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Li SX, Hu RK, Kong DL, Xu J, Bian Q, Guo ZY, Mei XB. Efficacy of Telitacicept in treating IgA vasculitis nephritis: a two-case report. Front Immunol 2025; 16:1564242. [PMID: 40375991 PMCID: PMC12078309 DOI: 10.3389/fimmu.2025.1564242] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/21/2025] [Accepted: 04/02/2025] [Indexed: 05/18/2025] Open
Abstract
Telitacicept, a B lymphocyte stimulator/A proliferation-inducing ligand dual-target fusion protein, mainly used for IgA nephropathy and systemic lupus erythematosus. We present two cases where adult patients with IgA vasculitis (IgAV) nephritis were successfully treated with Telitacicept, experienced no adverse reactions during the follow-up. Therefore, Telitacicept represents a promising additional treatment option for patients suffering from IgA vasculitis (IgAV).
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Affiliation(s)
- Shuang-xi Li
- Department of Nephrology, Changhai Hospital, The Naval Medical University, Shanghai, China
| | - Rui-kang Hu
- Medical School of Chinese People’s Liberation Army (PLA), Beijing, China
| | - De-liang Kong
- Department of Nephrology, Changhai Hospital, The Naval Medical University, Shanghai, China
| | - Jing Xu
- Department of Nephrology, Changhai Hospital, The Naval Medical University, Shanghai, China
| | - Qi Bian
- Department of Nephrology, Changhai Hospital, The Naval Medical University, Shanghai, China
| | - Zhi-Yong Guo
- Department of Nephrology, Changhai Hospital, The Naval Medical University, Shanghai, China
| | - Xiao-bin Mei
- Department of Nephrology, Changhai Hospital, The Naval Medical University, Shanghai, China
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Konstantopoulou A, Tsoliakos I, Berikopoulou MM, Kiorsavva A, Theochari M, Drosatou P, Messaritaki A, Dimopoulou D. A case-based review of IgA vasculitis complicated with gastrointestinal infections: insights from a norovirus-associated case in an adolescent. Rheumatol Int 2025; 45:114. [PMID: 40261399 DOI: 10.1007/s00296-025-05876-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2025] [Accepted: 04/10/2025] [Indexed: 04/24/2025]
Abstract
Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is a self-limited leukocytoclastic vasculitis targeting small-sized vessels. It is the most common vasculitis in children, but also targets adults who have usually worse prognosis. It typically presents with purpuric rash, arthritis, colicky abdominal pain and potential renal involvement. Gastrointestinal (GI) manifestations, although frequent, may be severe and occasionally complicated by infections. We present a 11-year-old male with a recent diagnosis of IgAV who was admitted with severe abdominal pain, hematemesis and hematochezia. Stool analysis identified Norovirus as the causative pathogen for the severe gastrointestinal symptoms. Treatment with proton pump inhibitors, high-dose corticosteroids and intravenous fluids resulted in symptoms resolution and clinical improvement. In addition, a case-based review of the literature was conducted to evaluate the prevalence of GI infections occurring after the development of IgAV that may result in severe complications or disease relapses. Thirty-five patients with a median age of 14 years were included in our study. Among these patients, 42.9% were diagnosed with a concomitant bacterial gastrointestinal pathogen, followed by 25.7% with viral gastroenteritis and 17.1% with parasitic gastrointestinal disease. Treatment was individualized, with 48.5% receiving corticosteroids and 8.6% receiving immunosuppressive therapy. This case and case-based review highlights the significance of careful management and monitoring of patients with IgAV complicated by infections, because severe complications can cause significant morbidity and mortality especially in adults.
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Affiliation(s)
- Argyro Konstantopoulou
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Ioannis Tsoliakos
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Maria M Berikopoulou
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Aikaterini Kiorsavva
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Maria Theochari
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Panagiota Drosatou
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Anna Messaritaki
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece
| | - Dimitra Dimopoulou
- Second Department of Pediatrics, Children's Hospital "Aghia Sofia", Thivon and Papadiamantopoulou, Athens, 11527, Greece.
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Kambara S, Nishio N, Sugiyama Y, Nishio Y, Takamoto Y, Kitai F, Takahashi Y, Hayashi N, Haruta K, Kondo M, Oike N, Miwa T, Watanabe N, Omori M, Kinoshita F, Furukawa T, Kawada JI, Kidokoro H, Sato Y, Takahashi Y. 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] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/06/2025] [Revised: 03/08/2025] [Accepted: 03/23/2025] [Indexed: 04/05/2025]
Abstract
This study aims to evaluate the impact of early steroid discontinuation on total dosage and outcomes in pediatric immunoglobulin A (IgA) vasculitis patients with uncontrolled abdominal pain. This retrospective cohort study included children younger than 16 years with newly diagnosed IgA vasculitis hospitalized for abdominal pain who received their first dose of steroids between April 1, 2013, and March 31, 2019, at 14 hospitals. Patients were divided into two groups: the standard (STD) group, which received steroid therapy for at least 8 consecutive days, and the early discontinuation attempt (EDA) group, which attempted discontinuation within 7 days. EDA was further divided into two subgroups: the early discontinuation (ED) group, which completed steroid treatment within a week, and the readministration (RA) group, which required readministration. Total steroid dosage, duration of therapy, hospital stay, and complications were compared. A total of 272 patients were analyzed: STD (n = 190) and EDA (n = 82). There were no significant differences in baseline characteristics. EDA had a shorter hospital stay (8.5 vs. 15.0 days, p < 0.01), fewer total steroid days (6 vs. 17.5 days, p < 0.01), and lower total steroid dosage (5.4 mg/kg vs. 15.4 mg/kg, p < 0.01) compared to STD, with no significant differences in complications. Among EDA patients, 22 (27%) required steroid readministration due to symptom recurrence; however, symptoms resolved in all RA patients, with lower total steroid dosage and duration compared to STD, without prolonging hospital stay. Conclusion: Discontinuing steroids within 7 days for abdominal pain in children with IgA vasculitis reduces total steroid dosage without increasing complications, even with occasional readministration. Clinical trial registration: Approval no. 2019-0394.
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Affiliation(s)
- Sumika Kambara
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Nobuhiro Nishio
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.
- Center for Advanced Medicine and Clinical Research, Department of Advanced Medicine, Nagoya University Hospital, 65 Tsurumai-Cho, Showa Ward, Nagoya City, Aichi Prefecture, 466-8560, Japan.
| | - Yuichiro Sugiyama
- Department of Pediatrics, Japanese Red Cross Aichi Medical Center Nagoya Daiichi Hospital, Nagoya, Japan
| | - Yosuke Nishio
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Yukina Takamoto
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Fumie Kitai
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Yuma Takahashi
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Nozomi Hayashi
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Kazunori Haruta
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Maki Kondo
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Naoko Oike
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Takeshi Miwa
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | | | - Marei Omori
- Department of Pediatrics, Hekinan Municipal Hospital, Hekinan, Aichi, Japan
| | - Fumie Kinoshita
- Center for Advanced Medicine and Clinical Research, Department of Advanced Medicine, Nagoya University Hospital, 65 Tsurumai-Cho, Showa Ward, Nagoya City, Aichi Prefecture, 466-8560, Japan
- Data Coordinating Center, Department of Advanced Medicine, Nagoya University Hospital, Nagoya, Japan
| | - Taiki Furukawa
- Medical IT Center, Nagoya University Hospital, Nagoya, Japan
| | - Jun-Ichi Kawada
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Hiroyuki Kidokoro
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Yoshiaki Sato
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
- Division of Neonatology, Center for Maternal-Neonatal Care, Nagoya University Hospital, Nagoya, Japan
| | - Yoshiyuki Takahashi
- Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan
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Oni L, Platt C, Marlais M, McCann L, Barakat F, Hesseling M, Cottis H, Protheroe S, Haigh G, Nott K, Marro J, King E, Kelly J, Sussens J, Mulvaney S, Whitby T, Morgan I, Sharma A, Al-Jayyousi R, Cheung CK, Ng C, Lander AD, Simmons W, Melling C, Grandison R, Treitl L, Salama AD, Dudley J. National recommendations for the management of children and young people with IgA vasculitis: a best available evidence, group agreement-based approach. Arch Dis Child 2024; 110:67-76. [PMID: 39379139 PMCID: PMC11671997 DOI: 10.1136/archdischild-2024-327364] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/24/2024] [Accepted: 09/18/2024] [Indexed: 10/10/2024]
Abstract
OBJECTIVE IgA vasculitis (IgAV) is the most frequently experienced subtype of vasculitis seen in children. Most children fully recover, however, complications including chronic kidney disease are recognised. The aim of this project was to use a best available evidence, group agreement, based approach to develop national recommendations for the initial management of IgAV and its associated complications. METHODS A fully representative multiprofessional guideline development group (GDG), consisting of 28 members, was formed and met monthly. Graded recommendations were generated using nationally accredited methods, which included a predefined scope, open consultation, systematic literature review, evidence appraisal, review of national or international guidelines and a period of open consultation. Audit measures and research priorities were incorporated. RESULTS The IgAV GDG met over a 14-month period. A total of 82 papers were relevant for evidence synthesis. For the initial management, four topic areas were identified with five key questions generating six graded recommendations related to classification, specialist referral and musculoskeletal involvement. For the associated complications, five topic areas with 12 key questions generated 15 graded recommendations covering nephritis, gastrointestinal and testicular involvement, atypical disease and follow-up. Open consultation feedback was incorporated. The guidelines were endorsed by the UK Kidney Association and Royal College of Paediatrics and Child Health and are available online. CONCLUSION Despite IgAV being a rare disease with limited evidence, a national standardised approach to the clinical management for children and young people has been achieved. This should unite approaches to care and act as a foundation for improvement.
