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Polat MC, Ekici Tekin Z, Keçeli AM, Çelikel E, Güngörer V, Karagöl C, Kaplan MM, Öner N, Öztürk D, Özçelik E, Işıklar Ekici M, Uğur Es Y, Yoğun SN, Çelikel Acar B. Can the affected intestinal segment visualized on abdominal ultrasound predict gastrointestinal tract bleeding in immunoglobulin A vasculitis? Mod Rheumatol 2025; 35:516-523. [PMID: 39539230 DOI: 10.1093/mr/roae103] [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: 09/10/2024] [Revised: 10/08/2024] [Accepted: 11/03/2024] [Indexed: 11/16/2024]
Abstract
OBJECTIVES The study aimed to evaluate immunoglobulin A vasculitis (IgAV) patients with gastrointestinal (GI) tract involvement and to reveal the relationship between the location and extent of the affected intestinal segment detected on the initial abdominal ultrasound and GI tract bleeding. METHODS This medical record review study was conducted on 117 IgAV patients with GI tract involvement between January 2016 and June 2023. Patients were divided into two groups: those with (n = 28) and without (n = 89) GI tract bleeding. Predictors of GI tract bleeding were investigated by comparing demographic, clinical characteristics, and laboratory findings. RESULTS Gender, age at diagnosis, symptoms at admission, rash distribution, GI tract complaints, and the elapsed time until the development of GI tract symptoms were similar in both groups. There was no difference between small intestinal, large intestinal, or small + large intestinal involvement (P = .89). The ileum was the most commonly affected intestinal segment in patients with and without GI tract bleeding (P = .37). Jejunal wall thickening (P = .04) and the number of affected intestinal segments (P = .008) were higher in patients with GI tract bleeding. CONCLUSIONS In IgAV patients, jejunum involvement and affected multiple intestinal segments shown by abdominal ultrasound are associated with GI tract bleeding.
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Affiliation(s)
- Merve Cansu Polat
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Zahide Ekici Tekin
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Avni Merter Keçeli
- Division of Pediatric Radiology, Department of Radiology, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Elif Çelikel
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Vildan Güngörer
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Cüneyt Karagöl
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Melike Mehveş Kaplan
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Nimet Öner
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Didem Öztürk
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Emine Özçelik
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Mehveş Işıklar Ekici
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Yasemin Uğur Es
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Sultan Nilay Yoğun
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
| | - Banu Çelikel Acar
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Bilkent, Ankara, Turkey
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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. 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] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [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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Chang LS, Nishida T, Nakamatsu D, Matsumoto K, Yamamoto M, Adachi S, Fukui K. Therapeutic success of factor XIII substitution for IgA vasculitis with gastrointestinal manifestation. Clin J Gastroenterol 2025:10.1007/s12328-025-02112-3. [PMID: 40117116 DOI: 10.1007/s12328-025-02112-3] [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: 12/28/2024] [Accepted: 03/03/2025] [Indexed: 03/23/2025]
Abstract
A 52-year-old man presented with severe abdominal pain, elevated C-reactive protein (CRP) levels, and characteristic skin findings, leading to a diagnosis of immunoglobulin A (IgA) vasculitis with gastrointestinal (GI) involvement. Initial evaluation, including contrast-enhanced computed tomography (CT) and esophagogastroduodenoscopy (EGD), revealed marked inflammation of the duodenum and a punched-out ulcer, both of which showed partial improvement with conservative treatment. However, the patient developed worsening abdominal pain, arthralgia, and purpura, accompanied by reduced plasma factor XIII activity (47%). Factor XIII substitution therapy was initiated as a monotherapy, resulting in immediate symptom relief and significant endoscopic improvement of the duodenal ulcer. This case highlights the potential of factor XIII monotherapy as an effective treatment option for adult IgA vasculitis with severe GI manifestations.
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Affiliation(s)
- Li-Sa Chang
- Department of Gastroenterology, Toyonaka Municipal Hospital, 4-14-1 Shibahara, Toyonaka, Osaka, 560-8565, Japan
- Department of Gastroenterology and Hepatology, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan
| | - Tsutomu Nishida
- Department of Gastroenterology, Toyonaka Municipal Hospital, 4-14-1 Shibahara, Toyonaka, Osaka, 560-8565, Japan.
| | - Dai Nakamatsu
- Department of Gastroenterology, Toyonaka Municipal Hospital, 4-14-1 Shibahara, Toyonaka, Osaka, 560-8565, Japan
| | - Kengo Matsumoto
- Department of Gastroenterology, Toyonaka Municipal Hospital, 4-14-1 Shibahara, Toyonaka, Osaka, 560-8565, Japan
| | - Masashi Yamamoto
- Department of Gastroenterology, Toyonaka Municipal Hospital, 4-14-1 Shibahara, Toyonaka, Osaka, 560-8565, Japan
| | - Shiro Adachi
- Department of Pathology, Toyonaka Municipal Hospital, Toyonaka, Osaka, 560-8565, Japan
| | - Koji Fukui
- Department of Gastroenterology, Toyonaka Municipal Hospital, 4-14-1 Shibahara, Toyonaka, Osaka, 560-8565, Japan
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Chen L, Zhong C, Fan L, Luo M, Cai L, Zhang B, Zhang H. Effective treatment with intravenous immunoglobulin for Henoch-Schönlein purpura with refractory gastrointestinal symptoms in an adolescent: A CARE-compliant case report. Medicine (Baltimore) 2024; 103:e40370. [PMID: 39495971 PMCID: PMC11537644 DOI: 10.1097/md.0000000000040370] [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: 07/02/2024] [Accepted: 10/16/2024] [Indexed: 11/06/2024] Open
Abstract
RATIONALE This article presents a complex case of refractory Henoch-Schönlein purpura (HSP), initially manifesting with complex gastrointestinal (GI) symptoms, and discusses diagnostic and therapeutic challenges encountered. It aims to enhance understanding of the disease and provide evidence for the potential efficacy of intravenous immunoglobulin (IVIG) treatment in this condition. PATIENT CONCERNS A 16-year-old male patient presented with persistent abdominal pain, nausea, vomiting, and constipation for 8 days, leading to hospital admission. DIAGNOSES Establishing a definitive diagnosis was challenging initially due to the absence of typical petechiae. However, the appearance of characteristic petechiae subsequently confirmed the diagnosis of HSP. INTERVENTIONS Initial treatment with methylprednisolone sodium succinate for 3 days failed to elicit improvement. Subsequently, IVIG was introduced as a combination therapy. OUTCOMES Following the combined administration of IVIG, the patient experienced complete resolution of abdominal pain, petechiae, and arthralgia within 4 days. LESSONS This case highlights the importance of considering HSP in the differential diagnosis of patients with complex GI symptoms. Furthermore, it suggests that IVIG may be a valuable therapeutic option for HSP patients with refractory GI symptoms. High-quality comparative trials are needed to establish more definitive evidence for the effectiveness of IVIG and to develop specific treatment guidelines.
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Affiliation(s)
- Liji Chen
- The Second Clinical Medical College of Guangzhou University of Traditional Chinese Medicine, Guangzhou, China
| | - Cailing Zhong
- Department of Gastroenterology, The Second Affiliated Hospital of Guangzhou University of Traditional Chinese Medicine, Guangzhou, China
| | - Longxiu Fan
- The Second Clinical Medical College of Guangzhou University of Traditional Chinese Medicine, Guangzhou, China
| | - Ming Luo
- Department of Gastroenterology, The Second Affiliated Hospital of Guangzhou University of Traditional Chinese Medicine, Guangzhou, China
| | - Linkun Cai
- Department of Gastroenterology, The Second Affiliated Hospital of Guangzhou University of Traditional Chinese Medicine, Guangzhou, China
| | - Beiping Zhang
- Department of Gastroenterology, The Second Affiliated Hospital of Guangzhou University of Traditional Chinese Medicine, Guangzhou, China
| | - Haiyan Zhang
- Department of Gastroenterology, The Second Affiliated Hospital of Guangzhou University of Traditional Chinese Medicine, Guangzhou, China
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Kato S, Gold BD, Kato A. Gastrointestinal manifestations and pathogenesis in childhood immunoglobulin A vasculitis. Front Pediatr 2024; 12:1459394. [PMID: 39497734 PMCID: PMC11532042 DOI: 10.3389/fped.2024.1459394] [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: 07/04/2024] [Accepted: 10/02/2024] [Indexed: 11/07/2024] Open
Abstract
Immunoglobulin A vasculitis (IgAV), previously known as Henoch-Schönlein purpura, is the most common form of systemic vasculitis in childhood. The primary organs involved are the skin, gastrointestinal (GI) tract, joints, and kidneys. The spectrum of GI involvement in IgAV ranges from being mild and self-limited to severe manifestations often requiring surgical intervention. Galactose-deficient IgA1 on the immunoglobulin hinge region and its immune complexes are thought to play a central pathogenetic role in IgAV, however, an association between such molecules and specific GI mucosal damage remains unclear. GI endoscopy (both upper and lower) shows a variety of mucosal findings, many of which are not specific for IgAV. In upper GI endoscopy, however, the mucosal features can be diagnostic when found localized in the more distal part of upper GI tract (second and/or third parts of the duodenum). Abdominal computed tomography and capsule endoscopy have demonstrated that the small intestine is most commonly involved in IgAV. The GI mucosal involvement when evaluated microscopically shows IgA deposition which is histologically diagnostic. Conversely, leukocytoclastic vasculitis is less useful. Since the 1960s, cases of duodenojejunitis, in which IgAV was suspected but evident purpura was not dermatologically present, have often been labeled as "idiopathic". In a pediatric case series, IgA enteropathy, without dermatological manifestations (i.e., purpura), was reported to have similar symptoms, as well as endoscopic characteristics and immunohistological findings as in IgAV. Subsequently, several case reports provide additional supportive evidence that IgA enteropathy must be a variant of IgAV. Thus, the immunologically driven auto-immune vasculitis results in the symptom complex dependent on the organ system involved, and the subsequent clinical features which are manifested. Present classification criteria are useful and universally available for diagnosing IgAV. However, based upon current knowledge including IgA enteropathy, minor modification of the IgAV criteria is proposed in the review.
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Affiliation(s)
| | - Benjamin D. Gold
- GI Care for Kids, Children’s Center for Digestive Healthcare, LLC, Atlanta, GA, United States
| | - Ayumu Kato
- Department of Pediatrics, Sendai City Hospital, Sendai, Japan
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Yeh PJ, Chen CC, Chao HC, Lai JY, Ming YC, Chen MC, Lai MW. The trends of pediatric duodenal ulcer and predictors of recurrence. J Formos Med Assoc 2024; 123:1070-1077. [PMID: 38644127 DOI: 10.1016/j.jfma.2024.04.011] [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: 09/05/2023] [Revised: 02/27/2024] [Accepted: 04/16/2024] [Indexed: 04/23/2024] Open
Abstract
BACKGROUND Duodenal ulcer (DU) causes various symptoms in children. The prevalence of Helicobacter pylori (Hp)-associated DU has been reducing in some regions, yet the updated trend in Taiwan is unknown. Risk factors of DU recurrence have not been comprehensively investigated in children. METHODS This retrospective study included children diagnosed with DU to evaluate the demographics, symptoms, diagnostics, treatment, and outcomes. Specific populations (infant, surgery required) were sorted for subgroup analysis. Predictors of DU recurrence was analyzed in patients who received endoscopic follow-ups. RESULTS A total of 488 children were included. Most patients were male (72.5%), school-aged (11.3 ± 4.8 years old), and with varied underlying diseases in one-fifth. The annual incidences were around 3-5%, with a declining trend of case numbers and the Hp-positive proportion. Hp infection, concurrent gastric ulcer, perforation, and mortality were noted in 32.7%, 16%, 1.6%, and 1% of patients. Patients with or without Hp infection showed different clinical features but similar outcomes. The characteristics of subpopulations were depicted respectively. Male sex, lower Hb level, and perforation were independent risk factors associated with recurrence. CONCLUSION Hp-positive DU seems to wane. Patients with male sex, lower Hb level, or perforation at diagnosis carried a higher risk of recurrence, which may warrant active surveillance and endoscopic follow-up.
