haematological indicator, the serum TBA level, helps identify HSP with and without AI and predicts intestinal necrosis in HSP with AI.
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Affiliation(s)
- Sijie Yu
- Department of Nephrology, Children’s Hospital of Chongqing Medical University, Chongqing, China
- Ministry of Education Key Laboratory of Child Development and Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China
- National Clinical Research Center for Child Health and Disorders (Chongqing), Children's Hospital of Chongqing Medical University, Chongqing, China
- China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China
- Chongqing Key Laboratory of Pediatrics, Children's Hospital of Chongqing Medical University, Chongqing, China
| | - Wei Feng
- Ministry of Education Key Laboratory of Child Development and Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China
- National Clinical Research Center for Child Health and Disorders (Chongqing), Children's Hospital of Chongqing Medical University, Chongqing, China
- China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China
- Chongqing Key Laboratory of Pediatrics, Children's Hospital of Chongqing Medical University, Chongqing, China
- Department of General Surgery, Children’s Hospital of Chongqing Medical University, Chongqing, China
| | - Yi Wang
- Ministry of Education Key Laboratory of Child Development and Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China
- National Clinical Research Center for Child Health and Disorders (Chongqing), Children's Hospital of Chongqing Medical University, Chongqing, China
- China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China
- Chongqing Key Laboratory of Pediatrics, Children's Hospital of Chongqing Medical University, Chongqing, China
- Department of General Surgery, Children’s Hospital of Chongqing Medical University, Chongqing, China
| | - Maoyuan Zhao
- Ministry of Education Key Laboratory of Child Development and Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China
- National Clinical Research Center for Child Health and Disorders (Chongqing), Children's Hospital of Chongqing Medical University, Chongqing, China
- China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China
- Chongqing Key Laboratory of Pediatrics, Children's Hospital of Chongqing Medical University, Chongqing, China
- Department of General Surgery, Children’s Hospital of Chongqing Medical University, Chongqing, China
| | - Yuying Tu
- Department of General Surgery, Jiangxi Hospital Affiliated to Children’s Hospital of Chongqing Medical University, Jiangxi, China
- Jiangxi Children’s Medical Center, Jiangxi Maternal and Child Health Hospital, Jiangxi, China
| | - Zhenhua Guo
- Ministry of Education Key Laboratory of Child Development and Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China
- National Clinical Research Center for Child Health and Disorders (Chongqing), Children's Hospital of Chongqing Medical University, Chongqing, China
- China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China
- Chongqing Key Laboratory of Pediatrics, Children's Hospital of Chongqing Medical University, Chongqing, China
- Department of General Surgery, Children’s Hospital of Chongqing Medical University, Chongqing, China
- Department of General Surgery, Jiangxi Hospital Affiliated to Children’s Hospital of Chongqing Medical University, Jiangxi, China
- Jiangxi Children’s Medical Center, Jiangxi Maternal and Child Health Hospital, Jiangxi, China
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Grewal MK, Adams MD, Valentini RP. Vasculitis and Kidney Disease. Pediatr Clin North Am 2022; 69:1199-1217. [PMID: 36880930 DOI: 10.1016/j.pcl.2022.07.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Pediatric vasculitis is a complex group of disorders that commonly presents with multisystem involvement. Renal vasculitis can be isolated to the kidneys or can occur as part of a broader multiorgan vasculitis. Depending on severity, renal vasculitis may present as acute glomerulonephritis (AGN) often associated with hypertension and sometimes with a rapidly deteriorating clinical course. Prompt diagnosis and initiation of therapy are key to preserving kidney function and preventing long-term morbidity and mortality. This review focuses on the clinical presentation, diagnosis, and treatment objectives for common forms of renal vasculitis seen in pediatric patients.
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Affiliation(s)
- Manpreet K Grewal
- Division of Nephrology and Hypertension, Department of Pediatrics, Children's Hospital of Michigan, 3901 Beaubien Boulevard, MI, 48201, USA; Department of Pediatrics, Central Michigan University College of Medicine, 1280 East Campus Drive, Mount Pleasant, MI 48858, USA
| | - Matthew D Adams
- Department of Pediatrics, Wayne State University School of Medicine, 540 East Canfield Street, Detroit, MI 48201, USA
| | - Rudolph P Valentini
- Division of Nephrology and Hypertension, Department of Pediatrics, Children's Hospital of Michigan, 3901 Beaubien Boulevard, MI, 48201, USA; Department of Pediatrics, Central Michigan University College of Medicine, 1280 East Campus Drive, Mount Pleasant, MI 48858, USA.