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Affiliation(s)
- Louise Oni
- Department of Women’s and Children’s Health, University of Liverpool, Liverpool, UK
- Department of Paediatric Nephrology, Alder Hey Children’s Hospital, Liverpool
| | - Caroline Platt
- Bristol Renal Unit, Bristol Royal Hospital for Children, Bristol, UK
| | - Matko Marlais
- Department of Paediatric Nephrology, Great Ormond Street Hospital, London, UK
| | - Liza McCann
- Department of Paediatric Rheumatology, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK
| | - Farah Barakat
- Kings College Hospital NHS Foundation Trust, London, UK
| | - Markus Hesseling
- Department of Paediatrics, Children’s health Ireland, Dublin, Ireland
| | - Hannah Cottis
- Department of Paediatrics, Royal Devon University Hospital, Devon, UK
| | - Sue Protheroe
- Department of Paediatric Gastroenterology, Birmingham Children’s Hospital, Birmingham, UK
| | - Gabrielle Haigh
- Department of Paediatrics, Betsi Cadwaladr Health Board, Wales, UK
| | - Kerstin Nott
- Department of Paediatric Rheumatology, Southampton Children’s Hospital, Southampton, UK
| | - Julien Marro
- University of Liverpool Medical School, Liverpool, UK
| | | | - Jane Kelly
- General Practice, Minchinhampton Surgery, Gloucestershire, UK
| | - Jill Sussens
- Nottingham Children's Hospital, Nottingham University Hospitals NHS Trust, Nottingham, UK
| | - Shirley Mulvaney
- Department of Paediatric Emergency Medicine, Alder Hey Children’s Hospital, Liverpool, UK
| | - Thomas Whitby
- General Paediatrics, Alder Hey Children's Hospital, Liverpool, Merseyside, UK
| | - Iona Morgan
- Department of Paediatrics, Royal Hospital for Children, Glasgow, UK
| | - Amita Sharma
- Department of Paediatrics, Royal Hospital for Children, Glasgow, UK
| | | | | | | | | | - William Simmons
- Department of Paediatric Pathology, Alder Hey Children’s Hospital, Liverpool, UK
| | - Charlotte Melling
- Department of Paediatric Surgery, Alder Hey Children’s Hospital, Liverpool, UK
| | | | | | - Alan D Salama
- Department of Renal Medicine, UCL Centre for Kidney and Bladder Health, London, UK
| | - Jan Dudley
- Bristol Renal Unit, Bristol Royal Hospital for Children, Bristol, UK
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Castañeda S, Quiroga-Colina P, Floranes P, Uriarte-Ecenarro M, Valero-Martínez C, Vicente-Rabaneda EF, González-Gay MA. IgA Vasculitis (Henoch-Schönlein Purpura): An Update on Treatment. J Clin Med 2024; 13:6621. [PMID: 39518760 PMCID: PMC11546386 DOI: 10.3390/jcm13216621] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/04/2024] [Revised: 10/27/2024] [Accepted: 11/01/2024] [Indexed: 11/16/2024] Open
Abstract
Objective: IgA vasculitis (IgAV), previously named as Henoch-Schönlein purpura, is the most frequent systemic vasculitis in children. In adults, IgAV is less common although it is associated with more severe disease. In fact, the frequency of glomerulonephritis (referred to as IgAV nephritis) in adults is higher than in children and tends to present more severely, with around 10-30% of those affected eventually progressing to end-stage renal disease. In this review, we describe the pathophysiology, main clinical features, diagnosis of the disease, and latest clinical data regarding IgAV therapy. Methods: A narrative literature review, primarily based on articles published in PubMed, was conducted. In addition to discussing the main aspects of glucocorticoids and conventional disease-modifying drugs used in the management of IgAV, this review focuses on the latest information reported regarding biologics and potential future therapies. Results: Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. Colchicine, dapsone, and methotrexate can be useful for controlling minor manifestations. Several immunomodulatory agents, such as cyclosporine A, tacrolimus, and mycophenolate mofetil, have shown favorable results as glucocorticoid-sparing agents. Leflunomide has shown promising results but requires further study. The use of rituximab has demonstrated efficacy in reducing relapse frequency, lowering the cumulative glucocorticoid burden, and achieving long-term remission of the disease in children and adults with IgAV. Immunoglobulins and plasma exchange therapy can also be useful in difficult and life-threatening situations. Other potential therapies with encouraging results include TRF-budesonide, B-cell-directed therapy, B-cell-depleting agents, sodium-glucose cotransporter-2 inhibitors, endothelin receptor antagonists, and complement pathway inhibitors. Conclusions: Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. The role of various immunomodulatory therapies, such as calcineurin inhibitors and mycophenolate mofetil, remains promising, while rituximab reduces the long-term side effects of glucocorticoids and can help achieve disease remission. Other potential therapies with encouraging results require further research.
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Affiliation(s)
- Santos Castañeda
- Rheumatology Division, H. Universitario La Princesa, IIS-Princesa, 28006 Madrid, Spain; (P.Q.-C.); (P.F.); (M.U.-E.); (C.V.-M.); (E.F.V.-R.)
- Department of Medicine, Universidad Autónoma de Madrid (UAM), 28049 Madrid, Spain
| | - Patricia Quiroga-Colina
- Rheumatology Division, H. Universitario La Princesa, IIS-Princesa, 28006 Madrid, Spain; (P.Q.-C.); (P.F.); (M.U.-E.); (C.V.-M.); (E.F.V.-R.)
| | - Paz Floranes
- Rheumatology Division, H. Universitario La Princesa, IIS-Princesa, 28006 Madrid, Spain; (P.Q.-C.); (P.F.); (M.U.-E.); (C.V.-M.); (E.F.V.-R.)
| | - Miren Uriarte-Ecenarro
- Rheumatology Division, H. Universitario La Princesa, IIS-Princesa, 28006 Madrid, Spain; (P.Q.-C.); (P.F.); (M.U.-E.); (C.V.-M.); (E.F.V.-R.)
| | - Cristina Valero-Martínez
- Rheumatology Division, H. Universitario La Princesa, IIS-Princesa, 28006 Madrid, Spain; (P.Q.-C.); (P.F.); (M.U.-E.); (C.V.-M.); (E.F.V.-R.)
| | - Esther F. Vicente-Rabaneda
- Rheumatology Division, H. Universitario La Princesa, IIS-Princesa, 28006 Madrid, Spain; (P.Q.-C.); (P.F.); (M.U.-E.); (C.V.-M.); (E.F.V.-R.)
- Department of Medicine, Universidad Autónoma de Madrid (UAM), 28049 Madrid, Spain
| | - Miguel A. González-Gay
- Department of Medicine and Psychiatry, School of Medicine, University of Cantabria, 39011 Santander, Spain
- Rheumatology Division, IIS-Fundación Jiménez Díaz, 28040 Madrid, Spain
- Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg 2000, South Africa
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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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7
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Younger DS. Headaches and Vasculitis. Neurol Clin 2024; 42:389-432. [PMID: 38575258 DOI: 10.1016/j.ncl.2023.12.003] [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: 04/06/2024]
Abstract
Vasculitis refers to heterogeneous clinicopathologic disorders that share the histopathology of inflammation of blood vessels. Unrecognized and therefore untreated, vasculitis of the nervous system leads to pervasive injury and disability making this a disorder of paramount importance to all clinicians. Headache may be an important clue to vasculitic involvement of central nervous system (CNS) vessels. CNS vasculitis may be primary, in which only intracranial vessels are involved in the inflammatory process, or secondary to another known disorder with overlapping systemic involvement. Primary neurologic vasculitides can be diagnosed with assurance after intensive evaluation that incudes tissue confirmation whenever possible.
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Affiliation(s)
- David S Younger
- Department of Medicine, Section of Neuroscience, City University of New York School of Medicine, New York, NY, USA; Department of Neurology, White Plains Hospital, White Plains, NY, USA.