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Affiliation(s)
- Pai-Jui Yeh
- Division of Pediatric Gastroenterology, Department of Pediatrics, Chang Gung Memorial Hospital, Linkou Branch and Chang Gung University College of Medicine, Taoyuan, Taiwan; Liver Research Center, Chang Gung Memorial Hospital, Linkou Branch, Taiwan
| | - Chien-Chang Chen
- Division of Pediatric Gastroenterology, Department of Pediatrics, Chang Gung Memorial Hospital, Linkou Branch and Chang Gung University College of Medicine, Taoyuan, Taiwan
| | - Hsun-Chin Chao
- Division of Pediatric Gastroenterology, Department of Pediatrics, Chang Gung Memorial Hospital, Linkou Branch and Chang Gung University College of Medicine, Taoyuan, Taiwan
| | - Jin-Yao Lai
- Department of Pediatric Surgery, Chang Gung Memorial Hospital, Linkou Branch and Chang Gung University College of Medicine, Taoyuan, Taiwan
| | - Yung-Ching Ming
- Department of Pediatric Surgery, Chang Gung Memorial Hospital, Linkou Branch and Chang Gung University College of Medicine, Taoyuan, Taiwan
| | - Mi-Chi Chen
- Division of Pediatric Gastroenterology, Department of Pediatrics, Chang Gung Memorial Hospital, Linkou Branch and Chang Gung University College of Medicine, Taoyuan, Taiwan; Liver Research Center, Chang Gung Memorial Hospital, Linkou Branch, Taiwan
| | - Ming-Wei Lai
- Division of Pediatric Gastroenterology, Department of Pediatrics, Chang Gung Memorial Hospital, Linkou Branch and Chang Gung University College of Medicine, Taoyuan, Taiwan; Liver Research Center, Chang Gung Memorial Hospital, Linkou Branch, Taiwan.
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Boissais A, Blasco H, Emond P, Lefèvre A, Bigot A, Ramdani Y, Maldent NF, Mulleman D, Pillebout E, Maillot F, Audemard-Verger A. A specific diagnostic metabolome signature in adult IgA vasculitis. Metabolomics 2024; 20:61. [PMID: 38787468 PMCID: PMC11126474 DOI: 10.1007/s11306-024-02107-0] [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: 04/30/2023] [Accepted: 02/28/2024] [Indexed: 05/25/2024]
Abstract
INTRODUCTION IgA vasculitis diagnosis relies primarily on clinical features and is confirmed by pathological findings. To date, there is no reliable noninvasive diagnostic biomarker. OBJECTIVE We aimed to explore the baseline serum metabolome of adult patients with IgA vasculitis to identify potential diagnostic biomarkers. METHODS We performed a study comparing the serum metabolome of patients with IgA vasculitis to that of patients with inflammatory condition, namely spondyloarthritis. Serum analyses were performed by high-performance liquid chromatography-mass spectrometry. RESULTS Fifty-five patients with IgA vasculitis and 77 controls with spondyloarthritis (age- and sex-matched) were included in this study. The median age of IgA vasculitis patients was 53 years. Two-thirds of patients were female (n = 32). At the time of vasculitis diagnosis, 100% of patients had skin involvement and 69% presented with glomerulonephritis (n = 38). Joint and digestive involvement were observed in 56% (n = 31) and 42% (n = 23) of patients. Four discriminative metabolites between the two groups were identified: 1-methyladenosine, L-glutamic acid, serotonin, and thymidine. The multivariate model built from the serum metabolomes of patients with IgA vasculitis and spondyloarthritis revealed an accuracy > 90%. As this model was significant according to the permutation test (p < 0.01), independent validation showed an excellent predictive value of the test set: sensitivity 98%; specificity 98%, positive predictive value 97% and negative predictive value 98%. CONCLUSION To our knowledge, this study is the first to use the metabolomic approach for diagnostic purposes in adult IgA vasculitis, highlighting a specific diagnostic metabolome signature.
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Affiliation(s)
- Alexandre Boissais
- Department of Internal Medicine and Clinical Immunology, University Hospital Center of Tours, Tours, France.
| | - Hélène Blasco
- Biochemistry and Molecular Biology Department, University Hospital Center of Tours, Tours, France
- UMR 1253, iBrain, University of Tours, 37000, InsermTours, France
| | - Patrick Emond
- UMR 1253, iBrain, University of Tours, 37000, InsermTours, France
- In Vitro Nuclear Medicine Department, University Hospital Center of Tours, Tours, France
| | - Antoine Lefèvre
- UMR 1253, iBrain, University of Tours, 37000, InsermTours, France
| | - Adrien Bigot
- Department of Internal Medicine and Clinical Immunology, University Hospital Center of Tours, Tours, France
| | - Yanis Ramdani
- Department of Internal Medicine and Clinical Immunology, University Hospital Center of Tours, Tours, France
| | - Nicole Ferreira Maldent
- Department of Internal Medicine and Clinical Immunology, University Hospital Center of Tours, Tours, France
| | - Denis Mulleman
- Center for Molecular Biophysics, UPR CNRS 4301, Tours, France
- Department of Rheumatology, University Hospital Center of Tours, Tours, France
- Nanomedicines and Nanoprobes Department, University of Tours, Tours, France
| | | | - François Maillot
- Department of Internal Medicine and Clinical Immunology, University Hospital Center of Tours, Tours, France
- UMR 1253, iBrain, University of Tours, 37000, InsermTours, France
| | - Alexandra Audemard-Verger
- Department of Internal Medicine and Clinical Immunology, University Hospital Center of Tours, Tours, France
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Sandhu K, Mcdonald K, Langmead L, Deb P. Terminal ileitis: a rare gastrointestinal manifestation of IgA vasculitis in a child. BMJ Case Rep 2024; 17:e240990. [PMID: 38697686 PMCID: PMC11085876 DOI: 10.1136/bcr-2020-240990] [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] [Indexed: 05/05/2024] Open
Abstract
A girl in middle childhood was referred to the paediatric surgical team with acute colicky abdominal pain and bile-stained vomiting. This was preceded by a viral illness. Investigations revealed raised inflammatory markers, and imaging of the abdomen demonstrated ileal and jejunal thickening. Concerns were raised regarding whether she had inflammatory bowel disease. Endoscopy revealed gastritis and duodenitis, and colonoscopy was unremarkable. Video capsule endoscopy demonstrated ulcers in the jejunum and ileum.On day 8 of admission, she developed a symmetrical purpuric rash over both ankles leading to the diagnosis of Henoch-Schonlein-related ileitis. Multidisciplinary team working led to appropriate management of the patient and avoided surgery. Video capsule endoscopy enabled visualisation of the small bowel. She was managed with 5 days of methylprednisolone followed by oral steroids. She made a good recovery with no sequelae. This case highlighted that terminal ileitis is a rare complication of IgA vasculitis with a good prognosis.
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Affiliation(s)
- Kirn Sandhu
- Paediatric Gastroenterology, Barts Health NHS Trust, London, UK
| | | | | | - Protima Deb
- Paediatric Gastroenterology, Barts Health NHS Trust, London, UK
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9
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Adler S. [Immunoglobulin A vasculitis]. INNERE MEDIZIN (HEIDELBERG, GERMANY) 2024; 65:114-121. [PMID: 38236411 DOI: 10.1007/s00108-023-01650-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Accepted: 12/14/2023] [Indexed: 01/19/2024]
Abstract
The immune-mediated small vessel vasculitis is known as Schoenlein-Henoch purpura predominantly from pediatrics and in these cases occurs more frequently after infections of the upper airways. In adults, immunoglobulin A (IgA) vasculitis often proceeds more severely und recurrently with the classical tetrad of skin manifestations in the sense of leukocytoclastic vasculitis, joint affection, gastrointestinal involvement and IgA nephritis, in contrast to the mostly mild and self-limiting course in children. The background of this systemic vasculitis with formation of IgA immune complexes is considered to be an altered glycosylation of IgA, as this causes the exposure of binding sites for autoantibodies so that an immune complex reaction can be elicited. This ultimately leads to perivascular deposition of IgA and a further activation of neutrophils. Groundbreaking in the diagnostics is the histological detection of leukocytoclastic vasculitis and in cases of renal manifestations a kidney biopsy with characteristic deposits of immune complexes, which cannot be clearly differentiated from IgA nephropathy. The treatment is aimed at the respective manifestation and is mostly based on consensus recommendations due to the lack of randomized studies. In addition to immunosuppressive medication, in the presence of a chronic kidney disease general nephroprotection is becoming increasingly more important also by inhibition of sodium-glucose transporter 2 (SGLT2). The type and extent of kidney involvement and also rare cardiac manifestations are the main determinants of the prognosis. Continuous medical accompaniment of those affected is necessary due to the possible progression of the disease and the risk of recurrence.
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Affiliation(s)
- Sabine Adler
- Rheumatologie und Immunologie, Kantonsspital Aarau, Tellstraße 25, 5001, Aarau, Schweiz.
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Mu J. Clinical characteristics and risk factors of IgA vasculitis with intussusception and intestinal perforation. J Paediatr Child Health 2024; 60:5-11. [PMID: 37942803 DOI: 10.1111/jpc.16516] [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: 05/31/2023] [Revised: 10/20/2023] [Accepted: 10/27/2023] [Indexed: 11/10/2023]
Abstract
AIM Intussusception is the most common surgical complication of IgA vasculitis (IgAV), and intestinal perforation is usually associated with intussusception. If they are not recognised and treated in a timely manner, serious consequences may occur. The objective is to summarise the clinical features of IgAV complicated by intussusception and intestinal perforation and explore its risk factors. METHODS The clinical data of 32 patients with IgAV complicated by surgical complications (25 cases of intussusception and 7 cases of intestinal perforation) were retrospectively analysed. A total of 160 IgAV children with gastrointestinal (GI) involvement but without surgical complications were randomly selected as a control group. Binary logistic regression analysis was performed to explore the risk factors for IgAV with intussusception and intestinal perforation. RESULTS Compared with the intussusception group, the intestinal perforation group had a significantly higher number of patients with GI symptoms prior to skin purpura and GI bleeding, a significantly higher number of days of abdominal pain, a significantly higher Wong-Baker score, and a significantly higher white blood cell count. Multivariate logistic regression analysis indicated that age ≤7 years, GI symptoms prior to skin purpura, abdominal pain intensity (Wong-Baker scale) and timing of glucocorticoid treatment were independent risk factors of IgAV with intussusception and intestinal perforation. CONCLUSION Age less than 7 years, severe abdominal pain, and GI symptoms prior to skin purpura were risk factors for IgAV with intussusception and intestinal perforation. Early use of glucocorticoids may prevent intussusception and intestinal perforation.