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Luo F, Li Y, Zhang Y, Song Y, Diao J. Bibliometric analysis of IgA vasculitis nephritis in children from 2000 to 2022. Front Public Health 2022; 10:1020231. [PMID: 36276396 PMCID: PMC9581235 DOI: 10.3389/fpubh.2022.1020231] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/16/2022] [Accepted: 09/15/2022] [Indexed: 01/28/2023] Open
Abstract
Background IgA vasculitis Nephritis (IgAVN) is a kidney-damaging disease that occurs during the course of IgA vasculitis (IgAV) and is the most serious complication of IgAV. However, there is a lack of reports of bibliometric analysis of IgAVN in children. The purpose of this study is to conduct a bibliometric analysis of IgAVN in children from 2000 to 2022, to explore the current status and cutting-edge trends in the field of IgAVN in children, and to establish new directions for subsequent research. Methods Screening the literature in the field of IgAVN in children in the Web of Science Core Collection (WoSCC) from 2000 to 2022. Visual analysis of their annual publications, countries, institutions, authors, journals, keywords, and references were using CiteSpace5.8.R3 and VOSviewer1.6.18. Results A total of 623 publications were included in the study, since the beginning of 2014, there has been an overall increasing trend in the number of articles issued. The most prolific country and institution were China and Zhejiang University. The most frequently cited author was Coppo R, with 331 citations, who has made great contributions to IgAVN. Mao Jianhua, Lee JS and Wyatt Robert J were the most prolific authors, all with 9 articles. Pediatric Nephrology was the most published and cited journal. The highest burst strength keyword is IgA vasculitis, and the highest burst strength reference is Davin JC, 2014. Conclusion The research hotspots and trends predicted by the analysis of this study provide a reference for in-depth research in this field with a view to promoting the development of IgAVN research in children.
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Affiliation(s)
- Fei Luo
- First College of Clinical Medicine, Shandong University of Traditional Chinese Medicine, Jinan, China,Department of Pediatrics, Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, China
| | - Yuzhe Li
- First College of Clinical Medicine, Shandong University of Traditional Chinese Medicine, Jinan, China,Department of Pediatrics, Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, China
| | - Yuan Zhang
- Department of Pediatrics, Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, China,College of Traditional Chinese Medicine, Shandong University of Traditional Chinese Medicine, Jinan, China
| | - Yehong Song
- First College of Clinical Medicine, Shandong University of Traditional Chinese Medicine, Jinan, China,Department of Pediatrics, Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, China
| | - Juanjuan Diao
- Department of Pediatrics, Affiliated Hospital of Shandong University of Traditional Chinese Medicine, Jinan, China,*Correspondence: Juanjuan Diao
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Sun L, Liu W, Li C, Zhang Y, Shi Y. Construction and internal validation of a predictive model for risk of gastrointestinal bleeding in children with abdominal Henoch-Schönlein purpura: A single-center retrospective case-control study. Front Immunol 2022; 13:1025335. [PMID: 36248897 PMCID: PMC9556720 DOI: 10.3389/fimmu.2022.1025335] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/22/2022] [Accepted: 09/15/2022] [Indexed: 11/13/2022] Open
Abstract
Early identification of gastrointestinal (GI) bleeding in children with abdominal Henoch-Schönlein purpura (HSP) is essential for their subsequent treatment, and a risk prediction model for GI bleeding in abdominal HSP was constructed in this study to assist physicians in their decision-making. In a single-center retrospective study, the children collected were divided into two parts, a training set and a validation set, according to the time of admission. In the training set, univariate analysis was performed to compare demographic data and laboratory tests between the two groups of children with GI and non-GI bleeding, and the independent risk factors were derived using binary logistic equations to develop a scoring model for predicting GI bleeding in children by odds ratio (OR) values and receiver operating characteristic curves. The scoring model was then internally validated in validation set. The results showed that there were 11 indicators were statistically different between the two groups in the training set, including white blood cells, neutrophil-to-lymphocyte ratio, platelets, eosinophils (EO), high sensitivity C-reactive protein (hsCRP), activated partial thromboplastin time (APTT), sodium, potassium (K), albumin (ALB), Total bilirubin, and Immunoglobulin E (IgE) in the univariate analysis. Among them, the independent risk factors for GI bleeding included the six indicators of EO ≤ 0.045×10^9/L, hsCRP ≥ 14.5 mg/L, APTT ≤ 28.1 s, K ≥ 4.18 mmol/L, ALB ≤ 40.6 g/L, and IgE ≥ 136 ng/mL. According to the OR values, where EO ≤ 0.045 ×10^9/L, hsCRP ≥ 14.5 mg/L, APTT ≤ 28.1 s, ALB ≤ 40.6 g/L each scored 3 points, K ≥ 4.18 mmol/L, IgE ≥ 136 ng/mL each scored 2 points, and the total score was 0-16 points. The sensitivity and specificity of predicting GI bleeding were 88.7% and 64.2%, respectively, when the child scored ≥ 7 points. In the validation set, the sensitivity, specificity and accuracy of the model in predicting GI bleeding were 77.4%, 74.5% and 75.2%, respectively. In conclusion, the construction of a scoring model to predict the risk of GI bleeding from abdominal HSP would greatly assist pediatricians in predicting and identifying children at high risk for GI bleeding at an early stage.