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Levanon S, Gotloib V, Kraus Y, Novofastovski I, Brikman S, Fawaz A, Egbaria M, Butbul Aviel Y, Balbir-Gurman A, Mader R, Bieber A. IgA vasculitis in adults, pediatrics and non-vasculitic IgA nephropathy, retrospective analysis from 2 centers. Medicine (Baltimore) 2023; 102:e36521. [PMID: 38115301 PMCID: PMC10727533 DOI: 10.1097/md.0000000000036521] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/26/2023] [Revised: 11/03/2023] [Accepted: 11/13/2023] [Indexed: 12/21/2023] Open
Abstract
Renal involvement represents the major long-term morbidity associated with IgA vasculitis (IgAV). Our aim was to evaluate clinical characteristics and long-term renal outcomes of IgAV in pediatrics and adults comparing to IgA nephropathy (IgAN). Our retrospective study included children and adults with IgAV and IgAN patients, admitted in a 13-year period (2007-2019) to rheumatology clinics and in hospital pediatric and internal medicine departments. We compared frequencies of clinical manifestations, laboratory findings, treatments, long-term outcomes at 1 year follow-up, including all-cause mortality and dialysis until the end of follow-up time. A total of 60 adult IgAV, 60 pediatric IgAV and 45 IgAN patients were evaluated. Adult IgAV patients were significantly older than IgAN patients (53.1 ± 17.4 years vs 45.1 ± 15.7 years respectively, P = .02) and had significantly higher rates of cardiovascular comorbidities. The risk and time to dialysis were similar among IgAN and adult IgAV groups. Yet, overall mortality at long term follow up was higher in IgAV adult group compared to IgAN. No dialysis or renal transplantation were reported in pediatric IgAV patients. IgAV and IgAN adult patients were comparable regarding risk of end stage renal disease. Of note, high mortality rates were observed among adult IgAV group.
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Affiliation(s)
| | - Vera Gotloib
- Pediatric Rheumatology Service, Emek Medical Center, Afula, Israel
| | | | | | - Shay Brikman
- Rheumatology, Emek Medical Center, Afula, Israel
- Rappaport Faculty of Medicine, Technion, Haifa, Israel
| | | | | | - Yonatan Butbul Aviel
- Rappaport Faculty of Medicine, Technion, Haifa, Israel
- The Ruth Rappaport Children’s Hospital, Pediatric Rheumatology Service, Haifa, Israel
| | - Alexandra Balbir-Gurman
- Rappaport Faculty of Medicine, Technion, Haifa, Israel
- Rambam Health Care Campus, Rheumatology, Haifa, Israel
| | - Reuven Mader
- Rheumatology, Emek Medical Center, Afula, Israel
- Rappaport Faculty of Medicine, Technion, Haifa, Israel
| | - Amir Bieber
- Rheumatology, Emek Medical Center, Afula, Israel
- Rappaport Faculty of Medicine, Technion, Haifa, Israel
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9
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Younger DS. Systemic vasculitis and headache. Curr Opin Neurol 2023; 36:631-646. [PMID: 37865837 PMCID: PMC10624412 DOI: 10.1097/wco.0000000000001223] [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: 10/23/2023]
Abstract
PURPOSE OF REVIEW Vasculitis refers to heterogeneous clinicopathologic disorders that share the histopathology of inflammation of blood vessels. Unrecognized and therefore untreated, vasculitis of the nervous system or so called neurovasculitides, lead to pervasive injury and disability making these disorder of paramount importance to clinicians. RECENT FINDINGS Headache is an important clue to vasculitic involvement of central nervous system (CNS) vessels. CNS vasculitis may be primary, in which only intracranial vessels are involved in the inflammatory process, or secondary to another known disorder with overlapping systemic involvement. A suspicion of vasculitis based on the history, clinical examination, or laboratory studies warrants prompt evaluation and treatment to forestall progression and avert cerebral ischemia or infarction. There has been remarkable progress in the pathogenesis, diagnosis, and treatment of primary adult and pediatric CNS vasculitides predicated on achievements in primary systemic forms. SUMMARY Vasculitis can be diagnosed with certainty after intensive evaluation that includes tissue confirmation whenever possible. Clinicians must choose from among the available immune modulating, suppressive, and targeted immunotherapies to induce and maintain remission status and prevent relapse, tempered by the recognition of anticipated medication side effects.
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Affiliation(s)
- David S Younger
- Department of Medicine, Section of Neuroscience, City University of New York School of Medicine, New York, NY; Department of Neurology, White Plains Hospital, White Plains, New York, USA
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10
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Rosenberg S, Sweetser P, Ismail L. Vomiting and Abdominal Pain in a 9-year-old Boy. Pediatr Rev 2023; 44:S103-S105. [PMID: 37777227 DOI: 10.1542/pir.2021-005385] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/02/2023]
Affiliation(s)
- Sedona Rosenberg
- University of Virginia School of Medicine, Department of Ophthalmology, Charlottesville, VA
| | - Peter Sweetser
- George Washington University School of Medicine and Health Sciences, Washington, DC
| | - Lana Ismail
- Children's National Hospital, Division of Hospital Medicine, Washington, DC
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11
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Guo H, Wang ZL, Tao Z. Delayed diagnosis of abdominal Henoch-Schonlein purpura in children: A case report. World J Clin Cases 2023; 11:6311-6317. [PMID: 37731573 PMCID: PMC10507560 DOI: 10.12998/wjcc.v11.i26.6311] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/10/2023] [Revised: 08/11/2023] [Accepted: 08/11/2023] [Indexed: 09/08/2023] Open
Abstract
BACKGROUND For children with abdominal Henoch-Schonlein purpura presenting abdominal pain as an initial symptom and severe clinical manifestations, but without purpura appearance on the skin, the diagnosis and treatment are relatively difficult. This study summarized the characteristics of this group of patients by literature review and provided additional references for further refinement of glucocorticoid therapy in this vasculitis. CASE SUMMARY A 6-year-old girl presented mainly with repeated abdominal pain and had received short-term out-of-hospital treatment with hydrocortisone. On day 7 after onset, gastroscopy revealed chronic non-atrophic gastritis and erosive duodenitis without purpuric rash, and no obvious resolution of the abdominal pain was found after treatment against infection and for protection of gastric mucosa. On day 14 the inflammatory indices continued to rise and the pain was relieved after enhanced anti-infective therapy, but without complete resolution. On day 19, the patient presented with aggravated abdominal pain with purplish-red dots on the lower limbs, by which Henoch-Schonlein purpura was confirmed. After 5 d of sequential treatment with methylprednisolone and prednisone, abdominal pain disappeared and she was discharged. CONCLUSION Henoch-Schonlein purpura-related rash may appear after long-term abdominal pain, and should be distinguished from acute and chronic gastrointestinal diseases at the early stage without typical rash. For bacterial infection-induced Henoch-Schonlein purpura, glucocorticoid therapy alone without clearing the infection may not relieve symptoms.
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Affiliation(s)
- Hui Guo
- Department of Pediatrics, West China Second University Hospital, Chengdu 610041, Sichuan Province, China
| | - Zhi-Ling Wang
- Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Chengdu 610000, Sichuan Province, China
| | - Zhu Tao
- Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Chengdu 610000, Sichuan Province, China
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Feng K, Liu C, Zhang K, Hao J. Successful treatment of Henoch-Schönlein purpura-associated hematochezia in a child with hemophilia A: a case report. BMC Pediatr 2023; 23:98. [PMID: 36859289 PMCID: PMC9979527 DOI: 10.1186/s12887-023-03874-w] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/07/2022] [Accepted: 01/27/2023] [Indexed: 03/03/2023] Open
Abstract
BACKGROUND Henoch-Schönlein purpura (HSP) is a common form of immunological vasculitis in children. Hemophilia A is a genetic disorder and characterized by spontaneous hemorrhage or prolonged bleeding due to factor VIII deficiency. Both diseases increase the risk of bleeding, but they have different mechanisms. How should we treat patients with both diseases? CASE PRESENTATION An 8-year-old male with hemophilia A was diagnosed with HSP while receiving coagulation factor VIII replacement therapy in our hospital. Hematochezia occurred 6 days after the diagnosis of HSP. And he treated with coagulation FVIII, methylprednisolone and hemostatic drugs. CONCLUSIONS There is no causal relationship between hemophilia A and HSP, but both diseases can cause bleeding. This child's hematochezia was caused by HSP, but hemophilia could not be ignored during the treatment. Our case report adds to the present body of knowledge about the treatment of HSP associated hematochezia in a child with hemophilia A.
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Affiliation(s)
- Kai Feng
- Department of Traditional Chinese Medicine, Beijing Children's Hospital, Capital Medicine University, National Center for Children's Health, No.56 Nanlishi Road, Beijing, 100045, China.