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Affiliation(s)
- Jing Mu
- Department of Traditional Chinese Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
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11
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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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12
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Du L, Liu C, Wang X, Mu J, Yang Y. Acute pancreatitis associated with immunoglobulin A vasculitis: report of fifteen cases. Clin Rheumatol 2023; 42:839-847. [PMID: 36197648 DOI: 10.1007/s10067-022-06398-3] [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: 04/13/2022] [Revised: 09/02/2022] [Accepted: 09/27/2022] [Indexed: 11/29/2022]
Abstract
OBJECTIVE Immunoglobulin A vasculitis (IgAV) is a systemic small vessel vasculitis common in children. Pancreatic involvement in IgAV is rare. The purpose of this study was to analyze the clinical characteristics of IgAV-related acute pancreatitis in children. METHODS Records of patients with IgAV-related acute pancreatitis admitted to our institution between January 2016 and December 2019 were reviewed. We summarized the clinical characteristics, laboratory characteristics, imaging findings, treatment, and outcomes of 15 children with IgAV-related acute pancreatitis. RESULTS The patients' median age was 9.6 years. Pancreatitis was the initial manifestation of IgAV in 3 patients. All patients had abdominal manifestations, including abdominal pain (15/15), vomiting (10/15), and gastrointestinal bleeding (7/15). Serum amylase and lipase levels were elevated in all patients. Serum amylase in 4 cases reached more than three-fold elevation and serum lipase in 14 cases reached more than three-fold elevation. Morphological abnormalities and abnormal signals of the pancreas were observed in 13 cases by magnetic resonance imaging. Pancreaticobiliary maljunction was seen in 3 cases by magnetic resonance cholangiopancreatography. Glucocorticoid therapy and intravenous immunoglobulins were used to treat acute pancreatitis in IgAV. All patients showed clinical improvement after treatment. During the follow-up period of 6-12 months, all 15 cases with pancreatitis were cured without recurrence. CONCLUSIONS Pancreatic involvement is rare in IgAV; however, this should be considered in IgAV patients with severe abdominal pain. The timely application of steroid therapy is important for IgAV-associated pancreatitis. Key Points • Acute pancreatitis is a rare complication of immunoglobulin A vasculitis (IgAV). • Acute pancreatitis can be the initial manifestation of IgAV. It is important to evaluate for pancreatitis while IgAV patients with severe abdominal pain. • A common image finding of IgAV-associated pancreatitis was swelling of the pancreas. • Glucocorticoid therapy and intravenous immunoglobulins is helpful in alleviating acute pancreatitis in IgAV.
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Affiliation(s)
- Lina Du
- Department of Chinese Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China
| | - Chang Liu
- Department of Chinese Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China
| | - Xintai Wang
- Department of Chinese Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China
| | - Jing Mu
- Department of Chinese Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China
| | - Yan Yang
- Department of Chinese Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China.
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13
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IgA vasculitis as an immune-related adverse event of durvalumab: A case report. Respir Investig 2023; 61:205-209. [PMID: 36773508 DOI: 10.1016/j.resinv.2023.01.005] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/31/2022] [Revised: 12/28/2022] [Accepted: 01/04/2023] [Indexed: 02/11/2023]
Abstract
A 78-year-old man with lung cancer underwent concurrent chemoradiotherapy followed by durvalumab for 24 cycles. After 6 months, he presented with anorexia and palpable purpura of the lower extremities, with increased proteinuria, hematuria, and elevated creatinine levels. Skin and kidney biopsies suggested a diagnosis of IgA vasculitis. No evidence of cancer progression was found; moreover, no infection or drug could be identified as the cause. Therefore, he was diagnosed with IgA vasculitis as an immune-related adverse event (irAE) caused by durvalumab. Because immune checkpoint inhibitors can cause vasculitis, clinicians should be cautious during their administration and after their discontinuation.
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14
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Acar BÇ, Tekin ZE, Güngörer V, Çelikel E, Kurt T, Tekgöz N, Sezer M, Karagöl C, Coşkun S, Kaplan MM, Polat MC, Öner N, Kuruç AI, Erten EE, Şenel E. Intussusception as a rare manifestation of immunoglobulin A vasculitis: Risk factors and treatment. J Paediatr Child Health 2023; 59:505-511. [PMID: 36692146 DOI: 10.1111/jpc.16335] [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: 11/15/2022] [Revised: 01/10/2023] [Accepted: 01/11/2023] [Indexed: 01/25/2023]
Abstract
AIM To identify the risk factors associated with intussusception in children with immunoglobulin A vasculitis (IgAV)-gastrointestinal (GI) tract involvement and to evaluate the outcomes of medical treatment and surgical intervention and the course of patients with intussusception. METHODS This retrospective study was conducted in 157 patients under 18 years of age who were followed up with the diagnosis of IgAV-GI tract involvement between January 2015 and September 2022. The characteristics of the patients who developed intussusception were evaluated in detail. RESULTS One hundred and fifty-seven patients with GI tract involvement were included in the study. The mean age of patients with IgAV-GI tract involvement was 8.7 ± 3.7 years. The female-to-male ratio was 1:1.5. Intussusception was detected in 14 patients (8.9%). Two patients (14.3%) underwent surgery, and the remaining 12 patients (85.7%) had their medical therapy intensified. Patients with GI tract involvement were divided into two groups as with (n = 14) and without (n = 143) intussusception. There was a statistically significant difference between the groups in the time from the onset of the first symptom of IgAV to the onset of steroids (P = 0.001). There were no statistically significant differences between the groups in age at onset of IgAV, gender distribution, erythrocyte sedimentation rate and C-reactive protein levels. CONCLUSIONS The time from the onset of the first symptom of IgAV to the start of steroids is a risk factor for the development of intussusception in patients with IgAV-GI tract involvement. In these patients, medical treatment usually reduces intussusception without the need for surgical intervention.
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Affiliation(s)
- Banu Ç Acar
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Zahide E Tekin
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Vildan Güngörer
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Elif Çelikel
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Tuba Kurt
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Nilüfer Tekgöz
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Müge Sezer
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Cüneyt Karagöl
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Serkan Coşkun
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Melike M Kaplan
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Merve C Polat
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Nimet Öner
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Aylin I Kuruç
- Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Elif E Erten
- Department of Pediatric Surgery, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Emrah Şenel
- Department of Pediatric Surgery, Ankara Yıldırım Beyazıt University, Ankara Bilkent City Hospital, Ankara, Turkey
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15
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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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16
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Leiomyoma as a lead point for small bowel intussusception in a 16-year-old. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2022. [DOI: 10.1016/j.epsc.2022.102262] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022] Open
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17
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Galam P, Mundakal JK. Chylous ascites in a case of henoch-schonlein purpura - A case report and literature review. Ann Afr Med 2022; 21:305-308. [PMID: 36412327 PMCID: PMC9850884 DOI: 10.4103/aam.aam_168_21] [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] [Indexed: 11/16/2022] Open
Abstract
Chylous ascites is a rare form of ascites characterized by the accumulation of lymph fluid in the peritoneal cavity. Henoch-Schonlein purpura (HSP) is a form of vasculitis usually seen in children affecting small vessels. Gastrointestinal (GI) manifestations of HSP are coming to the forefront as a presenting symptom. The presence of a rash usually succeeds the GI manifestations, making diagnosis difficult and leading to unnecessary surgical interventions. Our case shows a 38-year-old female who presented with an acute abdomen followed by an erythematous rash noticed later on, with radiological investigations suggestive of acute appendicitis. Chylous ascites was found as an incidental finding on diagnostic laparoscopy with a healthy appendix.
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Affiliation(s)
- Pushkar Galam
- Department of General Surgery, Dr. D. Y. Patil Medical College and Research Centre, Pimpri, Pune, Maharashtra, India
| | - Joseph Kurian Mundakal
- Department of General Surgery, Dr. D. Y. Patil Medical College and Research Centre, Pimpri, Pune, Maharashtra, India,Address for correspondence: Dr. Joseph Kurian Mundakal, Department of General Surgery, Dr. D. Y. Patil Medical College and Research Centre, Pimpri, Pune, Maharashtra, India. E-mail:
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18
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Zhang L, Jia X, Lai P, Wang K, Bao Y, Li X. Relevance of Intestinal Microbiota in Immunoglobulin A Vasculitis With Abdominal Involvement. Front Pediatr 2022; 10:943267. [PMID: 35911834 PMCID: PMC9329519 DOI: 10.3389/fped.2022.943267] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/13/2022] [Accepted: 06/21/2022] [Indexed: 12/04/2022] Open
Abstract
BACKGROUND We explored the intestinal microbiota changes in IgAV with abdominal involvement (IgAV-GI) at the acute and convalescent stages and evaluated the role of intestinal microbiota in the clinical course of patients with IgAV. METHODS A total of 37 patients with IgAV were included, and the control group comprised 37 age- and sex-matched healthy children. Stool samples were collected from 28 children with IgAV-GI (19 in the acute stage and 9 in the recovery stage) and from nine children with non-abdominal involvement. Fecal specimens were selected and DNA was obtained using an extraction kit which was then subjected to high-throughput sequencing and analysis. RESULTS There was no significant difference in the community structure of the intestinal microbiota among the IgAV-GI acute, IgAV-GI convalescence, and IgAV-non-GI stages. The abundance of Veillonella in the acute stage of IgAV-GI was significantly higher than that in IgAV-non-GI and convalescence stages, and Ruminococcus was the most abundant in IgAV-GI convalescence. The α-diversity of children with IgAV was significantly lower than that of healthy children, and healthy children had higher intestinal microbiota richness and more evenly distributed species. In terms of changes in intestinal microbial diversity in patients with IgAV at the genus level, obligate anaerobes such as Bifidobacterium, Prevotella, Coprobacter, Prevotella_9, Blautia, Romboutsia, Parabacteroide, Subdoligranulum, and Roseburia were significantly reduced, and the enrichment of facultative anaerobe was represented by Bacteroides, Lachnoclostridium, and Alistipe. CONCLUSION Different bacterial species may be involved in the pathogenesis of different types of IgAV-GI. Differences were observed in the intestinal microbiota between healthy children and children with IgAV.
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Affiliation(s)
- Linqian Zhang
- Jinhua Maternal and Child Health Care Hospital, Jinhua Women's and Children's Hospital, Jinhua, China
| | - Xinyi Jia
- Jinhua Maternal and Child Health Care Hospital, Jinhua Women's and Children's Hospital, Jinhua, China.,Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Panjian Lai
- Jinhua Maternal and Child Health Care Hospital, Jinhua Women's and Children's Hospital, Jinhua, China
| | - Kang Wang
- Jinhua Maternal and Child Health Care Hospital, Jinhua Women's and Children's Hospital, Jinhua, China
| | - Yunguang Bao
- Jinhua Maternal and Child Health Care Hospital, Jinhua Women's and Children's Hospital, Jinhua, China
| | - Xiaobing Li
- Jinhua Maternal and Child Health Care Hospital, Jinhua Women's and Children's Hospital, Jinhua, China
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Zn-based metal-organic framework: Luminescence sensing for the detection of Fe3+ ions and treatment activity on child allergic purpura. J SOLID STATE CHEM 2021. [DOI: 10.1016/j.jssc.2021.122483] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
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20
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Morotti F, Bracciolini G, Caorsi R, Cattaneo L, Gattorno M, Ravelli A, Felici E. Intravenous immunoglobulin for corticosteroid-resistant intestinal Henoch-Schönlein purpura: worth a controlled trial against corticosteroids? Rheumatology (Oxford) 2021; 60:3868-3871. [PMID: 34340243 DOI: 10.1093/rheumatology/keaa743] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/27/2020] [Revised: 10/01/2020] [Indexed: 11/14/2022] Open
Abstract
OBJECTIVES Henoch-Schönlein purpura (HScP) may present in children with severe, occasionally refractory, gastrointestinal (GI) involvement. The use of corticosteroids (CSs) is commonplace in the management of the disease, but to date no standardized protocol is available and, although rare, resistance to CS therapy may be challenging to clinicians. IVIG has been proposed as an effective alternative to CSs, but to date no controlled trial has been conducted to ascertain their real efficacy. We share our personal experience of successful IVIG treatment in two cases of GI HScP, comparing it with similar experiences reported in literature. METHODS Retrospective clinical data collection, comparison with available literature. RESULTS We describe two children with severe HScP GI vasculitis refractory to high-dose intravenous CSs that responded rapidly to IVIG administration, with complete recovery within a few days. Patient characteristics and response to IVIG administration were comparable to those of other previously reported cases. CONCLUSION Our observation confirms that IVIG may be useful in the treatment of CS-resistant HScP-related GI vasculitis in children, and highlights the need for more structured research, including a randomized trial against CSs, in order to ascertain their real effectiveness.