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Affiliation(s)
- Lingli Sun
- Department of Child Health, Wuhan Children’s Hospital (Wuhan Maternal and Child Healthcare Hospital), Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Wenjuan Liu
- Department of Rheumatology and Immunology, Wuhan Children’s Hospital (Wuhan Maternal and Child Healthcare Hospital), Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Changjian Li
- Department of Cardiology, Wuhan Children’s Hospital (Wuhan Maternal and Child Healthcare Hospital), Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Yong Zhang
- Department of Cardiology, Wuhan Children’s Hospital (Wuhan Maternal and Child Healthcare Hospital), Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Yuanyuan Shi
- Department of General Medicine, Wuhan Fourth Hospital, Puai Hospital, Wuhan, China
- *Correspondence: Yuanyuan Shi,
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Carucci NS, La Barbera G, Peruzzi L, La Mazza A, Silipigni L, Alibrandi A, Santoro D, Chimenz R, Conti G. Time of Onset and Risk Factors of Renal Involvement in Children with Henoch-Schönlein Purpura: Retrospective Study. CHILDREN (BASEL, SWITZERLAND) 2022; 9:1394. [PMID: 36138703 PMCID: PMC9497900 DOI: 10.3390/children9091394] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 07/01/2022] [Revised: 08/25/2022] [Accepted: 09/13/2022] [Indexed: 11/27/2022]
Abstract
BACKGROUND Henoch-Schönlein purpura (HSP) is a common systemic vasculitis in children, involving the skin, musculoskeletal system, gastrointestinal tract and kidneys. Some studies in children have shown possible risk factors linked with the development and severity of HSP Nephritis (HSPN). The aim of this study was to research predicting factors for the development of HSPN. METHODS We retrospectively evaluated 132 pediatric patients with HSP, according to EULAR/PRINTO/PRESS criteria. All patients were screened for HSPN by urinalysis. Finally, we compared demographic, clinical and laboratory data in HSP patients with and without nephritis. RESULTS The median age at HSP diagnosis [6.2 (2.6-17.5) vs. 5.5 (0.8-15.4) years, p = 0.03] and the incidence of abdominal pain (48 vs. 27%, p = 0.01) were significantly higher in HSPN patients. No differences were evidenced regarding gender, allergic diseases, skin recurrences, gastrointestinal involvement, musculoskeletal involvement, scrotal involvement, and laboratory data (white blood cell count, neutrophil count, lymphocyte count, platelet count, C-reactive protein, erythrocyte sedimentation rate, and blood concentration of IgA). CONCLUSIONS The age at diagnosis and abdominal pain were independent risk factors for renal involvement in HSP patients. However, due to the retrospective nature of this study, further long-term and prospective studies will be necessary.