| | - Chang Liu
- Department of Traditional Chinese Medicine, Beijing Children's Hospital, Capital Medicine University, National Center for Children's Health, No.56 Nanlishi Road, Beijing, 100045, China
| | - Keqing Zhang
- Department of Traditional Chinese Medicine, Beijing Children's Hospital, Capital Medicine University, National Center for Children's Health, No.56 Nanlishi Road, Beijing, 100045, China
| | - Jing Hao
- Department of Traditional Chinese Medicine, Beijing Children's Hospital, Capital Medicine University, National Center for Children's Health, No.56 Nanlishi Road, Beijing, 100045, China
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Abstract
BACKGROUND IgA vasculitis (IgAV), previously known as Henoch-Schönlein purpura, is the most common vasculitis of childhood but may also occur in adults. This small vessel vasculitis is characterised by palpable purpura, abdominal pain, arthritis or arthralgia and kidney involvement. This is an update of a review first published in 2009 and updated in 2015. OBJECTIVES To evaluate the benefits and harms of different agents (used singularly or in combination) compared with placebo, no treatment or any other agent for (1) the prevention of severe kidney disease in people with IgAV with or without kidney involvement at onset, (2) the treatment of established severe kidney disease (macroscopic haematuria, proteinuria, nephritic syndrome, nephrotic syndrome with or without acute kidney failure) in IgAV, and (3) the prevention of recurrent episodes of IgAV-associated kidney disease. SEARCH METHODS We searched the Cochrane Kidney and Transplant Register of Studies up to 2 February 2023 through contact with the Information Specialist using search terms relevant to this review. Studies in the Register are identified through searches of CENTRAL, MEDLINE, and EMBASE, conference proceedings, the International Clinical Trials Register (ICTRP) Search Portal and ClinicalTrials.gov. SELECTION CRITERIA Randomised controlled trials (RCTs) comparing interventions used to prevent or treat kidney disease in IgAV compared with placebo, no treatment or other agents were included. DATA COLLECTION AND ANALYSIS Two authors independently determined study eligibility, assessed the risk of bias and extracted data from each study. Statistical analyses were performed using the random-effects model, and the results were expressed as risk ratio (RR) for dichotomous outcomes and mean difference (MD) for continuous outcomes with 95% confidence intervals (CI). Confidence in the evidence was assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. MAIN RESULTS Twenty studies (1963 enrolled participants) were identified; one three-arm study has been assessed as two studies. Nine studies were at low risk of bias for sequence generation (selection bias), and nine studies were at low risk of bias for allocation concealment (selection bias). Blinding of participants and personnel (performance bias) and outcome assessment (detection bias) was at low risk of bias in four and seven studies, respectively. Nine studies reported complete outcome data (attrition bias), while 10 studies reported expected outcomes, so were at low risk of reporting bias. Five studies were at low risk of other bias. Eleven studies evaluated therapy to prevent persistent kidney disease in IgAV with or without kidney involvement at presentation. There was probably no difference in the risk of persistent kidney disease any time after treatment (5 studies, 746 children: RR 0.74, 95% CI 0.42 to 1.32) or at one, three, six and 12 months in children given prednisone for 14 to 28 days at presentation of IgAV compared with placebo or supportive treatment (moderate certainty evidence). There may be no differences in the risk of any persistent kidney disease with antiplatelet therapy (three studies) or heparin (two studies) in children with or without any kidney disease at study entry, although heparin may reduce the risk of proteinuria by three months compared with placebo or no specific treatment (2 studies, 317 children: RR 0.47, 95% CI 0.31 to 0.73). One study comparing montelukast with placebo found no differences in outcomes as assessed by severity scale scores. Nine studies examined the treatment of severe IgAV-associated kidney disease. In two studies (one involving 56 children and the other involving 54 adults), there may be no differences in efficacy outcomes or adverse effects with cyclophosphamide compared with placebo or supportive treatment. In two studies, there may be no differences in the numbers achieving remission of proteinuria with intravenous (IV) cyclophosphamide compared with mycophenolate mofetil (MMF) (65 children evaluated) or tacrolimus (142 children evaluated). In three small studies comparing cyclosporin with methylprednisolone (15 children), MMF with azathioprine (26 children), or MMF with leflunomide (19 children), it is unclear whether the treatment had any effect on the numbers in remission or the degree of proteinuria between treatment groups because of small numbers of included participants. In one study comparing plasmapheresis, cyclophosphamide and methylprednisolone with cyclophosphamide and methylprednisolone, there may be no difference in the numbers achieving remission. One study compared fosinopril with no specific therapy and reported fosinopril reduced the number of participants with proteinuria. No studies were identified that evaluated the efficacy of therapy on kidney disease in participants with recurrent episodes of IgAV. AUTHORS' CONCLUSIONS There are no substantial changes in conclusions from this update compared with the initial review or the previous update despite the addition of five studies. From generally low to moderate certainty evidence, we found that there may be little or no benefit in the use of corticosteroids or antiplatelet agents to prevent persistent kidney disease in children with IgAV in participants with no or minimal kidney involvement at presentation. We did not find any studies which evaluated corticosteroids in children presenting with IgAV and nephritic and/or nephrotic syndrome, although corticosteroids are recommended in such children in guidelines. Though heparin may be effective in reducing proteinuria, this potentially dangerous therapy is not justified to prevent serious kidney disease when few children with IgAV develop severe kidney disease. There may be no benefit of cyclophosphamide compared with no specific treatment or corticosteroids. While there may be no benefit in the efficacy of MMF or tacrolimus compared with IV cyclophosphamide in children or adults with IgAV and severe kidney disease, adverse effects, particularly infections, may be lower in MMF or tacrolimus-treated children. Because of small patient numbers and events leading to imprecision in results, it remains unclear whether cyclosporin, MMF or leflunomide have any role in the treatment of children with IgAV and severe kidney disease. We did not identify any studies which evaluated corticosteroids.
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Affiliation(s)
- Deirdre Hahn
- Department of Nephrology, The Children's Hospital at Westmead, Westmead, Australia
| | - Elisabeth M Hodson
- Sydney School of Public Health, The University of Sydney, Sydney, Australia
- Cochrane Kidney and Transplant, Centre for Kidney Research, The Children's Hospital at Westmead, Westmead, Australia
| | - Jonathan C Craig
- Cochrane Kidney and Transplant, Centre for Kidney Research, The Children's Hospital at Westmead, Westmead, Australia
- College of Medicine and Public Health, Flinders University, Adelaide, Australia
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14
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Popov H, Koleva T, Stoyanov GS. Bullous Henoch-Schönlein Purpura and Associated Nephritis: A Pathological Case Report. Cureus 2023; 15:e35051. [PMID: 36942172 PMCID: PMC10024341 DOI: 10.7759/cureus.35051] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/16/2023] [Indexed: 02/18/2023] Open
Abstract
Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood, presenting with purpura, predominantly of the lower extremities and occasionally with renal involvement as well. Although associated with childhood, HSP, although rarely, can also develop in adults as well. Here we present a patient in his sixties, presenting with a myriad of rash units on his lower extremities, including bullous ones, and a constellation of chronic kidney failure. Skin and renal biopsy specimens revealed morphological changes and immune depositions representative of HSP. Despite treatment, the patient's kidney failure slowly progressed, and he expired eight months after his presentation due to associated complications. Although rare, the bullous form of HSP can be viewed as a more aggressive form of the disease, as seen by the presentation constellation and rapid progression in our case.
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Affiliation(s)
- Hristo Popov
- General and Clinical Pathology, Forensic Medicine and Deontology, Medical University of Varna, Varna, BGR
| | | | - George S Stoyanov
- General and Clinical Pathology, St. Marina University Hospital, Varna, BGR
- General and Clinical Pathology, Forensic Medicine and Deontology, Medical University of Varna, Varna, BGR
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15
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Girao A, Fernandes JA, Mira FS, Pina R. Immunoglobulin a (IgA) Vasculitis in the Elderly. Cureus 2023; 15:e34422. [PMID: 36874655 PMCID: PMC9978861 DOI: 10.7759/cureus.34422] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/30/2023] [Indexed: 02/02/2023] Open
Abstract
IgA vasculitis is a small vessel vasculitis mediated by the deposition of IgA immune complexes. It mostly occurs in children and is rare in adults, with increased severity and mortality in the latter. Its aetiology remains largely unknown, and its prognosis depends primarily on the extent of renal involvement. We present the case of a 71-year-old woman with purpuric lesions in both lower and upper limbs associated with fever, abdominal pain, vomiting and blood in her stools for the past month. The patient was diagnosed with IgA vasculitis and the full systemic involvement (renal, dermatological, intestinal, and cerebral) of the disease was identified with excellent response to parenteral corticotherapy.
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Affiliation(s)
- Adriana Girao
- Internal Medicine Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, PRT
| | - José A Fernandes
- Internal Medicine Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, PRT
| | - Filipe S Mira
- Nephrology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, PRT
| | - Rui Pina
- Internal Medicine Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, PRT
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16
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Younger DS. Adult and childhood vasculitis. HANDBOOK OF CLINICAL NEUROLOGY 2023; 195:653-705. [PMID: 37562892 DOI: 10.1016/b978-0-323-98818-6.00008-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 08/12/2023]
Abstract
Vasculitis refers to heterogeneous clinicopathologic disorders that share the histopathology of inflammation of blood vessels. Unrecognized and therefore untreated, vasculitis of the nervous system leads to pervasive injury and disability, making this a disorder of paramount importance to all clinicians. There has been remarkable progress in the pathogenesis, diagnosis, and treatment of primary CNS and PNS vasculitides, predicated on achievement in primary systemic forms. Primary neurological vasculitides can be diagnosed with assurance after intensive evaluation that incudes tissue confirmation whenever possible. Clinicians must choose from among the available immune modulating, suppressive, and targeted immunotherapies to induce and maintain remission status and prevent relapse, unfortunately without the benefit of RCTs, and tempered by the recognition of anticipated medication side effects. It may be said that efforts to define a disease are attempts to understand the very concept of the disease. This has been especially evident in systemic and neurological disorders associated with vasculitis. For the past 100 years, since the first description of granulomatous angiitis of the brain, the CNS vasculitides have captured the attention of generations of clinical investigators around the globe to reach a better understanding of vasculitides involving the central and peripheral nervous system. Since that time it has become increasingly evident that this will necessitate an international collaborative effort.
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Affiliation(s)
- David S Younger
- Department of Clinical Medicine and Neuroscience, CUNY School of Medicine, New York, NY, United States; Department of Medicine, Section of Internal Medicine and Neurology, White Plains Hospital, White Plains, NY, United States.