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Affiliation(s)
- Francesco Morotti
- SCDU of Pediatrics, Department of Health Sciences, Università degli Studi del Piemonte Orientale, Novara, Italy
| | - Giulia Bracciolini
- Pediatrics and Pediatric Emergency Unit, The Children Hospital, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy
| | - Roberta Caorsi
- Clinica Pediatrica e Reumatologia, IRCCS Istituto Giannina Gaslini, Genoa, Italy
| | - Lorella Cattaneo
- Pediatrics and Pediatric Emergency Unit, The Children Hospital, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy
| | - Marco Gattorno
- Clinica Pediatrica e Reumatologia, IRCCS Istituto Giannina Gaslini, Genoa, Italy
| | - Angelo Ravelli
- Clinica Pediatrica e Reumatologia, IRCCS Istituto Giannina Gaslini, Genoa, Italy
| | - Enrico Felici
- Pediatrics and Pediatric Emergency Unit, The Children Hospital, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.,'Umberto Bosio' Centre for Digestive Diseases, The Children Hospital, Azienda Ospedaliera Nazionale SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy
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21
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Zhao Q, Yang Y, He SW, Wang XT, Liu C. Risk factors for intussusception in children with Henoch-Schönlein purpura: A case-control study. World J Clin Cases 2021; 9:6244-6253. [PMID: 34434991 PMCID: PMC8362585 DOI: 10.12998/wjcc.v9.i22.6244] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/12/2021] [Revised: 05/07/2021] [Accepted: 06/03/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND The etiology of Henoch-Schönlein purpura (HSP) with intussusception remains undefined.
AIM To investigate the risk factors for intussusception in children with HSP and gastrointestinal (GI) involvement.
METHODS Sixty children with HSP and concomitant intussusception admitted to the Beijing Children’s Hospital of Capital Medical University between January 2006 and December 2018 were enrolled in this study. One hundred pediatric patients with HSP and GI involvement but without intussusception, admitted to the same hospital during the same period, were randomly selected as a control group. The baseline clinical characteristics of all patients, including sex, age of onset, duration of disease, clinical manifestations, laboratory test results, and treatments provided, were assessed. Univariate and multiple logistic regression analyses were performed to identify possible risk factors.
RESULTS The 60 children in the intussusception group comprised 27 girls (45%) and 33 boys (55%) and the 100 children in the non-intussusception group comprised 62 girls (62%) and 38 boys (38%). The median age of all patients were 6 years and 5 mo. Univariate and multiple regression analyses revealed age at onset, not receiving glucocorticoid therapy within 72 h of emergence of GI symptoms, hematochezia, and D-dimer levels as independent risk factors for intussusception in children with HSP (P < 0.05).
CONCLUSION The four independent risk factors for intussusception in pediatric HSP with GI involvement would be a reference for early prevention and treatment of this potentially fatal disease.
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Affiliation(s)
- Qian Zhao
- Traditional Chinese Medicine Department, National Children's Medical Center, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China
| | - Yan Yang
- Traditional Chinese Medicine Department, National Children's Medical Center, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China
| | - Song-Wei He
- Traditional Chinese Medicine Department, National Children's Medical Center, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China
| | - Xin-Tai Wang
- Traditional Chinese Medicine Department, National Children's Medical Center, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China
| | - Chang Liu
- Traditional Chinese Medicine Department, National Children's Medical Center, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China
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Koskela M, Nihtilä J, Ylinen E, Kolho KL, Nuutinen M, Ritari J, Jahnukainen T. HLA-DQ and HLA-DRB1 alleles associated with Henoch-Schönlein purpura nephritis in Finnish pediatric population: a genome-wide association study. Pediatr Nephrol 2021; 36:2311-2318. [PMID: 33591409 PMCID: PMC8260528 DOI: 10.1007/s00467-021-04955-7] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/23/2020] [Revised: 12/18/2020] [Accepted: 01/19/2021] [Indexed: 12/19/2022]
Abstract
BACKGROUND The pathophysiology of Henoch-Schönlein purpura (HSP) is still unclear, but several findings suggest that genetic factors may influence disease susceptibility. We aimed to perform a genome-wide association study (GWAS) in pediatric HSP patients with an emphasis on severe HSP nephritis. METHODS The study included 46 HSP patients, 42 of whom had undergone kidney biopsy. Forty-nine pediatric patients with an inflammatory bowel disease (IBD) served as an autoimmune disease control group while Finnish bone marrow and blood donors represented the general reference population (n = 18,757). GWAS was performed for HSP and IBD samples in a case-control manner against the reference population. The analysis also included imputation of human leukocyte antigen (HLA) alleles. RESULTS GWAS analysis in HSP revealed several polymorphisms from the HLA region that surpassed the genome-wide significance level. Three HLA class II alleles were also significantly more frequent in HSP than in the reference population: DQA1*01:01, DQB1*05:01, and DRB1*01:01. Haplotype DQA1*01:01/DQB1*05:01/DRB1*01:01 occurred in 43.5% of HSP patients, whereas its frequency was 8.2% in IBD patients and 15.0% in the reference population. HSP patients with this haplotype showed similar baseline clinical findings and outcome as HSP patients negative for the haplotype. In IBD patients, no polymorphism or HLA allele appeared significant at the genome-wide level. CONCLUSIONS Our results suggest that haplotype DQA1*01:01/DQB1*05:01/DRB1*01:01 is associated with susceptibility to HSP, but not with the severity of the kidney involvement. These HLA associations did not occur in IBD patients, suggesting that they are specific to HSP and not related to susceptibility to autoimmune diseases in general.
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Affiliation(s)
- Mikael Koskela
- Children's Hospital, Pediatric Research Center, University of Helsinki, Helsinki University Hospital, Helsinki, Finland. .,Department of Pediatric Nephrology and Transplantation, New Children's Hospital, University of Helsinki and Helsinki University Hospital, PO Box 347, Stenbäckinkatu 9, 00029 HUS, Helsinki, Finland.
| | - Julia Nihtilä
- University of Helsinki, Helsinki, Finland.,Finnish Red Cross Blood Service, Helsinki, Finland
| | - Elisa Ylinen
- Department of Pediatric Nephrology and Transplantation, New Children's Hospital, University of Helsinki and Helsinki University Hospital, PO Box 347, Stenbäckinkatu 9, 00029 HUS, Helsinki, Finland
| | - Kaija-Leena Kolho
- Children's Hospital, Pediatric Research Center, University of Helsinki, Helsinki University Hospital, Helsinki, Finland.,Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland
| | - Matti Nuutinen
- Department of Children and Adolescents, Oulu University Hospital, Oulu, Finland.,PEDEGO Research Unit, Research Unit for Pediatrics, Dermatology, Clinical Genetics, Obstetrics and Gynecology, Medical Research Center Oulu (MRC Oulu), Oulu, Finland
| | - Jarmo Ritari
- Finnish Red Cross Blood Service, Helsinki, Finland
| | - Timo Jahnukainen
- Department of Pediatric Nephrology and Transplantation, New Children's Hospital, University of Helsinki and Helsinki University Hospital, PO Box 347, Stenbäckinkatu 9, 00029 HUS, Helsinki, Finland
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Gastrointestinal involvement in IgA vasculitis: a single-center 11-year study on a cohort of 118 children. Clin Rheumatol 2021; 40:5041-5046. [PMID: 34273001 DOI: 10.1007/s10067-021-05863-9] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/03/2021] [Revised: 07/08/2021] [Accepted: 07/09/2021] [Indexed: 10/20/2022]
Abstract
INTRODUCTION AND OBJECTIVES Immunoglobulin A vasculitis (IgAV), the most common childhood vasculitis, is associated with gastrointestinal (GI) involvement in 50-75% of cases. The aim of this study was to describe the characteristics of GI involvement in a cohort of hospitalized children with IgAV. METHODS We retrospectively evaluated patients hospitalized for IgAV at Meyer Children's University Hospital, from January 2010 to December 2020. The children's families were interviewed by phone and asked about disease relapses. RESULTS In the study period, 118 children had GI involvement, corresponding to 75% of children hospitalized for IgAV. Their median age was 7 years (interquartile range 6-9). The most frequent GI manifestations were abdominal pain (96%), bleeding (71%, mostly occult), vomiting (58%), and diarrhea (17%). GI complications, observed in 18%, were intussusception (14%), appendicitis (3%), gallbladder hydrops (2%), and ileal perforation (1%). Abdomen ultrasound, performed in all cases, was abnormal in 68%. Abdomen X-ray, performed in 19 patients, showed pathologic findings in 84% of them. Selected children with severe manifestations also underwent abdomen computed tomography (2/118; 2%) and GI endoscopy (5/118; 4%). Steroids were used in 80 (67.8%) cases. The use of second- and third-line therapies was necessary in three cases. Relapses, investigated in 80 cases (68%), occurred in 21% of them. Key Points • The present retrospective study describes a cohort of 118 patients hospitalized for gastrointestinal (GI) involvement in immunoglobulin A vasculitis (IgAV) at a tertiary care children's hospital from January 2010 to December 2020. • The most frequent GI manifestations were abdominal pain (96%), vomiting (58%), and diarrhea (17%). GI bleeding was reported in 71% of children, and it was occult in most cases. • GI complications, occurring in 18% of cases, were intussusception (14%), appendicitis (3%), gallbladder hydrops (2%), and ileal perforation (1%). • Steroid treatment has been successfully used in severe GI manifestations; however, our data do not support its association with low relapse risk. CONCLUSIONS The present study describes a large pediatric cohort of GI involvement in IgAV. Steroid treatment should be used when GI manifestations are severe. The association of steroid use with relapse risk is not currently established.