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Affiliation(s)
- Nicolina Stefania Carucci
- Pediatric Nephrology and Rheumatology Unit, AOU G Martino, University of Messina, 98125 Messina, Italy
| | - Giulia La Barbera
- Pediatric Nephrology and Rheumatology Unit, AOU G Martino, University of Messina, 98125 Messina, Italy
| | - Licia Peruzzi
- Pediatric Nephrology Unit, Regina Margherita Department, Azienda Ospedaliero-Universitaria Città Della Salute E Della Scienza, 10126 Torino, Italy
| | - Antonella La Mazza
- Pediatric Nephrology and Rheumatology Unit, AOU G Martino, University of Messina, 98125 Messina, Italy
| | - Lorena Silipigni
- Pediatric Nephrology and Rheumatology Unit, AOU G Martino, University of Messina, 98125 Messina, Italy
| | - Angela Alibrandi
- Unit of Statistical and Mathematical Sciences, Department of Economics, University of Messina, 98125 Messina, Italy
| | - Domenico Santoro
- Unit of Nephrology and Dialysis, Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy
| | - Roberto Chimenz
- Pediatric Nephrology and Rheumatology Unit, AOU G Martino, University of Messina, 98125 Messina, Italy
| | - Giovanni Conti
- Pediatric Nephrology and Rheumatology Unit, AOU G Martino, University of Messina, 98125 Messina, Italy
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Yüksel S. [Frontal region oedema as the first sign in Henoch-Schönlein purpura]. An Pediatr (Barc) 2022; 98:S1695-4033(22)00190-4. [PMID: 36090689 PMCID: PMC9448641 DOI: 10.1016/j.anpedi.2022.07.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
Affiliation(s)
- Selçuk Yüksel
- Departamento de Reumatología y Nefrología Pediátricas, Facultad de Medicina, Universidad de Pamukkale, Denizli, Turquía
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Clinical Characteristics and Outcomes of IgA Vasculitis in the Elderly. J Clin Rheumatol 2022; 28:325-327. [PMID: 35067511 DOI: 10.1097/rhu.0000000000001828] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
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Clinical analysis of 99 children with Henoch-Schönlein purpura complicated with overt gastrointestinal bleeding. Clin Rheumatol 2022; 41:3783-3790. [PMID: 35941339 DOI: 10.1007/s10067-022-06323-8] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/11/2021] [Revised: 05/17/2022] [Accepted: 07/28/2022] [Indexed: 11/03/2022]
Abstract
OBJECTIVE To analyze and summarize the clinical features, diagnosis, and treatment of children with Henoch-Schönlein purpura (HSP) complicated by overt gastrointestinal bleeding (GI bleeding) for achieving early identification, prevention, and treatment in terms of severe GI bleeding. METHODS A retrospective analysis was conducted on children with HSP complicated by overt GI bleeding who were admitted to the Department of Traditional Chinese Medicine of Beijing Children's Hospital from January 2017 to December 2019. According to the severity of GI bleeding, the patients were divided into mild bleeding group (61 cases) and moderate and severe bleeding group (38 cases). Inflammatory parameters, coagulation function, GI ultrasound findings, and clinical features were compared. Logistic regression analysis was used to determine the related variables affecting the severity of GI bleeding, and the ROC curve was used to determine the variable test efficacy. RESULTS Onset in summer, wide distribution of skin rash with facial involvement, the elevation of D-dimer and high neutrophil-to-lymphocyte ratio (NLR) had significant effects on the severity of GI bleeding. ROC curve analysis showed that the optimal cut-off points of NLR and D-dimer for predicting severe GI bleeding in children with HSP were 10.56 and 0.89 mg/L, respectively. CONCLUSION Facial rash may be a warning sign of GI bleeding. Enhanced monitoring of NLR and D-dimer is helpful for early recognition of GI bleeding as well as assessment of severity.
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Torun Bayram M, Heybeli C, Yıldız G, Soylu A, Celik A, Sarioglu S, Kavukçu S. Comparison of clinical, pathological and long-term renal outcomes of children with Henoch-Schonlein purpura nephritis and IgA nephropathy. Int Urol Nephrol 2022; 54:1925-1932. [PMID: 34846620 DOI: 10.1007/s11255-021-03063-7] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/19/2021] [Accepted: 11/11/2021] [Indexed: 02/05/2023]