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17
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Kallash M, Vogt BA, Zeid A, Khin E, Najjar M, Aldughiem A, Benoit E, Stotter B, Rheault M, Warejko JK, Daga A. The scope of treatment of pediatric IgA vasculitis nephritis and its outcome: a Pediatric Nephrology Research Consortium study. Pediatr Nephrol 2022; 37:2687-2697. [PMID: 35233641 DOI: 10.1007/s00467-022-05496-3] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/23/2021] [Revised: 02/02/2022] [Accepted: 02/02/2022] [Indexed: 11/24/2022]
Abstract
BACKGROUND IgA vasculitis (IgAV) is the most common type of vasculitis in children. There is a lack of consensus for management of significant IgAV nephritis (IgAVN). This study was designed to identify the most used treatment options and describe their efficacy. METHODS This is a multicenter retrospective study of children age 1-21 years with IgAVN who were managed for at least 6 months by a nephrologist. Subjects with at least microscopic hematuria and proteinuria and/or decreased kidney function were enrolled. Kidney outcome was assessed by eGFR and urine protein/creatinine (UPC) ratios at 2-4 weeks, 3, 6, and 12 months post-diagnosis. RESULTS A total of 128 subjects with median age of 7 years (range 2-18) were included. Of these, 69 subjects had kidney biopsy with crescents detected in 53%. AKI (P = 0.039), nephrosis (P = 0.038), and crescents on biopsy (P = 0.013) were more likely in older patients. Patients with UPC > 1 mg/mg were more likely to get a kidney biopsy (P < 0.001) and to be treated with steroids ± immunosuppressive (IS) agents (P = 0.001). Sixty-six percent of patients were treated with steroids and/or IS agents for variable durations. Anti-metabolite agents were the most common IS agents used with variability in dosing and duration. At 12 months, most subjects had a normal eGFR (79%) (median 123, range 68-207 mL/min/1.73 m2) and no proteinuria (median UPC 0.15, range 0.01-4.02 mg/mg). CONCLUSIONS IS agents are frequently used in managing IgAVN associated with heavy proteinuria, nephrosis, and/or AKI. Prospective studies are needed to determine indications and needed duration of IS therapy. A higher resolution version of the Graphical abstract is available as Supplementary information.
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Affiliation(s)
- Mahmoud Kallash
- Section of Pediatric Nephrology, The Ohio State University College of Medicine, Nationwide Children's Hospital, Columbus, OH, USA.
| | - Beth A Vogt
- Section of Pediatric Nephrology, The Ohio State University College of Medicine, Nationwide Children's Hospital, Columbus, OH, USA
| | - Ahmed Zeid
- Section of Pediatric Nephrology, The Ohio State University College of Medicine, Nationwide Children's Hospital, Columbus, OH, USA
| | - Ei Khin
- Section of Pediatric Nephrology, Texas Tech University Health Science Center El Paso, El Paso, TX, USA
| | - Mohammed Najjar
- Section of Pediatrics, The Ohio State University College of Medicine, Nationwide Children's Hospital, Columbus, OH, USA
| | - Ahmad Aldughiem
- Section of Pediatric Nephrology, Dayton Children's Hospital, Dayton, OH, USA
| | - Elizabeth Benoit
- Boston Children's Hospital, Harvard Medical School, Boston, MA, USA
| | - Brian Stotter
- Division of Nephrology, Hypertension, and Pheresis, Department of Pediatrics, Washington University School of Medicine and St. Louis Children's Hospital, St. Louis, MO, USA
| | - Michelle Rheault
- Division of Pediatric Nephrology, University of Minnesota Masonic Children's Hospital, Minneapolis, MN, USA
| | - Jillian K Warejko
- Section of Pediatric Nephrology, Yale University School of Medicine, New Haven, CT, USA
| | - Ankana Daga
- Pediatric Nephrology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA
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18
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Son MBF, Berbert L, Young C, Dallas J, Newhams M, Chen S, Ardoin SP, Basiaga ML, Canny SP, Crandall H, Dhakal S, Dhanrajani A, Sagcal-Gironella ACP, Hobbs CV, Huie L, James K, Jones M, Kim S, Lionetti G, Mannion ML, Muscal E, Prahalad S, Schulert GS, Tejtel KS, Villacis-Nunez DS, Wu EY, Zambrano LD, Campbell AP, Patel MM, Randolph AG. Postdischarge Glucocorticoid Use and Clinical Outcomes of Multisystem Inflammatory Syndrome in Children. JAMA Netw Open 2022; 5:e2241622. [PMID: 36367723 PMCID: PMC9652757 DOI: 10.1001/jamanetworkopen.2022.41622] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/13/2022] Open
Abstract
IMPORTANCE Minimal data are available regarding the postdischarge treatment of multisystem inflammatory syndrome in children (MIS-C). OBJECTIVES To evaluate clinical characteristics associated with duration of postdischarge glucocorticoid use and assess postdischarge clinical course, laboratory test result trajectories, and adverse events in a multicenter cohort with MIS-C. DESIGN, SETTING, AND PARTICIPANTS This retrospective cohort study included patients with MIS-C hospitalized with severe illness and followed up for 3 months in an ambulatory setting. Patients younger than 21 years who were admitted between May 15, 2020, and May 31, 2021, at 13 US hospitals were included. Inclusion criteria were inpatient treatment comprising intravenous immunoglobulin, diagnosis of cardiovascular dysfunction (vasopressor requirement or left ventricular ejection fraction ≤55%), and availability of complete outpatient data for 3 months. EXPOSURES Glucocorticoid treatment. MAIN OUTCOMES AND MEASURES Main outcomes were patient characteristics associated with postdischarge glucocorticoid treatment, laboratory test result trajectories, and adverse events. Multivariable regression was used to evaluate factors associated with postdischarge weight gain (≥2 kg in 3 months) and hyperglycemia during illness. RESULTS Among 186 patients, the median age was 10.4 years (IQR, 6.7-14.2 years); most were male (107 [57.5%]), Black non-Hispanic (60 [32.3%]), and Hispanic or Latino (59 [31.7%]). Most children were critically ill (intensive care unit admission, 163 [87.6%]; vasopressor receipt, 134 [72.0%]) and received inpatient glucocorticoid treatment (178 [95.7%]). Most were discharged with continued glucocorticoid treatment (173 [93.0%]); median discharge dose was 42 mg/d (IQR, 30-60 mg/d) or 1.1 mg/kg/d (IQR, 0.7-1.7 mg/kg/d). Inpatient severity of illness was not associated with duration of postdischarge glucocorticoid treatment. Outpatient treatment duration varied (median, 23 days; IQR, 15-32 days). Time to normalization of C-reactive protein and ferritin levels was similar for glucocorticoid duration of less than 3 weeks vs 3 or more weeks. Readmission occurred in 7 patients (3.8%); none was for cardiovascular dysfunction. Hyperglycemia developed in 14 patients (8.1%). Seventy-five patients (43%) gained 2 kg or more after discharge (median 4.1 kg; IQR, 3.0-6.0 kg). Inpatient high-dose intravenous and oral glucocorticoid therapy was associated with postdischarge weight gain (adjusted odds ratio, 6.91; 95% CI, 1.92-24.91). CONCLUSIONS AND RELEVANCE In this multicenter cohort of patients with MIS-C and cardiovascular dysfunction, postdischarge glucocorticoid treatment was often prolonged, but clinical outcomes were similar in patients prescribed shorter courses. Outpatient weight gain was common. Readmission was infrequent, with none for cardiovascular dysfunction. These findings suggest that strategies are needed to optimize postdischarge glucocorticoid courses for patients with MIS-C.