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24
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Sestan M, Kifer N, Frkovic M, Sapina M, Srsen S, Batnozic Varga M, Ovuka A, Held M, Gudelj Gracanin A, Kozmar A, Bulimbasic S, Coric M, Laskarin G, Gagro A, Jelusic M. Gastrointestinal involvement and its association with the risk for nephritis in IgA vasculitis. Ther Adv Musculoskelet Dis 2021; 13:1759720X211024828. [PMID: 34262620 PMCID: PMC8252396 DOI: 10.1177/1759720x211024828] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2020] [Accepted: 05/24/2021] [Indexed: 01/29/2023] Open
Abstract
Background: We analysed clinical and biochemical parameters in predicting severe gastrointestinal (GI) manifestations in childhood IgA vasculitis (IgAV) and the risk of developing renal complications. Methods: A national multicentric retrospective study included children with IgAV reviewed in five Croatian University Centres for paediatric rheumatology in the period 2009–2019. Results: Out of 611 children, 281 (45.99%) had at least one GI manifestation, while 42 of 281 (14.95%) had the most severe GI manifestations. Using logistic regression several clinical risk factors for the severe GI manifestations were identified: generalized rash [odds ratio (OR) 2.09 (95% confidence interval (CI) 1.09–4.01)], rash extended on upper extremities (OR 2.77 (95% CI 1.43–5.34)] or face [OR 3.69 (95% CI 1.42–9.43)] and nephritis (IgAVN) [OR 4.35 (95% CI 2.23–8.50)], as well as lower values of prothrombin time (OR 0.05 (95% CI 0.01–0.62)], fibrinogen [OR 0.45 (95% CI 0.29–0.70)] and IgM [OR 0.10 (95% I 0.03–0.35)]] among the laboratory parameters. Patients with severe GI involvement more frequently had relapse of the disease [OR 2.14 (CI 1.04–4.39)] and recurrent rash [OR 2.61 (CI 1.27–5.38)]. Multivariate logistic regression found that the combination of age, GI symptoms at the beginning of IgAV and severity of GI symptoms were statistically significant predictors of IgAVN. Patients in whom IgAV has started with GI symptoms [OR 6.60 (95% CI 1.67–26.06)], older children [OR 1.22 (95% CI 1.02–1.46)] with severe GI form of IgAV (OR 5.90 (95% CI 1.12–31.15)] were particularly high-risk for developing IgAVN. Conclusion: We detected a group of older children with the onset of GI symptoms before other IgAV symptoms and severe GI form of the IgAV, with significantly higher risk for acute and chronic complications of IgAV.
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Affiliation(s)
- Mario Sestan
- Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
| | - Nastasia Kifer
- Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
| | - Marijan Frkovic
- Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
| | - Matej Sapina
- Department of Paediatrics, Josip Juraj Strossmayer University of Osijek, Medical Faculty and Faculty of Dental Medicine and Health Osijek, University Hospital Centre Osijek, Osijek, Croatia
| | - Sasa Srsen
- Department of Paediatrics, University of Split School of Medicine, University Hospital Centre Split, Split, Croatia
| | - Mateja Batnozic Varga
- Department of Paediatrics, Josip Juraj Strossmayer University of Osijek, Medical Faculty Osijek, University Hospital Centre Osijek, Osijek, Croatia
| | - Aleksandar Ovuka
- Department of Paediatrics, University of Rijeka, Faculty of Medicine, University Hospital Centre Rijeka, Rijeka, Croatia
| | - Martina Held
- Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
| | | | - Ana Kozmar
- Clinical Department of Laboratory Diagnostics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
| | - Stela Bulimbasic
- Department of Pathology and Cytology, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
| | - Marijana Coric
- Department of Pathology and Cytology, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
| | | | - Alenka Gagro
- Department of Paediatrics, Children's Hospital Zagreb, Zagreb, Croatia
| | - Marija Jelusic
- Department of Paediatrics, University of Zagreb School of Medicine, Division of Clinical Immunology, Rheumatology and Allergology, Centre of Reference for Paediatric and Adolescent Rheumatology of Ministry of Health of the Republic Croatia, University Hospital Centre Zagreb, Kispaticeva 12, Zagreb, 10 000, Croatia
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Fohle E, Montgomery S, Murat J, Ness R, Lo TS. An Unusual Timing of Amoxicillin-Induced IgA Vasculitis in an Elderly Patient. Cureus 2021; 13:e15757. [PMID: 34290934 PMCID: PMC8289393 DOI: 10.7759/cureus.15757] [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: 06/19/2021] [Indexed: 11/05/2022] Open
Abstract
Immunoglobulin A vasculitis is a small vessel vasculitis which is usually common in the pediatric group. It is rare in adult population but more severe than in children. Proposed triggers include infections, malignancy and medications. For most part, the association is made when immunoglobulin A vasculitis develops within two weeks after starting the implicated medication. A 66-year-old male who was treated with amoxicillin/clavulanate for presumed right fourth toe infection but returned to the emergency department 48 hours later with palpable purpura of lower limbs, arthralgia with swollen hands and colicky abdominal pain with nausea. Abdominal computed tomography (CT) scan showed mildly dilated small bowel. Skin biopsies showed leukocytoclastic vasculitis with IgA deposit on immunofluorescence. The patient was treated with a short course of steroid and the rash was significantly reduced during subsequent follow-up. Although amoxicillin/clavulanate is widely prescribed, clinicians need to be aware of this risk and immediately stop it if signs of drug-induced vasculitis develop.
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Affiliation(s)
- Emmanuel Fohle
- Internal Medicine, University of North Dakota, Fargo, USA
| | - Sean Montgomery
- Internal Medicine, University of North Dakota School of Medicine and Health Sciences, Grand Forks, USA
| | - Joseph Murat
- Internal Medicine, Veterans Affairs Medical Center, Fargo, USA
| | - Rachel Ness
- Dermatology, Fargo Center for Dermatology, Fargo, USA
| | - Tze Shien Lo
- Infectious Disease, Veterans Affairs Medical Center, Fargo, USA
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26
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Jia L, Wu J, Wei J, Du L, Wang P, Zhang Y, Yu Y, Wang X, Yang Y, Gao Y. Proteomic analysis of urine reveals biomarkers for the diagnosis and phenotyping of abdominal-type Henoch-Schonlein purpura. Transl Pediatr 2021; 10:510-524. [PMID: 33850810 PMCID: PMC8039785 DOI: 10.21037/tp-20-317] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/09/2023] Open
Abstract
BACKGROUND Abdominal-type Henoch-Schonlein purpura (HSP) is a common refractory disease in children. Currently, no specific diagnostic biomarker is available for HSP. METHODS Children with abdominal type HSP were first diagnosed with three syndromes using Chinese traditional medicine. The urinary proteomes among the three syndromes of patients with abdominal type HSP and healthy controls were compared using two label-free proteomics quantifications, including data-dependent acquisition and data-independent acquisition. RESULTS For the comparison between patients with abdominal type HSP and healthy children, a total of 75 differential urinary proteins were identified by determining the overlap of the two experiments. The ingenuity pathway analysis (IPA) analysis showed that these differential proteins were correlated with the pathogenesis of abdominal type HSP. Of these, 37 proteins were distributed in 13 solid tissues as tissue-enriched proteins. Monitoring changes in these proteins might help us detect uncommon clinical manifestations of HSP. Patients with abdominal type HSP can be further distinguished into three syndromes based on the urine proteome. Finally, a panel of six urinary proteins (P25774, P09417, Q7Z5L0, P60900, P14550 and P09668) was constructed for both the diagnosis and phenotyping of abdominal type HSP. CONCLUSIONS Urinary protein biomarkers for the diagnosis and phenotyping of abdominal type HSP were identified, which will contribute to the personalized treatment of patients with abdominal type HSP.
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Affiliation(s)
- Lulu Jia
- Clinical Research Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing, China
| | - Jianqiang Wu
- Medical Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Jing Wei
- Department of Biochemistry and Molecular Biology, School of Life Sciences, Beijing Normal University, Gene Engineering Drug and Biotechnology Beijing Key Laboratory, Beijing, China
| | - Lina Du
- Department of Chinese Medicine, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing, China
| | - Panpan Wang
- Department of Chinese Medicine, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing, China
| | - Yanju Zhang
- Clinical Research Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing, China
| | - Yuncui Yu
- Clinical Research Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing, China
| | - Xiaoling Wang
- Clinical Research Center, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing, China
| | - Yan Yang
- Department of Chinese Medicine, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing, China
| | - Youhe Gao
- Department of Biochemistry and Molecular Biology, School of Life Sciences, Beijing Normal University, Gene Engineering Drug and Biotechnology Beijing Key Laboratory, Beijing, China
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27
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Tan SW, James V, Warier A, Ong GYK. Point-of-care ultrasound identification of pneumatosis intestinalis associated with Henoch-Schönlein purpura gastrointestinal involvement: A case report. World J Emerg Med 2021; 12:76-78. [PMID: 33505555 DOI: 10.5847/wjem.j.1920-8642.2021.01.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
Affiliation(s)
- Sek Wan Tan
- Children's Emergency, KK Women's and Children's Hospital, 229899, Singapore
| | - Vigil James
- Children's Emergency, KK Women's and Children's Hospital, 229899, Singapore
| | - Aswin Warier
- Children's Emergency, KK Women's and Children's Hospital, 229899, Singapore
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28
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Surgical management of small bowel-small bowel intussusception in Henoch-Schönlein Purpura. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2021. [DOI: 10.1016/j.epsc.2020.101703] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
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29
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Zhao M, Xu S, Cavagnaro MJ, Zhang W, Shi J. Quantitative Analysis and Visualization of the Interaction Between Intestinal Microbiota and Type 1 Diabetes in Children Based on Multi-Databases. Front Pediatr 2021; 9:752250. [PMID: 34976889 PMCID: PMC8715853 DOI: 10.3389/fped.2021.752250] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/02/2021] [Accepted: 11/04/2021] [Indexed: 12/12/2022] Open
Abstract
Background: As an important autoimmune disease, type 1 diabetes (T1D) is often diagnosed in children, but due to the complexity of the etiology of diabetes and many other factors, the disease pathogenesis of diabetes is still unclear. The intestinal microbiota has been proved to have close relationships with T1D in recent years, which is one of the most important molecular bases of pathogenesis and prognosis factors for T1D. Using the multi-omics and multicenter sample analysis method, a number of intestinal microbiota in T1D have been discovered and explained, which has provided comprehensive and rich information. However, how to find more useful information and get an intuitive understanding that people need conveniently in the huge data sea has become the focus of attention. Therefore, quantitative analysis and visualization of the interaction between intestinal microbiota and T1D in children are urgently needed. Methods: We retrieved the detailed original data from the National Center for Biotechnology Information, GMREPO, and gutMEGA databases and other authoritative multiple projects with related research; the ranking of intestinal microbiota abundance from healthy people, overall T1D patients, and T1D in children (0-18 years old) were detailed analyzed, classified, and visualized. Results: A total of 515 bacterial species and 161 related genera were fully analyzed. Also, Prevotella copri was led by 21.25% average abundance, followed by Clostridium tertium of 10.39% in all-cross T1D patients. For children with T1D, Bacteroides vulgatus has high abundance in all age periods, whereas the abundance of each intestinal microbiota was more uniform in female samples, with the ranking from high to low as Bacteroides dorei 9.56%, P. copri 9.53%, Streptococcus pasteurianus 8.15%, and C. tertium 7.53%, whereas in male samples, P. copri was accounted for the largest by 22.72%. The interaction between intestinal microbiota and comparison between healthy people and children with T1D was also detailed analyzed. Conclusions: This study provides a new method and comprehensive perspectives for the evaluation of the interaction between intestinal microbiota and T1D in children. A set of useful information of intestinal microbiota with its internal interaction and connections has been presented, which could be a compact, immediate, and practical scientific reference for further molecular biological and clinical translational research of T1D in children.