Abstract
PURPOSE To compare clinical, pathological, and long-term renal outcomes of children with Henoch-Schonlein purpura nephritis (HSPN) and IgA nephropathy (IgAN). METHODS The medical records of patients diagnosed as HSPN and IgAN during childhood were evaluated retrospectively. HSPN and IgAN groups were compared in terms of gender, age, upper respiratory infection history, blood pressure; presence of nephrotic and/or nephritic syndrome; hemoglobin level, leukocyte count, C-reactive protein (CRP), serum albumin (sAlb), creatinine, complement 3 (sC3), complement 4 (sC4) and immunoglobulin A (sIgA) levels; estimated glomerular filtration rate (eGFR) and proteinuria levels; and renal pathology findings at the onset of disease; total follow-up time; and blood pressure, eGFR and proteinuria levels at the last visit. RESULTS Fifty-four patients were enrolled in the study [38 (70%) HSPN and 16 (30%) IgAN]. The median follow-up time was 60.5 and 72.0 months in HSPN and IgAN groups, respectively (p > 0.05). The HSPN and IgAN groups were also not different in terms of gender, age at the onset; leukocyte count, eGFR, sC3-sC4-sIgA levels; and the presence of endocapillary, extracapillary and mesangial proliferation, tubular atrophy, interstitial fibrosis and IgA, IgM, C3 accumulation in renal tissue. Upper respiratory tract infection history was more common in children with IgAN (8/16 vs 8/38, p = 0.045). sAlb (3.96 ± 0.58 vs 4.40 ± 0.46 g/dL, p = 0.005), hemoglobin (12.1 ± 1.3 vs 13.3 ± 1.2 g/dL, p = 0.004,) and the incidence of mesangial IgG deposition (15/38 vs 11/16, p = 0.049) were lower, while CRP (16.3 ± 7.2 vs 7.8 ± 4.4 mg/L, p = 0.002) and proteinuria (72.1 ± 92.4 vs 34.2 ± 37.9 mg/m2/24 h, p = 0.041) was higher in HSPN group at the onset of disease. Proteinuria and eGFR were similar between the two groups at last visit. CONCLUSION Children with HSPN and IgAN have little clinical and histological differences in our population. The most prominent difference at presentation with nephritis was higher proteinuria in HSPN probably associated with inflammation due to systemic vasculitis. Long-term renal outcome was good in both HSPN and IgAN.
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Affiliation(s)
- Meral Torun Bayram
- Department of Pediatric Nephrology, Medical Faculty, Dokuz Eylül University, Balçova, 35340, Izmir, Turkey.
| | - Cihan Heybeli
- Departments of Nephrology, Dokuz Eylül University Medical Faculty, Izmir, Turkey
| | - Gizem Yıldız
- Department of Pediatric Nephrology, Medical Faculty, Dokuz Eylül University, Balçova, 35340, Izmir, Turkey
| | - Alper Soylu
- Department of Pediatric Nephrology, Medical Faculty, Dokuz Eylül University, Balçova, 35340, Izmir, Turkey
| | - Ali Celik
- Departments of Nephrology, Dokuz Eylül University Medical Faculty, Izmir, Turkey
| | - Sülen Sarioglu
- Department of Pathology, Medical Faculty, Dokuz Eylül University, Izmir, Turkey
| | - Salih Kavukçu
- Department of Pediatric Nephrology, Medical Faculty, Dokuz Eylül University, Balçova, 35340, Izmir, Turkey
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Sood R, Parekh P, Raj N, Saani I. Case Report: An Adult Presentation of Henoch-Schönlein Purpura. Cureus 2022; 14:e26385. [PMID: 35923669 PMCID: PMC9339340 DOI: 10.7759/cureus.26385] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/28/2022] [Indexed: 12/05/2022] Open
Abstract
Henoch-Schönlein purpura (HSP) is an immunoglobulin A (IgA)-mediated multisystem vasculitis commonly affecting children under 10 years of age. Although diagnostic criteria exist, making a diagnosis is often difficult as this condition can present atypically in adults. We discuss a 22-year-old female with a delayed diagnosis of HSP, resulting in significant anxiety and distress. Our patient’s symptoms improved with analgesia and corticosteroids, which were initiated upon diagnosis and she experienced two mild, self-limiting relapses over two years following symptom resolution. Our case illustrates that an integrated multidisciplinary approach is needed to effectively diagnose, safely manage and monitor patients presenting with HSP. Although self-limiting in nature, HSP has the potential to manifest into life-threatening conditions such as end-stage renal failure, which stresses the importance of early diagnosis and management.