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Affiliation(s)
- Mary Beth F. Son
- Division of Immunology, Boston Children’s Hospital, Boston, Massachusetts
- Department of Pediatrics, Harvard Medical School, Boston, Massachusetts
| | - Laura Berbert
- Institute Centers for Clinical and Translational Research, Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts
| | - Cameron Young
- Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children’s Hospital, Boston, Massachusetts
| | - Johnathan Dallas
- Division of Immunology, Boston Children’s Hospital, Boston, Massachusetts
- Western Atlantic University School of Medicine, Freeport, Grand Bahama, Bahamas
| | - Margaret Newhams
- Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children’s Hospital, Boston, Massachusetts
| | - Sabrina Chen
- Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children’s Hospital, Boston, Massachusetts
| | - Stacy P. Ardoin
- Rheumatology, Nationwide Children’s Hospital, Department of Pediatrics, Ohio State College of Medicine, Columbus
| | - Matthew L. Basiaga
- Division of Pediatric Rheumatology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota
| | - Susan P. Canny
- Division of Pediatric Rheumatology, Seattle Children’s Hospital, Department of Pediatrics, University of Washington, Seattle
| | - Hillary Crandall
- Division of Pediatric Critical Care, Department of Pediatrics, University of Utah, Primary Children’s Hospital, Salt Lake City
| | - Sanjeev Dhakal
- Division of Rheumatology, Cincinnati Children’s Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio
| | - Anita Dhanrajani
- Pediatric Rheumatology, Department of Pediatrics, University of Mississippi Medical Center, Jackson
| | - Anna Carmela P. Sagcal-Gironella
- Department of Pediatrics, Hackensack Meridian School of Medicine, Hackensack, New Jersey
- Division of Rheumatology, Joseph M. Sanzari Children’s Hospital, Hackensack, New Jersey
| | - Charlotte V. Hobbs
- Division of Pediatric Infectious Disease, Department of Pediatrics, University of Mississippi Medical Center, Jackson
| | - Livie Huie
- Division of Pediatric Rheumatology, University of Alabama at Birmingham
| | - Karen James
- Division of Pediatric Rheumatology, Department of Pediatrics, University of Utah, Primary Children’s Hospital, Salt Lake City
| | - Madelyn Jones
- Rheumatology, Nationwide Children’s Hospital, Department of Pediatrics, Ohio State College of Medicine, Columbus
| | - Susan Kim
- Pediatric Rheumatology, UCSF Benioff Children’s Hospital, Department of Pediatrics, University of California at San Francisco School of Medicine
| | - Geraldina Lionetti
- Pediatric Rheumatology, UCSF Benioff Children’s Hospital, Department of Pediatrics, University of California at San Francisco School of Medicine
| | | | - Eyal Muscal
- Division of Rheumatology, Texas Children’s Hospital, Department of Pediatrics, Baylor School of Medicine, Houston
| | - Sampath Prahalad
- Children’s Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia
| | - Grant S. Schulert
- Division of Rheumatology, Cincinnati Children’s Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio
| | - Kristen Sexson Tejtel
- Division of Cardiology, Texas Children’s Hospital, Department of Pediatrics, Baylor School of Medicine, Houston
| | - D. Sofia Villacis-Nunez
- Children’s Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia
| | - Eveline Y. Wu
- Division of Pediatric Rheumatology, UNC Children’s Hospital, Department of Pediatrics, University of North Carolina School of Medicine, Chapel Hill
| | - Laura D. Zambrano
- COVID-19 Response Team, Centers for Disease Control and Prevention, Atlanta, Georgia
| | - Angela P. Campbell
- COVID-19 Response Team, Centers for Disease Control and Prevention, Atlanta, Georgia
| | - Manish M. Patel
- COVID-19 Response Team, Centers for Disease Control and Prevention, Atlanta, Georgia
- Public Health Service Commissioned Corps, Rockville, Maryland
| | - Adrienne G. Randolph
- Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children’s Hospital, Boston, Massachusetts
- Departments of Pediatrics and Anesthesiology, Harvard Medical School, Boston, Massachusetts
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Nishikura N, Ohta R, Katagiri N, Okayasu T, Sano C. Refractory Immunoglobulin A (IgA) Vasculitis in an Elderly Patient: A Case Report. Cureus 2022; 14:e28996. [PMID: 36249629 PMCID: PMC9549259 DOI: 10.7759/cureus.28996] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/09/2022] [Indexed: 11/24/2022] Open
Abstract
Immunoglobulin A (IgA) vasculitis is small-vessel arteritis triggered by autoimmunity and allergies. IgA vasculitis among elderly patients is rare, and there is a lack of evidence regarding the choice of medicine and treatment duration. The main treatment for IgA vasculitis is steroids which can be cured with a small dose of prednisolone without immunosuppressants. Here, we report a case of a 90-year-old patient with the chief complaint of appetite loss and purpura on the legs who was diagnosed with IgA vasculitis based on biopsy results. The patient was initially treated with prednisolone effectively but exacerbated with steroid tapering, eventually requiring the use of an immunosuppressant. This case highlights the importance of monitoring the symptoms of IgA vasculitis while tapering steroids and clarifying the timing of immunosuppressant initiation.
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20
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Affiliation(s)
- Clement D Lee
- From the Children's Hospital of Philadelphia, Philadelphia
| | - Evan Shirey
- From the Children's Hospital of Philadelphia, Philadelphia
| | | | - Chén C Kenyon
- From the Children's Hospital of Philadelphia, Philadelphia
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21
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Sood R, Parekh P, Raj N, Saani I. Case Report: An Adult Presentation of Henoch-Schönlein Purpura. Cureus 2022; 14:e26385. [PMID: 35923669 PMCID: PMC9339340 DOI: 10.7759/cureus.26385] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/28/2022] [Indexed: 12/05/2022] Open
Abstract
Henoch-Schönlein purpura (HSP) is an immunoglobulin A (IgA)-mediated multisystem vasculitis commonly affecting children under 10 years of age. Although diagnostic criteria exist, making a diagnosis is often difficult as this condition can present atypically in adults. We discuss a 22-year-old female with a delayed diagnosis of HSP, resulting in significant anxiety and distress. Our patient’s symptoms improved with analgesia and corticosteroids, which were initiated upon diagnosis and she experienced two mild, self-limiting relapses over two years following symptom resolution. Our case illustrates that an integrated multidisciplinary approach is needed to effectively diagnose, safely manage and monitor patients presenting with HSP. Although self-limiting in nature, HSP has the potential to manifest into life-threatening conditions such as end-stage renal failure, which stresses the importance of early diagnosis and management.
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22
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Lectin and alternative complement pathway activation in cutaneous manifestations of IgA-vasculitis: A new target for therapy? Mol Immunol 2022; 143:114-121. [DOI: 10.1016/j.molimm.2022.01.011] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2021] [Revised: 01/21/2022] [Accepted: 01/23/2022] [Indexed: 11/18/2022]
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Wang S, Tang H, Du W, Ding Y. Massive gastrointestinal hemorrhage caused by Henoch-Schoenlein purpura: A case report. Medicine (Baltimore) 2021; 100:e28240. [PMID: 34918691 PMCID: PMC8677960 DOI: 10.1097/md.0000000000028240] [Citation(s) in RCA: 2] [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] [Received: 10/20/2021] [Accepted: 11/24/2021] [Indexed: 01/05/2023] Open
Abstract
RATIONALE Henoch-Schoenlein purpura (HSP) is a systemic small-vessel vasculitis that commonly occurs in children. Gastrointestinal HSP can rarely progress to gastrointestinal perforation, followed by massive gastrointestinal bleeding. PATIENT CONCERNS An 8-year-old Chinese boy was transferred to the pediatric intensive care unit of our hospital with an emergency occurrence of purpura, severe hematemesis, large bloody stools, and sharp abdominal pain, and complained of abdominal pain and rash 2 weeks prior. DIAGNOSIS The patient had purpura with lower limb predominance, abdominal pain, and gastrointestinal bleeding. Immunofluorescence microscopy of histological sections showed granular and lumpy IgA focal deposition in the blood vessel walls. He was diagnosed with HSP. INTERVENTIONS Initially, he was treated with methylprednisolone, posterior pituitary injection, somatostatin, and hemocoagulase, together with the infusion of large blood products. Postoperatively, he was administered nasal continuous positive airway pressure -assisted ventilation, anti-infection treatment, albumin transfusion, platelet transfusion, abdominal drainage, methylprednisolone, fluconazole anti-fungal treatment, and wound dressing. OUTCOMES There was no evidence of rebleeding, abdominal pain, or purpura at the 2-month follow-up assessment. LESSONS Abdominal HSP should be alert to gastrointestinal perforation when using hormone therapy.
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Cannon L, Wu EY. Recent Advances in Pediatric Vasculitis. Rheum Dis Clin North Am 2021; 47:781-796. [PMID: 34635304 DOI: 10.1016/j.rdc.2021.07.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Abstract
This article provides an overview of the clinical presentation and diagnosis of select pediatric primary systemic vasculitides. Important advances in understanding the pathogenesis of these rare diseases also are discussed and efforts to harmonize treatment through consensus-based guidelines and multicenter and international collaborations highlighted.
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Affiliation(s)
- Laura Cannon
- Division of Pediatric Rheumatology, Department of Pediatrics, Duke University, 2301 Erwin Road, DUMC Box 3212, Durham, NC 27710, USA
| | - Eveline Y Wu
- Division of Pediatric Rheumatology, Department of Pediatrics, The University of North Carolina Chapel Hill, 030 MacNider Hall, CB #7231, Chapel Hill, NC 27599, USA; Division of Allergy/Immunology, Department of Pediatrics, The University of North Carolina Chapel Hill, Chapel Hill, NC.
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IgA Vasculitis: a Review and Update on the Management of Renal and Extrarenal Disease, Highlighting What’s New for Biomarkers and Treatment. CURRENT PEDIATRICS REPORTS 2021. [DOI: 10.1007/s40124-021-00247-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
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26
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Weissman AS, Patel VS, Mushfiq O. Case of Gut Necrosis in Adult-Onset Immunoglobulin A Vasculitis (Henoch-Schönlein Purpura). J Investig Med High Impact Case Rep 2021; 8:2324709620925565. [PMID: 32434396 PMCID: PMC7243385 DOI: 10.1177/2324709620925565] [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] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is an immune-mediated small vessel vasculitis characterized by palpable purpura, arthralgia, abdominal pain, and renal disease. It is primarily a childhood disease and usually resolves spontaneously with supportive therapy. Treatment of IgAV in adults is controversial with no clearly established guidelines. We report a rare case of IgAV in an adult male who developed gut necrosis and perforation while receiving glucocorticoid therapy for treatment of acute glomerulonephritis. A 44-year-old male was admitted with joint pain, leg swelling, mild abdominal pain, and a diffuse rash. Laboratory values revealed acute kidney injury with significant proteinuria and hematuria. The patient was started on glucocorticoid therapy for suspected IgAV nephritis, which was confirmed by kidney biopsy. Several days later, he complained of worsening abdominal pain. Imaging demonstrated bowel ischemia and perforation requiring multiple abdominal surgeries. The patient was critically ill in the intensive care unit with worsening renal failure requiring dialysis. He was discharged a month later after gradual recovery with stable but moderately impaired kidney function. IgAV is less common in adults; however, the disease is more severe with a higher risk of long-term complications. Adult patients with renal involvement may benefit from glucocorticoid therapy in preventing progression to end-stage renal disease. However, glucocorticoids may mask the symptoms of abdominal complications like gut necrosis and perforation causing delay in diagnosis and treatment. Therefore, vigilance to detect early signs of gut ischemia is imperative when treating an adult case of IgAV nephritis with glucocorticoids.