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Affiliation(s)
- Mingyi Zhao
- Department of Pediatric, The Third Xiangya Hospital, Central South University, Changsha, China
| | - Shaokang Xu
- Department of Pediatric, The Third Xiangya Hospital, Central South University, Changsha, China.,Xiangya School of Medicine, Central South University, Changsha, China
| | | | - Wei Zhang
- Xiangya School of Medicine, Central South University, Changsha, China
| | - Jian Shi
- Department of Spine Surgery, The Third Xiangya Hospital, Central South University, Changsha, China.,Department of Hematology and Critical Care Medicine, The Third Xiangya Hospital, Central South University, Changsha, China
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31
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Ueno M, Shimodate Y, Okada K, Takaya R, Yamamoto H, Mizuno M. Upper gastrointestinal ischemia as a rare complication of paroxysmal nocturnal hemoglobinuria. Clin Case Rep 2021; 9:506-510. [PMID: 33489204 PMCID: PMC7813057 DOI: 10.1002/ccr3.3567] [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: 08/18/2020] [Revised: 10/12/2020] [Accepted: 10/30/2020] [Indexed: 11/10/2022] Open
Abstract
When patients with PNH present with abdominal symptoms, thrombosis-induced gastrointestinal injury should be considered; computed tomography and esophagogastroduodenoscopy may help make the diagnosis of this potentially serious complication.
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Affiliation(s)
- Masayuki Ueno
- Department of Gastroenterology and HepatologyKurashiki Central HospitalKurashikiJapan
| | - Yuichi Shimodate
- Department of Gastroenterology and HepatologyKurashiki Central HospitalKurashikiJapan
| | - Kazuya Okada
- Department of Hematology/OncologyKurashiki Central HospitalKurashikiJapan
| | - Ryosuke Takaya
- Department of Hematology/OncologyKurashiki Central HospitalKurashikiJapan
| | - Hiroshi Yamamoto
- Department of Gastroenterology and HepatologyKurashiki Central HospitalKurashikiJapan
| | - Motowo Mizuno
- Department of Gastroenterology and HepatologyKurashiki Central HospitalKurashikiJapan
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32
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McLaughlin SK, Lawrence L, Adler J, Mehta H. Henoch-Schönlein Purpura-Associated Hemorrhagic Shock After Secondary Norovirus Infection. Cureus 2020; 12:e11653. [PMID: 33391892 PMCID: PMC7769491 DOI: 10.7759/cureus.11653] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
Henoch-Schönlein purpura (HSP) is a small-vessel vasculitis, typically involving the skin, joints, kidneys, and gastrointestinal (GI) tract. Although GI bleeding with HSP can occur, massive GI hemorrhage is rare. It is well documented that HSP can be triggered by a preceding infection, often of the upper respiratory tract. Infections that occur after the development of HSP and trigger worsening of the disease or new complications have not been well reported. We present the case of a three-year-old previously healthy boy who developed HSP with typical signs and symptoms, including hematochezia that resolved after treatment with intravenous steroids. The patient then contracted norovirus and subsequently developed massive GI bleeding, leading to hemorrhagic shock and requiring admission to an intensive care unit. This case demonstrates that secondary infection, such as norovirus infection, can precipitate worsening of underlying HSP vasculitis and lead to acute clinical decompensation. Clinicians should be aware of the risk of acute clinical changes in patients with HSP.
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Affiliation(s)
| | - Lindsey Lawrence
- Pediatric Gastroenterology, University of Michigan, Ann Arbor, USA
| | - Jeremy Adler
- Pediatric Gastroenterology, University of Michigan, Ann Arbor, USA
| | - Hiral Mehta
- Pediatrics, Children's Hospital of Philadelphia, Philadelphia, USA
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33
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d'Angelo DM, Franchini S, Mohn A, Breda L. Factor XIII as a potential predictor of severe gastrointestinal involvement in Henoch Schoenlein purpura: A case study research. J Paediatr Child Health 2020; 56:1821-1823. [PMID: 32297405 DOI: 10.1111/jpc.14886] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/29/2019] [Revised: 01/18/2020] [Accepted: 03/13/2020] [Indexed: 12/01/2022]
Affiliation(s)
- Debora M d'Angelo
- Department of Pediatrics, Gabriele d'Annunzio University of Chieti and Pescara, Chieti, Italy
| | - Simone Franchini
- Department of Pediatrics, Gabriele d'Annunzio University of Chieti and Pescara, Chieti, Italy
| | - Angelika Mohn
- Department of Pediatrics, Gabriele d'Annunzio University of Chieti and Pescara, Chieti, Italy
| | - Luciana Breda
- Pediatric Rheumatology Unit, SS Annunziata Hospital, Chieti, Italy
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34
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Trapani S, Rubino C, Indolfi G. Gastrointestinal involvement in childhood vasculitides. Acta Paediatr 2020; 109:2226-2236. [PMID: 32479665 DOI: 10.1111/apa.15381] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/25/2020] [Revised: 05/18/2020] [Accepted: 05/26/2020] [Indexed: 02/01/2023]
Abstract
AIM The aim of this narrative review was to provide a comprehensive summary of the characteristics of gastrointestinal (GI) involvement in the most common paediatric primary vasculitides. METHODS We used PubMed to primarily identify papers, reviews, case series and editorials published in English from 2000 until 31 January 2020. Based on this, we report the prevalence, clinical manifestations, diagnostic approaches and management of GI involvement in each vasculitis. RESULTS Vasculitides are inflammatory blood vessel diseases, and the majority can affect the GI system with vascular, GI tract or solid organ involvement. GI involvement can sometimes complicate and delay the correct diagnosis. Clinical findings are usually nonspecific symptoms, such as fever, abdominal pain, nausea, vomiting and diarrhoea. Bleeding should alert paediatricians to the possibility of severe complicated vasculitis. Diagnosis relies mostly on imaging. If it goes unrecognised, GI involvement in paediatric vasculitis is a serious cause of morbidity and even mortality, related to bowel ischaemia and perforation. Treatment of GI symptoms depends on the type of vasculitis and usually involves high-dose steroids and immunosuppressants. CONCLUSION GI manifestations in the most common paediatric primary vasculitides were usually nonspecific, diagnosis mostly relied on imaging, and treatment usually involved high-dose corticosteroids and immunosuppressants.
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Affiliation(s)
- Sandra Trapani
- Department of Health Sciences, University of Florence and Meyer Children's University Hospital, Florence, Italy
| | - Chiara Rubino
- Post-graduate School of Pediatrics, University of Florence, Florence, Italy
| | - Giuseppe Indolfi
- Meyer Children's University Hospital and Department, NEUROFARBA, University of Florence, Florence, Italy
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35
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Al E'ed A. Henoch-Schönlein purpura in Saudi Arabia: characteristics and rare vital organ involvement. Minerva Pediatr (Torino) 2020; 73:363-370. [PMID: 32960004 DOI: 10.23736/s2724-5276.20.05962-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
Henoch-Schönlein purpura (HSP) is the most common small vessel vasculitis in children with an annual incidence of between 10 and 30 per 100,000. It is an inflammation that affects many organs primarily in the skin, gastrointestinal (GI) tract, musculoskeletal, also vital organs like kidneys and lungs which may lead to chronic kidney disease and pulmonary hemorrhage. In this review, we are showing the characteristics of Saudi patients with HSP and the relation with preceding infections, gender, and seasonal variations in addition to the common and most severe systemic involvements. We found cases that were challenging to diagnose and others with unfortunate fetal complications.
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Affiliation(s)
- Ashwaq Al E'ed
- Department of Pediatrics, College of Medicine, Qassim University, Qassim, Saudi Arabia -
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36
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Yamauchi N, Tanda S, Kashiwagi S, Ohnishi A, Kugai M, Akazawa T, Matsumoto T, Yamauchi J, Muramatsu A, Fujimoto S. Fatal gastrointestinal bleeding due to IgA vasculitis complicated with tuberculous lymphadenitis: A case report and literature review. Clin Case Rep 2020; 8:1741-1747. [PMID: 32983488 PMCID: PMC7495800 DOI: 10.1002/ccr3.2938] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2019] [Revised: 03/28/2020] [Accepted: 04/25/2020] [Indexed: 01/24/2023] Open
Abstract
We report a case of IgA vasculitis that developed during the treatment of tuberculosis. Patients with tuberculosis who are on antituberculosis treatment can be administered steroids for severe disease or complications.
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Affiliation(s)
| | - Shuji Tanda
- Department of NephrologyAkashi City HospitalAkashiJapan
| | - Saori Kashiwagi
- Department of GastroenterologyAkashi City HospitalAkashiJapan
| | | | - Munehiro Kugai
- Department of GastroenterologyAkashi City HospitalAkashiJapan
| | - Takako Akazawa
- Department of GastroenterologyAkashi City HospitalAkashiJapan
| | | | - Junko Yamauchi
- Department of GastroenterologyAkashi City HospitalAkashiJapan
| | - Akira Muramatsu
- Department of GastroenterologyAkashi City HospitalAkashiJapan
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Zhang LH, Lan T, He D, Wen SL, Tong H. A challenging case on diagnosing Henoch–Schönlein purpura. EUR J INFLAMM 2020. [DOI: 10.1177/2058739220929580] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023] Open
Abstract
Henoch–Schönlein purpura (HSP) could involve multiple organs, including gastrointestinal tract. It is commonly observed in children, but occasionally also in the elderly. It is challenging to diagnose HSP with delayed purpura. Herein, we report an elderly with HSP, whose non-thrombocytopenic purpura erupted more than 1 month after the first onset of abdominal pain.
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Affiliation(s)
- Lin-Hao Zhang
- Lab of Gastroenterology and Hepatology, West China Hospital, Sichuan University, Chengdu, China
- Division of Peptides Related with Human Diseases, West China Hospital, Sichuan University, Chengdu, China
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, China
| | - Tian Lan
- Lab of Gastroenterology and Hepatology, West China Hospital, Sichuan University, Chengdu, China
- Division of Peptides Related with Human Diseases, West China Hospital, Sichuan University, Chengdu, China
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, China
| | - Du He
- Department of Pathology, West China Hospital, Sichuan University, Chengdu, China
| | - Shi-Lei Wen
- Department of Human Anatomy, West China School of Basic Medical Sciences and Forensic Medicine, Sichuan University, Chengdu, China
| | - Huan Tong
- Lab of Gastroenterology and Hepatology, West China Hospital, Sichuan University, Chengdu, China
- Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu, China
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38
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Du L, Wang P, Liu C, Li S, Yue S, Yang Y. Multisystemic manifestations of IgA vasculitis. Clin Rheumatol 2020; 40:43-52. [PMID: 32557258 DOI: 10.1007/s10067-020-05166-5] [Citation(s) in RCA: 32] [Impact Index Per Article: 6.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/19/2020] [Revised: 05/07/2020] [Accepted: 05/11/2020] [Indexed: 01/27/2023]
Abstract
Immunoglobulin A vasculitis (IgAV), also known as Henoch-Schönlein Purpura, is one of the most common kind of systemic vasculitis in children, and due to the involvement of small blood vessels throughout the body, this disease can cause a variety of symptoms in different organs. Our aim was to review the data on various systemic manifestations of IgAV. A research of the literature was performed in PubMed database, utilizing the MeSH terms "IgA vasculitis" and "Henoch Schönlein Purpura". According to the predetermined structure of the manuscript, we extracted and sorted out the relevant data. Clinically, almost all the patients will present with palpable skin purpura, together with arthritis, gastrointestinal tract involvement, or kidney damage. Other rare systemic manifestations include neurological symptoms, scrotal involvement, and cardiopulmonary disease. When uncommon complications occur, patients may be misdiagnosed as other diseases, thus delaying treatment. Although the course of IgAV is mostly self-limited, misdiagnosis can also lead to a poor prognosis. A comprehensive awareness to the clinical manifestations of IgAV is the necessary prerequisite for its timely diagnosis. Prompt diagnosis and adequate treatment are essential for optimal results.