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A boy with purpura and generalized edema: Answers. Pediatr Nephrol 2022; 37:1321-1323. [PMID: 35084564 DOI: 10.1007/s00467-021-05408-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/22/2021] [Accepted: 11/24/2021] [Indexed: 10/19/2022]
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Ruan JW, Fan GZ, Niu MM, Jiang Q, Li RX, Qiu Z, Hu P. Serum immunoglobulin profiles in Chinese children with Henoch-Schönlein purpura. Scand J Immunol 2022; 96:e13191. [PMID: 35538715 DOI: 10.1111/sji.13191] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/20/2021] [Revised: 04/28/2022] [Accepted: 05/08/2022] [Indexed: 11/28/2022]
Abstract
OBJECTIVE The present study focuses on the associations of serum immunoglobulin with disease activity, relapse/recurrence and renal involvement in Henoch-Schönlein purpura (HSP) children. METHODS 1683 Chinese children with HSP were recruited from January 2015 to January 2021. Laboratory data of blood samples and urine tests were collected. Renal biopsy was performed by the percutaneous technique. Patients' histories associated with HSP onset were obtained by interviews and questionnaires. RESULTS (1) IgA and IgE were dramatically elevated in HSP patients as compared with their normal values, and subject to a 1.75-3.09 fold and a 1.97-2.61 fold increase, respectively. (2) No significant correlation of the disease activity with IgA and IgE was determined, respectively. (3) The serum IgA levels were significantly lower in patients with relapse/recurrence than that in patients without relapse/recurrence, which may be attributed to the transmission of IgA-mediated immune complexes from blood to vessel walls. (4) No significant correlation was found between serum IgA, IgE levels and the pathological classification. CONCLUSIONS HSP children have marked disorders of serum immunoglobulin profiles, characterized by significant increases in IgA and IgE. The detection of serum IgA may be applicable to predict relapse/recurrence of HSP, whereas not associated with disease activity and renal involvement.
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Affiliation(s)
- Jin Wei Ruan
- Department of Pediatrics, the First Affiliated Hospital of Anhui Medical University, Hefei, PR China.,Department of Pediatrics, the Fourth Affiliated Hospital of Anhui Medical University, Hefei, People's Republic of China
| | - Guo Zhen Fan
- Department of Pediatrics, the First Affiliated Hospital of Anhui Medical University, Hefei, PR China
| | - Man Man Niu
- Department of Pediatrics, the First Affiliated Hospital of Anhui Medical University, Hefei, PR China
| | - Qi Jiang
- Department of Pediatrics, the First Affiliated Hospital of Anhui Medical University, Hefei, PR China
| | - Rui Xue Li
- Department of Pediatrics, the First Affiliated Hospital of Anhui Medical University, Hefei, PR China
| | - Zhen Qiu
- Department of Pediatrics, the First Affiliated Hospital of Anhui Medical University, Hefei, PR China
| | - Peng Hu
- Department of Pediatrics, the First Affiliated Hospital of Anhui Medical University, Hefei, PR China.,Department of Pediatrics, the Fourth Affiliated Hospital of Anhui Medical University, Hefei, People's Republic of China
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Hopp RJ, Niebur HB. Persistent Hyper IgA as a Marker of Immune Deficiency: A Case Report. Antibodies (Basel) 2022; 11:antib11020030. [PMID: 35645203 PMCID: PMC9149891 DOI: 10.3390/antib11020030] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2021] [Revised: 03/28/2022] [Accepted: 04/18/2022] [Indexed: 02/05/2023] Open
Abstract
An elevated IgA level obtained in a 10-year-old male a year after an episode of pneumococcal sepsis led to the discovery of a broad-based IgG-specific antibody deficiency syndrome. The specifics of the case and pertinent literature are presented, including a discussion of the hyper-IgD syndrome. An elevated IgA, greater than two standard deviations above the expected age range should prompt a complete workup for selective antibody deficiency syndrome and adds an additional associated marker of an indolent hyper-IgD syndrome in a different clinical circumstance, although the lack of antibody response to vaccines is atypical of the hyper-IgD syndrome.
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Wallace CE, Sharma A. Adult Onset Immunoglobulin A (IgA) Vasculitis Secondary to Group A Streptococcus Infection. Cureus 2022; 14:e23987. [PMID: 35541301 PMCID: PMC9084608 DOI: 10.7759/cureus.23987] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2022] [Accepted: 03/30/2022] [Indexed: 11/20/2022] Open
Abstract
Immunoglobulin A (IgA) vasculitis is a small blood vessel vasculitis that is mediated by immune complex deposition. While it is the most common cause of childhood vasculitis, the disease is uncommon in adults with variable clinical manifestations. A 65-year-old female presented with a diffuse erythematous, pruritic, painful rash across her legs, back, and arms of 12 days’ duration. Associated symptoms included fatigue, lower extremity swelling, and migratory arthralgias of the knees and ankles. Skin examination revealed edematous, blanchable, erythematous, annular papules and plaques on the legs, back, and arms with pitting edema of the lower legs. Laboratory testing revealed an elevated erythrocyte sedimentation rate, hypoalbuminemia, proteinuria, hematuria, and a positive antistreptolysin O titer, indicative of recent group A Streptococcus infection. Treatment with systemic corticosteroids led to a resolution of all her symptoms. Adult onset IgA vasculitis differs in clinical manifestation and treatment from that of the pediatric population. This case demonstrates the importance of considering IgA vasculitis as a differential diagnosis in adults presenting with small vessel vasculitis.