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Affiliation(s)
| | | | - Omar Mushfiq
- Medical College of Georgia at Augusta University, Augusta, GA, USA
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Khader Y, Burmeister C, Patel D, Ambati A, Altorok N. Henoch-Schonlein Purpura Presenting as Upper Gastrointestinal Bleed in an Adult Patient. Cureus 2021; 13:e13879. [PMID: 33868843 PMCID: PMC8043251 DOI: 10.7759/cureus.13879] [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] [Indexed: 11/05/2022] Open
Abstract
Henoch-Schonlein purpura (HSP) is a multi-system autoimmune disease that is relatively common in pediatric patients. HSP usually manifests as palpable purpura, arthralgia, abdominal pain, and acute kidney injury. Here, we present a case of an adult male with hematemesis as the initial presenting symptom of HSP. A previously healthy, 18-year-old Caucasian male presented with a one-day history of hematemesis associated with abdominal pain and non-bloody diarrhea. He also reported bilateral knee and ankle arthralgias with a painless skin rash on both lower extremities. Physical exam was positive for palpable, purpuric, non-blanchable skin rash involving bilateral lower extremities. Notable labs on admission included a white cell count of 10.8 x 109/L and C-reactive protein of 4.8 mg/L. Upper endoscopy showed non-bleeding erosive gastropathy and duodenal erosions. Skin biopsy of the left leg showed immunoglobulin A (IgA) deposition within the walls of the superficial dermal vessels. The patient was started on intravenous methylprednisolone 500 mg daily followed by a steroid taper. Due to incomplete clinical response to steroids, mycophenolate mofetil 1000 mg twice daily was added and maintained for three months. His symptoms improved significantly, and he no longer complained of abdominal pain or diarrhea. Gastrointestinal manifestations are common in HSP patients. However, the diagnosis will be challenging when these symptoms precede other classical manifestations of HSP. History and physical exam are key components in accurately diagnosing HSP; nevertheless, skin biopsy remains the gold standard to confirm the diagnosis.
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Affiliation(s)
- Yasmin Khader
- Internal Medicine, University of Toledo, Toledo, USA
| | | | - Dipen Patel
- Internal Medicine, University of Toledo, Toledo, USA
| | - Amala Ambati
- Internal Medicine, University of Toledo, Toledo, USA
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29
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Atay O. Other Diseases of the Small Intestine and Colon. PEDIATRIC GASTROINTESTINAL AND LIVER DISEASE 2021:534-540.e3. [DOI: 10.1016/b978-0-323-67293-1.00049-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/06/2025]
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30
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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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31
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Intravenous γ Globulin for Intractable Abdominal Pain due to IgA Vasculitis. Case Rep Pediatr 2020; 2020:8867621. [PMID: 33123401 PMCID: PMC7586148 DOI: 10.1155/2020/8867621] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2020] [Revised: 09/26/2020] [Accepted: 09/30/2020] [Indexed: 11/17/2022] Open
Abstract
IgA vasculitis (formerly known as Henoch–Schönlein purpura or anaphylactoid purpura) is a usually benign vasculitis that affects children of school age. The disease is characterized by the tetrad of palpable purpura, arthralgia/arthritis, abdominal pain, and hematuria. Treatment of IgA vasculitis is mainly supportive, with administration of simple analgesics. Corticosteroids have been shown to reduce and/or ameliorate the occurrence of abdominal pain which may be severe. We present two children with IgA vasculitis and severe abdominal pain despite corticosteroid administration, who responded promptly to intravenous γ globulin (IVIg) with complete resolution of their symptoms and review of the relevant medical literature. Given the toxicity and/or need for long-term administration of other second-line immunosuppressive therapies in corticosteroid-resistant IgA vasculitis, such as rituximab, cyclosporine, cyclophosphamide, azathioprine, or colchicine, we propose that IVIg may be a useful and safe treatment option, although randomized controlled clinical trials are needed in order to clarify its role in the treatment of abdominal pain in IgA vasculitis.
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Mosalem O, Hernandez Garcilazo N, Saleh Y, Abu Rous F. Pulmonary embolism as the primary presentation of IgA vasculitis. BMJ Case Rep 2020; 13:13/8/e235884. [PMID: 32859624 DOI: 10.1136/bcr-2020-235884] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
A 47-year-old man presented to the emergency department with acute onset of dyspnoea and a week history of painful erythematous rash on both of his legs. CT angiogram of the chest showed saddle pulmonary embolism resulting in right ventricular strain and obstructive shock. Due to the atypical nature of his skin rash, a skin biopsy from one of these lesions was done and came consistent with the diagnosis of IgA vasculitis.
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Affiliation(s)
- Osama Mosalem
- Department of Medicine, Michigan State University, East Lansing, Michigan, USA .,Department of Medicine, Sparrow Health System, Lansing, Michigan, USA
| | - Nora Hernandez Garcilazo
- Department of Medicine, Michigan State University, East Lansing, Michigan, USA.,Department of Medicine, Sparrow Health System, Lansing, Michigan, USA
| | - Yehia Saleh
- Department of Medicine, Michigan State University, East Lansing, Michigan, USA.,Department of Cardiology, Houston Methodist DeBakey Heart and Vascular Center, Houston, Texas, USA
| | - Fawzi Abu Rous
- Department of Medicine, Michigan State University, East Lansing, Michigan, USA.,Department of Hematology and Oncology, Henry Ford Health System, Detroit, Michigan, USA
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33
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Derrow AH, Helfrich AM. Case 1: Acute Pancreatitis in a 13-year-old Girl. Pediatr Rev 2020; 41:416-418. [PMID: 32737254 DOI: 10.1542/pir.2018-0199] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
Affiliation(s)
- Avram H Derrow
- Pediatric Clinic, 52nd Medical Operations Squadron, 52nd Medical Group, Spangdahlem Air Base, Germany
| | - Alison M Helfrich
- Pediatric Clinic, 52nd Medical Operations Squadron, 52nd Medical Group, Spangdahlem Air Base, Germany
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34
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Maritati F, Canzian A, Fenaroli P, Vaglio A. Adult-onset IgA vasculitis (Henoch-Schönlein): Update on therapy. Presse Med 2020; 49:104035. [PMID: 32645417 DOI: 10.1016/j.lpm.2020.104035] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/10/2019] [Accepted: 03/05/2019] [Indexed: 12/22/2022] Open
Abstract
Immunoglobulin A vasculitis (IgAV, formerly Henoch-Schönlein purpura) is a systemic inflammatory disease affecting small vessels. While it is common and usually benign in childhood, in adults it is rarer has a more severe course. Its main manifestations are cutaneous purpura, arthralgias or arthritis, acute enteritis and glomerulonephritis. Renal involvement is associated with a poor prognosis in adults. The treatment of adult-onset IgAV is still a matter of debate: although in patients with a non-severe phenotype remission can occur spontaneously, more severe cases may need immunosuppressive therapy. There are some areas of uncertainty with respect to the efficacy of immunosuppressive regimens: almost all data come from studies performed in children or from patients with IgA nephropathy and/or IgA-crescentic glomerulonephritis. The only randomised study performed in adults with IgAV and renal involvement showed that immunosuppressive therapy with cyclophosphamide did not improve renal outcome nor did it affect patient survival. The possible efficacy of other drugs is reported only in small case series. Recent evidences show that rituximab could be an effective therapeutic option for adult-onset IgAV, but this also needs to be confirmed in controlled trials. In this review, we focus on therapeutic options for adult-onset IgAV treatment, and discuss the main results of the studies performed so far.
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Affiliation(s)
| | - Alice Canzian
- Nephrology Unit, Parma University Hospital, Parma, Italy
| | | | - Augusto Vaglio
- Department of Biochemical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, and Meyer Children's Hospital, Firenze, Italy.
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35
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Basu P, Russell-Goldman E, Nazarian RM, Das S. Alcohol-Associated Immunoglobulin A Vasculitis: A Case Report and Review of the Literature. Dermatopathology (Basel) 2020; 6:288-293. [PMID: 32596212 PMCID: PMC7315200 DOI: 10.1159/000507307] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/15/2019] [Accepted: 03/17/2020] [Indexed: 11/23/2022] Open
Abstract
Immunoglobulin A (IgA)-mediated leukocytoclastic vasculitis is a cutaneous small-vessel vasculitis characterized by skin findings of palpable purpura. It may occur secondary to infections, neoplasms, drugs, and systemic conditions, although it is most commonly idiopathic. A known, but rare, trigger for IgA vasculitis is alcohol consumption. We present a case of a man with IgA vasculitis associated with alcohol use and review the literature on alcohol-associated vasculitis. Although rarely reported, alcohol-associated IgA vasculitis is an important entity to consider for appropriate diagnosis and management of such patients.