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Affiliation(s)
- Lina Du
- Department of Chinese Medicine, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, No. 56 Nanlishi Road, Beijing, 100045, China
| | - Panpan Wang
- Department of Chinese Medicine, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, No. 56 Nanlishi Road, Beijing, 100045, China
| | - Chang Liu
- Department of Chinese Medicine, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, No. 56 Nanlishi Road, Beijing, 100045, China
| | - Shaojing Li
- Department of Chinese Medicine, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, No. 56 Nanlishi Road, Beijing, 100045, China
| | - Shuang Yue
- Department of Chinese Medicine, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, No. 56 Nanlishi Road, Beijing, 100045, China
| | - Yan Yang
- Department of Chinese Medicine, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, No. 56 Nanlishi Road, Beijing, 100045, China.
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39
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Patel AV, Jotwani PM, Fitzpatrick LJ, Gottesman SP. IgA vasculitis: a rare cause of rash, epigastric pain and melena. QJM 2020; 113:369-370. [PMID: 31538191 DOI: 10.1093/qjmed/hcz232] [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: 11/14/2022] Open
Affiliation(s)
- A V Patel
- Division of Gastroenterology, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA
| | - P M Jotwani
- Department of Medicine, Hackensack Meridian Health Mountainside Medical Center, Montclair, NJ, USA
| | - L J Fitzpatrick
- Department of Dermatology, Zucker School of Medicine at Hofstra/Northwell, New Hyde Park, NY, USA
| | - S P Gottesman
- Department of Dermatology, Zucker School of Medicine at Hofstra/Northwell, New Hyde Park, NY, USA
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40
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Hočevar A, Tomšič M, Jurčić V, Perdan Pirkmajer K, Rotar Ž. Predicting gastrointestinal and renal involvement in adult IgA vasculitis. Arthritis Res Ther 2019; 21:302. [PMID: 31878954 PMCID: PMC6933935 DOI: 10.1186/s13075-019-2089-2] [Citation(s) in RCA: 24] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/20/2019] [Accepted: 12/16/2019] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Immunoglobulin A vasculitis (IgAV) is still poorly defined in the adult population. We aimed to determine the predictors of gastrointestinal (GI) or renal involvement in adult IgAV. METHODS The prospective study included histologically proven adult IgAV cases diagnosed between January 2013 and July 2019 at our secondary/tertiary rheumatology center. We evaluated the role of clinical and the laboratory parameters as markers predicting the GI or renal involvement in IgAV, using the multiple logistic regression analysis. RESULTS During the 79-month observation period, we identified 214 new adult IgAV cases (59.3% males, median (interquartile range) age 64.6 (57.2-76.7) years). The GI tract and renal involvement developed in 58 (27.1%) and 83 (38.8%) cases, respectively (concurrently in 26 (12.1%) cases). In the multivariate logistic regression analysis, generalized purpura (OR 6.74 (95%CI 3.18-14.31)), the pre-treatment neutrophil to lymphocyte ratio (NLR) > 3.5 (OR 2.78 (95%CI 1.34-5.75)), and elevated serum IgA levels (OR 0.40 (95%CI 0.20-0.79)) were extracted as factors associated with GI complications, whereas current smoking (OR 3.23 (95%CI 1.50-6.98)), generalized purpura (OR 1.98 (95%CI 1.08-3.61)), elevated serum IgA (OR 2.25 (95%CI 1.21-4.18)), NLR > 3.5 (OR 1.96 (95%CI 1.02-3.77)), and marginally age (1.02 (95%CI 1.01-1.04)) emerged as factors associated with renal complications. CONCLUSION Generalized purpura and pre-treatment NLR predicted both GI and renal involvement, whereas active smoking was associated with renal involvement, and the serum IgA level had a divergent effect on renal and GI involvement in adult IgAV.
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Affiliation(s)
- Alojzija Hočevar
- Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta 62, 1000, Ljubljana, Slovenia. .,Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
| | - Matija Tomšič
- Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta 62, 1000, Ljubljana, Slovenia.,Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
| | - Vesna Jurčić
- Faculty of Medicine, Institute of Pathology, University of Ljubljana, Ljubljana, Slovenia
| | - Katja Perdan Pirkmajer
- Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta 62, 1000, Ljubljana, Slovenia
| | - Žiga Rotar
- Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta 62, 1000, Ljubljana, Slovenia
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Bai LP, Yu J, Sun YX, Wang JM. Efficacy of montelukast for the treatment of pediatric allergic purpura. Medicine (Baltimore) 2019; 98:e17239. [PMID: 31574836 PMCID: PMC6775355 DOI: 10.1097/md.0000000000017239] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/04/2022] Open
Abstract
BACKGROUND This study aims to evaluate the efficacy and safety of montelukast for the treatment of patients with pediatric allergic purpura (PAP). METHODS We will retrieve the following electronic databases from inception to the present: MEDILINE, Embase, CENTRAL, CINAHL, AMED, Chinese Biomedical Literature Database, China National Knowledge Infrastructure Database, Wanfang, and VIP database without language limitation. Two authors will carry out study selection, data extraction, and quality evaluation independently. RevMan V5.3 software will be used for statistical software. RESULTS This study will summarize high-quality evidence-based medicine to evaluate the efficacy and safety of montelukast for the treatment of PAP. CONCLUSION This study will provide strong evidence to determine whether montelukast is an effective and safety treatment for PAP. SYSTEMATIC REVIEW REGISTRATION PROSPERO CRD42019145472.
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Affiliation(s)
| | - Jing Yu
- Department of Endocrinology, Affiliated Hongqi Hospital of Mudanjiang Medical University
| | - Ya-xin Sun
- Department of Library, Mudanjiang Medical University
| | - Jiu-mei Wang
- Department of Dermatology, Affiliated Hongqi Hospital of Mudanjiang Medical University, Mudanjiang, China
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Shimoyama T, Matsuda N, Kurobe M, Hayakawa T, Nishioka M, Shimohira M, Takasawa K. Colonoscopic diagnosis and reduction of recurrent intussusception owing to Henoch-Schönlein purpura without purpura. Paediatr Int Child Health 2019; 39:219-223. [PMID: 29621936 DOI: 10.1080/20469047.2018.1457273] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
About 50-75% of patients with Henoch-Schönlein purpura (HSP) develop gastro-intestinal symptoms with surgical complications such as intussusception occurring in 0.7-13.6%. In 10-40% of patients, however, gastro-intestinal manifestations may precede the onset of purpura. In patients with gastro-intestinal tract involvement without purpura, confirming the diagnosis of HSP and determining the appropriate treatment remains difficult. A seven-year-old boy presented with recurrent intussusception owing to HSP without purpura. It was confirmed pathologically and treated via colonoscopy. Early colonoscopic intervention can contribute to the early diagnosis of HSP and its subsequent management by avoiding unnecessary surgical invasion.
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Affiliation(s)
- Teruyoshi Shimoyama
- a Departments of Pediatrics , Kawaguchi Municipal Medical Center , Saitama , Japan
| | - Nozomi Matsuda
- a Departments of Pediatrics , Kawaguchi Municipal Medical Center , Saitama , Japan
| | - Masashi Kurobe
- a Departments of Pediatrics , Kawaguchi Municipal Medical Center , Saitama , Japan
| | - Takehiko Hayakawa
- b Departments of Gastroenterology , Kawaguchi Municipal Medical Center , Saitama , Japan
| | - Masato Nishioka
- a Departments of Pediatrics , Kawaguchi Municipal Medical Center , Saitama , Japan
| | - Masayuki Shimohira
- a Departments of Pediatrics , Kawaguchi Municipal Medical Center , Saitama , Japan
| | - Kei Takasawa
- a Departments of Pediatrics , Kawaguchi Municipal Medical Center , Saitama , Japan.,c Department of Pediatrics and Developmental Biology , Tokyo Medical and Dental University , Tokyo , Japan
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43
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Gweon T, Kwon J. Rapid progression and recovery of massive gastric mucosal damage complicated by Henoch‑Sch�nlein purpura in a patient with liver cirrhosis: A case report. Exp Ther Med 2019; 18:2051-2054. [PMID: 31452703 PMCID: PMC6704504 DOI: 10.3892/etm.2019.7815] [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: 12/25/2018] [Accepted: 07/05/2019] [Indexed: 11/18/2022] Open
Abstract
Endoscopic findings associated with gastric ischemia include friable gastric mucosa, irregular ulceration, petechiae and purpura. The etiology of gastric ischemia includes shock, sepsis, thrombosis, embolism and vasculitis. Henoch-Schönlein purpura (HSP) is a leukocytoclastic vasculitis that affects small vessels in the skin, kidney and gastrointestinal (GI) tract. Rapid progression of HSP manifested with acute gastric mucosal bleeding has been rarely reported. The present case study reports on a patient who had undergone endoscopic variceal ligation for esophageal varix bleeding and the gastric mucosa exhibited no evidence of HSP. After 9 days, reexamination using endoscopy to check for new-onset hematemesis revealed massive necrosis in the gastric mucosa, friable mucosal tissue and acute ischemic GI bleeding. The patient was treated with corticosteroid injections for 7 days, which caused a marked regression of the hemorrhagic gastric mucosa. The present case study reported on this patient with acute GI bleeding caused by HSP who was successfully treated with corticosteroids.
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Affiliation(s)
- Tae‑Geun Gweon
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Incheon 21431, Republic of Korea
| | - Jung Kwon
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Incheon 21431, Republic of Korea
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Bose S, Pathireddy S, Baradhi KM, Aeddula NR. Seizures, renal failure and acute respiratory failure: not your typical case of Henoch-Schonlein purpura. BMJ Case Rep 2019; 12:12/7/e229939. [PMID: 31320374 DOI: 10.1136/bcr-2019-229939] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
A 30-year-old Caucasian woman with no prior medical history presented with pedal oedema, arthralgias and abdominal pain with diarrhoea, following a respiratory infection. She had mild abdominal tenderness along with a purpuric rash on the extremities and was anaemic. Following initial workup for anaemia and rash, her condition deteriorated with renal impairment, respiratory failure and seizures necessitating ventilatory support, dialysis and steroids. Serologies were negative, and skin biopsy showed leucocytoclastic vasculitis without vascular IgA deposition, and renal biopsy showed subendothelial, mesangial deposits of IgA with C3 indicative of Henoch-Schonlein purpura (HSP). She was treated with steroids, haemodialysis and on 6-month follow-up recovered renal function. We present the case to illustrate that HSP, though rare in adults, can present with multiorgan failure, with renal, pulmonary and central nervous system involvement, and the need for early diagnosis and prompt treatment for rapid clinical recovery.