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Formeck CL, Manrique-Caballero CL, Gómez H, Kellum JA. Uncommon Causes of Acute Kidney Injury. Crit Care Clin 2022; 38:317-347. [DOI: 10.1016/j.ccc.2021.11.010] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
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SÜRÜCÜ KARA İ, ÖZDAMAR M, KOLKIRAN A, AYDIN PEKER N, TOPAL İ. Intussusepsiyon Henoch-Schonlein purpurası'nın ilk tezahürü olabilir mi? CUKUROVA MEDICAL JOURNAL 2022. [DOI: 10.17826/cumj.1029691] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/07/2022] Open
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Jin K, Parreau S, Warrington KJ, Koster MJ, Berry GJ, Goronzy JJ, Weyand CM. Regulatory T Cells in Autoimmune Vasculitis. Front Immunol 2022; 13:844300. [PMID: 35296082 PMCID: PMC8918523 DOI: 10.3389/fimmu.2022.844300] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2021] [Accepted: 01/28/2022] [Indexed: 12/14/2022] Open
Abstract
Blood vessels are indispensable for host survival and are protected from inappropriate inflammation by immune privilege. This protection is lost in patients with autoimmune vasculitides, a heterogeneous group of diseases causing damage to arteries, arterioles, and capillaries. Vasculitis leads to vascular wall destruction and/or luminal occlusion, resulting in hemorrhage and tissue ischemia. Failure in the quantity and quality of immunosuppressive regulatory T cells (Treg) has been implicated in the breakdown of the vascular immune privilege. Emerging data suggest that Treg deficiencies are disease-specific, affecting distinct pathways in distinct vasculitides. Mechanistic studies have identified faulty CD8+ Tregs in Giant Cell Arteritis (GCA), a vasculitis of the aorta and the large aortic branch vessels. Specifically, aberrant signaling through the NOTCH4 receptor expressed on CD8+ Treg cells leads to rerouting of intracellular vesicle trafficking and failure in the release of immunosuppressive exosomes, ultimately boosting inflammatory attack to medium and large arteries. In Kawasaki’s disease, a medium vessel vasculitis targeting the coronary arteries, aberrant expression of miR-155 and dysregulated STAT5 signaling have been implicated in undermining CD4+ Treg function. Explorations of mechanisms leading to insufficient immunosuppression and uncontrolled vascular inflammation hold the promise to discover novel therapeutic interventions that could potentially restore the immune privilege of blood vessels and pave the way for urgently needed innovations in vasculitis management.
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Affiliation(s)
- Ke Jin
- Department of Medicine, Mayo College of Medicine and Science, Rochester, MN, United States
| | - Simon Parreau
- Department of Medicine, Mayo College of Medicine and Science, Rochester, MN, United States
| | - Kenneth J. Warrington
- Department of Medicine, Mayo College of Medicine and Science, Rochester, MN, United States
| | - Matthew J. Koster
- Department of Medicine, Mayo College of Medicine and Science, Rochester, MN, United States
| | - Gerald J. Berry
- Department of Pathology, Stanford University School of Medicine, Stanford, CA, United States
| | - Jörg J. Goronzy
- Department of Medicine, Mayo College of Medicine and Science, Rochester, MN, United States
- Department of Medicine, Stanford University School of Medicine, Stanford, CA, United States
| | - Cornelia M. Weyand
- Department of Medicine, Mayo College of Medicine and Science, Rochester, MN, United States
- Department of Medicine, Stanford University School of Medicine, Stanford, CA, United States
- *Correspondence: Cornelia M. Weyand,
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Farooq H, Aemaz Ur Rehman M, Asmar A, Asif S, Mushtaq A, Qureshi MA. The pathogenesis of COVID-19-induced IgA nephropathy and IgA vasculitis: A systematic review. J Taibah Univ Med Sci 2022; 17:1-13. [PMID: 34602936 PMCID: PMC8479423 DOI: 10.1016/j.jtumed.2021.08.012] [Citation(s) in RCA: 21] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2021] [Revised: 08/19/2021] [Accepted: 08/28/2021] [Indexed: 12/21/2022] Open
Abstract