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Affiliation(s)
- Subhasish Bose
- Department of Nephrology, Lynchburg Nephrology, Lynchburg, Virginia, USA
| | - Samata Pathireddy
- Department of Internal Medicine, Deaconess Health System, Evansville, Indiana, USA
| | - Krishna M Baradhi
- Department of Nephrology, University of Oklahoma School of Community Medicine, Tulsa, Oklahoma, USA
| | - Narothama Reddy Aeddula
- Department of Medicine, Deaconess Health System, Evansville, Indiana, USA.,Department of Medicine, Indiana University School of Medicine, Evansville, Indiana, USA
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Tanaka T, Hiramatsu K, Saito Y, Nosaka T, Takahashi K, Naito T, Ofuji K, Matsuda H, Ohtani M, Nemoto T, Suto H, Nakamoto Y. The Usefulness of Video Capsule Endoscopy in Evaluating Gastrointestinal Manifestations of Immunoglobulin A Vasculitis. Intern Med 2019; 58:1979-1985. [PMID: 30996162 PMCID: PMC6702007 DOI: 10.2169/internalmedicine.2097-18] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/18/2022] Open
Abstract
Objective Immunoglobulin (Ig) A vasculitis (IgAV) is a systemic vasculitis characterized by purpura and gastrointestinal involvement showing abdominal pain that usually occurs after the emergence of purpura. Criteria for evaluating gastrointestinal manifestations of IgAV are unavailable at present, so we conducted a study to investigate the usefulness of video capsule endoscopy (VCE) for detecting gastrointestinal manifestations of IgAV. Methods The clinical data of 10 patients who underwent VCE for IgAV at our hospital from 2012 to 2017 were collected. Nine patients underwent esophagogastroduodenoscopy, and five underwent colonoscopy. We compared the endoscopic severity determined by VCE to the findings of esophagogastroduodenoscopy and colonoscopy. Results The rates of positive findings of esophagogastroduodenoscopy, VCE, and colonoscopy were 56%, 100%, and 80%, respectively. In 70% of cases, the most severe gastrointestinal findings were detected by VCE. VCE was performed on average 16.8 days after the emergence of purpura, and in 8 of 10 patients, its result triggered the initiation of steroid therapy or the control of the steroid dose. Conclusion VCE is very useful for confirming gastrointestinal involvement in IgAV and may be used to determine the timing of steroid therapy initiation. We recommend performing VCE when IgAV is suspected in patients with gastrointestinal symptoms.
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Affiliation(s)
- Tomoko Tanaka
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
| | - Katsushi Hiramatsu
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
| | - Yasushi Saito
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
| | - Takuto Nosaka
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
| | - Kazuto Takahashi
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
| | - Tatsushi Naito
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
| | - Kazuya Ofuji
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
| | - Hidetaka Matsuda
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
| | - Masahiro Ohtani
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
| | - Tomoyuki Nemoto
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
| | - Hiroyuki Suto
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
- Department of Gastroenterology, Fukui Katsuyama General Hospital, Japan
| | - Yasunari Nakamoto
- Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Japan
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Ozen S, Marks SD, Brogan P, Groot N, de Graeff N, Avcin T, Bader-Meunier B, Dolezalova P, Feldman BM, Kone-Paut I, Lahdenne P, McCann L, Pilkington C, Ravelli A, van Royen A, Uziel Y, Vastert B, Wulffraat N, Kamphuis S, Beresford MW. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis—the SHARE initiative. Rheumatology (Oxford) 2019; 58:1607-1616. [DOI: 10.1093/rheumatology/kez041] [Citation(s) in RCA: 101] [Impact Index Per Article: 16.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2018] [Revised: 01/09/2019] [Indexed: 01/25/2023] Open
Abstract
Abstract
Objectives
IgA vasculitis (IgAV, formerly known as Henoch–Schönlein purpura) is the most common cause of systemic vasculitis in childhood. To date, there are no internationally agreed, evidence-based guidelines concerning the appropriate diagnosis and treatment of IgAV in children. Accordingly, treatment regimens differ widely. The European initiative SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) aims to optimize care for children with rheumatic diseases. The aim therefore was to provide internationally agreed consensus recommendations for diagnosis and treatment for children with IgAV.
Methods
Recommendations were developed by a consensus process in accordance with the EULAR standard operating procedures. An extensive systematic literature review was performed, and evidence-based recommendations were extrapolated from the included papers. These were evaluated by a panel of 16 international experts via online surveys and subsequent consensus meeting, using nominal group technique. Recommendations were accepted when ⩾80% of experts agreed.
Results
In total, 7 recommendations for diagnosis and 19 for treatment of paediatric IgAV were accepted. Diagnostic recommendations included: appropriate use of skin and renal biopsy, renal work-up and imaging. Treatment recommendations included: the importance of appropriate analgesia and angiotensin-converting enzyme inhibitor use and non-renal indications for CS use, as well as a structured approach to treating IgAV nephritis, including appropriate use of CS and second-line agents in mild, moderate and severe disease along with use of angiotensin-converting enzyme inhibitors and maintenance therapy.
Conclusion
The SHARE initiative provides international, evidence-based recommendations for the diagnosis and treatment of IgAV that will facilitate improvement and uniformity of care.
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Affiliation(s)
- Seza Ozen
- Department of Paediatrics, Hacettepe University, Ankara, Turkey
| | - Stephen D. Marks
- Great Ormond Street Hospital for Children NHS Foundation Trust, University College London Great Ormond Street Institute of Child Health, London, UK
| | - Paul Brogan
- Great Ormond Street Hospital for Children NHS Foundation Trust, University College London Great Ormond Street Institute of Child Health, London, UK
| | - Noortje Groot
- Wilhelmina Children’s Hospital, University Medical Center, Utrecht
- Sophia Children’s Hospital, Erasmus University Medical Centre, Rotterdam, The Netherlands
- Department of Paediatric Rheumatology, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK
| | - Nienke de Graeff
- Wilhelmina Children’s Hospital, University Medical Center, Utrecht
| | - Tadej Avcin
- Department of Paediatric Rheumatology, University Children’s Hospital Ljubljana, Ljubljana, Slovenia
| | | | - Pavla Dolezalova
- General University Hospital and 1 Faculty of Medicine, Charles University, Prague, Czech Republic
| | - Brian M. Feldman
- The Hospital for Sick Children, University of Toronto, Toronto, Canada
| | - Isabelle Kone-Paut
- Department of Paediatric Rheumatology, Bicêtre University Hospital, Paris, France
| | - Pekka Lahdenne
- Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland
| | - Liza McCann
- Department of Paediatric Rheumatology, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK
| | - Clarissa Pilkington
- Great Ormond Street Hospital for Children NHS Foundation Trust, University College London Great Ormond Street Institute of Child Health, London, UK
| | | | - Annet van Royen
- Wilhelmina Children’s Hospital, University Medical Center, Utrecht
| | - Yosef Uziel
- Meir Medical Centre, Tel Aviv University, Tel Aviv, Israel
| | - Bas Vastert
- Wilhelmina Children’s Hospital, University Medical Center, Utrecht
| | - Nico Wulffraat
- Wilhelmina Children’s Hospital, University Medical Center, Utrecht
| | - Sylvia Kamphuis
- Sophia Children’s Hospital, Erasmus University Medical Centre, Rotterdam, The Netherlands
| | - Michael W. Beresford
- Department of Paediatric Rheumatology, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK
- Institute of Translational Medicine, University of Liverpool and Alder Hey children's NHS Foundation Trust, Members of Liverpool Health Partners, Liverpool, UK
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The clinical spectrum of Henoch-Schönlein purpura in children: a single-center study. Clin Rheumatol 2019; 38:1707-1714. [PMID: 30734116 DOI: 10.1007/s10067-019-04460-1] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/21/2018] [Revised: 01/25/2019] [Accepted: 01/28/2019] [Indexed: 10/27/2022]
Abstract
OBJECTIVES Henoch-Schönlein purpura (HSP) is the most common vasculitis of children. The aim of this study is to evaluate the demographic and clinic findings of patients with HSP and also to determine predictive factors for assessing the development of gastrointestinal system (GIS) and renal involvement. METHODS This study was performed prospectively among children with HSP who are under 18 years of age and being followed-up in the Pediatric Rheumatology Unit of Health Sciences University Kanuni Sultan Süleyman Training and Research Hospital between January 2016 and January 2018. RESULTS A total of 265 patients, 137 boys (51.7%) and 128 girls (48.3%), were involved to the study. The mean ± standard deviation of age at the diagnosis was 7.5 ± 3.2. The most common disease onset season was spring (31.7%). The rate of arthritis, GIS involvement, and renal involvement were 54%, 51.3%, and 29.1%, respectively. GIS bleeding was more frequent in males than females (p = 0.007). Boys over 7 years of age had significantly more common GIS bleeding (p = 0.04). Intussusception, relapse, and serious GIS involvement requiring hospitalization and steroid treatment were highly associated with severe renal involvement. CONCLUSIONS We demonstrated that patients suffering intussusception, relapse, and serious GIS involvement or requiring hospitalization and steroid treatment had tendency to present with severe renal involvement. Therefore, these patients should be followed up carefully for not overlooking renal involvement of HSP.
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Sato S, Migita K. Gastrointestinal Involvement of Systemic Vasculitis. GASTROINTESTINAL AND HEPATIC MANIFESTATIONS OF RHEUMATIC DISEASES 2019:137-153. [DOI: 10.1007/978-981-13-6524-9_9] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Neviackas J, Kotwal V, Attar B. A Rare Cause of Gastrointestinal Bleeding and Rash in an Older Woman. Gastroenterology 2018; 154:1592-1593. [PMID: 29614299 DOI: 10.1053/j.gastro.2017.11.298] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/03/2017] [Revised: 11/23/2017] [Accepted: 11/30/2017] [Indexed: 12/02/2022]
Affiliation(s)
- Jordan Neviackas
- Department of Gastroenterology and Hepatology, John H. Stroger Hospital, Chicago, Illinois
| | - Vikram Kotwal
- Department of Gastroenterology and Hepatology, John H. Stroger Hospital, Chicago, Illinois
| | - Bashar Attar
- Department of Gastroenterology and Hepatology, John H. Stroger Hospital, Chicago, Illinois
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50
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Rubino C, Paci M, Resti M, Lionetti P, Trapani S. Late Relapse of Henoch-Schönlein Purpura in an Adolescent Presenting as Severe Gastroduodenitis. Front Pediatr 2018; 6:355. [PMID: 30525016 PMCID: PMC6256137 DOI: 10.3389/fped.2018.00355] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/08/2018] [Accepted: 10/31/2018] [Indexed: 12/30/2022] Open
Abstract
Henoch-Schönlein purpura is a systemic vasculitis, commonly affecting children. Gastrointestinal manifestations are observed in 50-75% of patients; it is well known they may occur before skin lesions in about 20% of cases during the first vasculitic episode. Relapses occur in about one third of patients, typically within 4 months from the initial presentation and with milder symptoms. We report the case of a 17-year old girl with an atypical relapse of Henoch-Schönlein purpura, presenting with acute abdominal symptoms 5 years after the first episode. Esophagogastroduodenoscopy showed duodenal multiple hyperemic and hemorrhagic lesions. To our knowledge this is the first case of hemorrhagic-erosive duodenitis representing a relapse of Henoch-Schönlein purpura occurring several years after the initial episode. Duodenojejunal inflammation should be considered as primary manifestation of Henoch-Schönlein purpura, not only during the first episode, but also in relapses. Endoscopy can be helpful for differential diagnosis, especially in patients with atypical manifestations. Further studies are needed to evaluate risk factors for Henoch-Schönlein purpura recurrence and the possible role of fecal calprotectin as an early marker for gastrointestinal involvement.
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Affiliation(s)
- Chiara Rubino
- Pediatric Department, Anna Meyer Children's Hospital, Florence, Italy
| | - Monica Paci
- Gastroenterology Unit, Anna Meyer Children's Hospital, Florence, Italy
| | - Massimo Resti
- Pediatric Department, Anna Meyer Children's Hospital, Florence, Italy
| | - Paolo Lionetti
- Gastroenterology Unit, Anna Meyer Children's Hospital, Florence, Italy
| | - Sandra Trapani
- Pediatric Department, Anna Meyer Children's Hospital, Florence, Italy
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