OBJECTIVE IgA nephropathy (IgAN) and IgA vasculitis (IgAV) are part of a similar clinical spectrum. Both clinical conditions occur with the coronavirus disease 2019 (COVID-19). This review aims to recognize the novel association of IgAN and IgAV with COVID-19 and describe its underlying pathogenesis. METHODS We conducted a systematic literature search and data extraction from PubMed, Cochrane, ScienceDirect, and Google Scholar following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. RESULTS Our search identified 13 cases reporting IgAV and IgAN associated with COVID-19 infection and 4 cases of IgAN following COVID-19 vaccination. The mean, mode, and median ages of patients were 23.8, 4, and 8 years, respectively. Most cases associated with COVID-19 infection were reported in males (77%). Rash and purpura (85%) were the most common clinical features, followed by gastrointestinal symptoms (62%). In symptomatic cases, skin or renal biopsy and immunofluorescence confirmed the diagnosis of IgAN or IgAV. Most patients were treated with steroids and reported recovery or improvement; however, death was reported in two patients. CONCLUSION There is a paucity of scientific evidence on the pathogenesis of the association of IgAN and IgAV with COVID-19, which thus needs further study. Current research suggests the role of IgA-mediated immune response, evidenced by early seroconversion to IgA in COVID-19 patients and the role of IgA in immune hyperactivation as the predominant mediator of the disease process. Clinicians, especially nephrologists and paediatricians, need to recognize this association, as this disease is usually self-limited and can lead to complete recovery if prompt diagnosis and treatment are provided.
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Affiliation(s)
| | | | - Abyaz Asmar
- Department of Medicine, Mayo Hospital, King Edward Medical University, Lahore, Pakistan
| | - Salman Asif
- Department of Medicine, Mayo Hospital, King Edward Medical University, Lahore, Pakistan
| | - Aliza Mushtaq
- Department of Medicine, Mayo Hospital, King Edward Medical University, Lahore, Pakistan
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Liu C, Luo L, Fu M, Li Z, Liu J. Analysis of children with Henoch-Schonlein purpura secondary to infection. Clin Rheumatol 2022; 41:803-810. [PMID: 34993728 DOI: 10.1007/s10067-021-06007-9] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2021] [Revised: 11/26/2021] [Accepted: 11/26/2021] [Indexed: 12/27/2022]
Abstract
OBJECTIVES Henoch-Schonlein purpura (HSP) is the most common childhood vasculitis, infection is the most essential inducement. We hypothesized that infection could impact the blood routine characteristics and/or outcome of vasculitis. Thus, we aim to find the most common infectious agent in HSP patients and identify convenient indicators to predict renal involvement in HSP patients with infection. METHOD We conducted a retrospective study of 208 HSP children and 98 healthy children. Clinical parameters were compared in those cases. RESULTS A total of 68.75% of patients were infected with various pathogens. The mean platelet volume (MPV) (P < 0.02) was lower in HSP patients with infection than patients without infection. Mycoplasma pneumoniae (MP) infection accounted for the largest proportion (45.77%). MPV in HSP nephritis (HSPN) group was lower than in HSP patients (excluded renal involvement) in patients with MP infection. Logistic regression analysis found that age and MPV were risk factors for the occurrence of MP-infected HSPN. The receiver operating characteristic curve (ROC) analysis showed that the combination of MPV with the onset age at the optimal cut-off point had 81% sensitivity in predicting whether HSP patients with MP infection would develop into HSPN. CONCLUSIONS Our research revealed that MP was the most commonly infected pathogen of children's HSP. MPV was an essential predictor of nephritis in HSP patients with MP infection. This discovery can prompt clinical treatments as well as reduce costs. Key Points • Mycoplasma pneumoniae (MP) accounts for the largest proportion in HSP children with infection. • MPV can be used as a predictor for the development of MP-triggered HSP to HSPN.
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Affiliation(s)
- Can Liu
- Clinical Laboratory, Hunan Children's Hospital, Changsha, 410007, China.
| | - Lingli Luo
- Clinical Laboratory, Hunan Children's Hospital, Changsha, 410007, China
| | - Min Fu
- Clinical Laboratory, Hunan Children's Hospital, Changsha, 410007, China
| | - Zhengqiu Li
- Clinical Laboratory, Hunan Children's Hospital, Changsha, 410007, China
| | - Jianlong Liu
- Clinical Laboratory, Hunan Children's Hospital, Changsha, 410007, China
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50
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Stone JR. Diseases of small and medium-sized blood vessels. Cardiovasc Pathol 2022. [DOI: 10.1016/b978-0-12-822224-9.00020-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/17/2022] Open
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