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Affiliation(s)
- Pallavi Basu
- School of Medicine, University of California San Diego, La Jolla, California, USA
| | | | - Rosalynn M Nazarian
- Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA
| | - Shinjita Das
- Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts, USA
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36
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Zheng C, Childers J, Rabinovich E, Nazareth-Pidgeon K. Recurrent Henoch-Schönlein Purpura with bullous rash and pulmonary nodules. Pediatr Rheumatol Online J 2020; 18:40. [PMID: 32448396 PMCID: PMC7245848 DOI: 10.1186/s12969-020-00436-7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/13/2020] [Accepted: 05/06/2020] [Indexed: 11/26/2022] Open
Abstract
BACKGROUND Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. It has a characteristic rash described as palpable purpura that most frequently affects the distal lower extremities and buttocks. HSP rarely presents with bullous rash nor pulmonary nodules. CASE PRESENTATION We present a novel case of a 12-years-old female with recurrent pediatric HSP with a combination of the rare manifestations of bullous rash and pulmonary nodules. She initially presented with the bullous rash, chest pain, cough, and abdominal pain. Patient was successfully treated with intravenous pulse corticosteroids followed by a high dose oral corticosteroid taper, with resolution of the bullous rash and pulmonary nodules. CONCLUSION The rare manifestations of scarring bullous rash and pulmonary nodules can be presenting features of pediatric HSP, the combination of which has not been previously reported. The treatment of intravenous corticosteroid resolved patient's abdominal symptoms, rash and pulmonary nodules.
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Affiliation(s)
- Christopher Zheng
- grid.189509.c0000000100241216Department of Pediatrics, Duke University Hospital, 2301 Erwin Rd, Durham, NC 27705 USA
| | - Julie Childers
- grid.189509.c0000000100241216Department of Pediatrics, Duke University Hospital, 2301 Erwin Rd, Durham, NC 27705 USA
| | - Egla Rabinovich
- grid.189509.c0000000100241216Department of Pediatrics, Duke University Hospital, 2301 Erwin Rd, Durham, NC 27705 USA
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Yi F, Bai Z, Li Y, Xu X, Guo X, Qi X. A good response to glucocorticoid for Henoch-Schönlein purpura with abdominal pain and gastrointestinal bleeding in an adult: A CARE case report. Medicine (Baltimore) 2020; 99:e18602. [PMID: 31895810 PMCID: PMC6946351 DOI: 10.1097/md.0000000000018602] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/09/2019] [Revised: 11/07/2019] [Accepted: 12/04/2019] [Indexed: 02/07/2023] Open
Abstract
RATIONALE Henoch-Schönlein purpura (HSP) is a small-vessel vasculitis that has been extensively studied in children, but little is known about its natural history in adults. There is no consensus regarding the treatment of glucocorticosteroids use for HSP. The efficacy of glucocorticoid for preventing from severe complications or relapse is also controversial in HSP. PATIENT CONCERNS A 21-year-old male was admitted to the hospital due to abdominal pain for more than 20 days, hematochezia for more than 10 days, and rash for 2 days. DIAGNOSES The diagnosis of HSP is based on the European League against Rheumatism and the Paediatric Rheumatology European Society in 2006. INTERVENTIONS The patient received glucocorticosteroids treatment for 17 days at the time of first hospitalization. OUTCOMES The abdominal pain and hematochezia completely disappeared on the 6th day after the use of glucocorticosteroids, and purpura completely disappeared on the 8th day. LESSONS Our patient has a good response to glucocorticoid. Glucocorticosteroids may be effective for the treatment of HSP.
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Affiliation(s)
- Fangfang Yi
- Department of Gastroenterology, General Hospital of Northern Theater Command, Shenyang
- Postgraduate College, Dalian Medical University, Dalian
| | - Zhaohui Bai
- Department of Gastroenterology, General Hospital of Northern Theater Command, Shenyang
- Postgraduate College, Shenyang Pharmaceutical University, Shenyang
| | - Yingying Li
- Department of Gastroenterology, General Hospital of Northern Theater Command, Shenyang
- Postgraduate College, Jinzhou Medical University, Jinzhou 121001, P.R. China
| | - Xiangbo Xu
- Department of Gastroenterology, General Hospital of Northern Theater Command, Shenyang
- Postgraduate College, Shenyang Pharmaceutical University, Shenyang
| | - Xiaozhong Guo
- Department of Gastroenterology, General Hospital of Northern Theater Command, Shenyang
| | - Xingshun Qi
- Department of Gastroenterology, General Hospital of Northern Theater Command, Shenyang
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Roman C, Dima B, Muyshont L, Schurmans T, Gilliaux O. Indications and efficiency of dapsone in IgA vasculitis (Henoch-Schonlein purpura): case series and a review of the literature. Eur J Pediatr 2019; 178:1275-1281. [PMID: 31230197 DOI: 10.1007/s00431-019-03409-5] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/11/2019] [Revised: 06/06/2019] [Accepted: 06/09/2019] [Indexed: 01/09/2023]
Abstract
Immunoglobulin A (IgA) vasculitis (Henoch-Schonlein purpura (HSP)) is the most common vasculitis in children. It is characterized by purpuric rash, arthritis, gastrointestinal, and/or renal involvement. Spontaneous resolution is the typical outcome. In chronic cutaneous manifestations of IgA vasculitis, dapsone seems to show a good effectiveness. Multiple case reports and case series about dapsone in chronic IgA vasculitis are available. However, no clear evaluation of its indications, its effectiveness, or its usage guidelines (optimal dosage or duration of treatment) is available. We reviewed the published cases of IgA vasculitis treated by dapsone and compared them with 2 similar cases that we encountered. Seventeen patients (ranging from 22 months old to 16 years old) with severe or persistent clinical signs of IgA vasculitis were included. Dapsone showed good results on the resolution of cutaneous lesions but not on renal manifestations. Complications (methemoglobinemia) were observed on 1 patient. Half of the patients relapsed after treatment discontinuation. The difference between the time lapse before initiation and the duration of the treatment was not significant.Conclusion: We suggest that dapsone can have a positive effect in chronic IgA vasculitis when cutaneous manifestations last more than 6 weeks at the dosage of 1-2 mg/kg once per day during 1 week. What is Known: • IgA vasculitis or Henoch-Schonlein purpura is the most common vasculitis in children and affects mostly small vessels of the skin, kidney, and gastrointestinal tract. It resolves spontaneously in most of the cases. Exceptionally, cutaneous lesions can last several weeks. • Dapsone is a bacteriostatic antibacterial sulfonamide drug found to be effective in the treatment of some inflammatory dermatological diseases like IgA vasculitis. What is New: • Dapsone is effective against chronic purpuric lesion (> 6 weeks) at the minimal dose of 1 mg/kg/day. • Relapse occurs frequently after discontinuation but responds after a second course of treatment. A longer duration of treatment or a delay in treatment by dapsone does not seem to influence the relapse rate.
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Affiliation(s)
- Céline Roman
- Department of Pediatrics, Hôpital Civil Marie Curie, CHU of Charleroi, 140 Chaussée de Bruxelles, 6042, Charleroi (Lodelinsart), Belgium
| | - Bogdan Dima
- Department of Pediatrics, Hôpital Civil Marie Curie, CHU of Charleroi, 140 Chaussée de Bruxelles, 6042, Charleroi (Lodelinsart), Belgium.,Pediatric Department, Cliniques de l'Europe Sainte-Elisabeth, 206 Avenue de Frélaan, 1180, Brussels, Belgium
| | - Laurence Muyshont
- Department of Pediatrics, Hôpital Civil Marie Curie, CHU of Charleroi, 140 Chaussée de Bruxelles, 6042, Charleroi (Lodelinsart), Belgium
| | - Thierry Schurmans
- Department of Pediatrics, Hôpital Civil Marie Curie, CHU of Charleroi, 140 Chaussée de Bruxelles, 6042, Charleroi (Lodelinsart), Belgium
| | - Olivier Gilliaux
- Department of Pediatrics, Hôpital Civil Marie Curie, CHU of Charleroi, 140 Chaussée de Bruxelles, 6042, Charleroi (Lodelinsart), Belgium.
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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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Kurnia B. Henoch-Schonlein Purpura in Children: The Role of Corticosteroids. Open Access Maced J Med Sci 2019; 7:1812-1814. [PMID: 31316664 PMCID: PMC6614272 DOI: 10.3889/oamjms.2019.538] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/19/2019] [Revised: 05/01/2019] [Accepted: 06/02/2019] [Indexed: 01/08/2023] Open
Abstract
BACKGROUND Henoch-schonlein purpura (HSP) is an IgA-mediated systemic small vessel vasculitis. It is the most common form of systemic vasculitis in children. CASE REPORT A 9 years old girl admitted to the hospital with chief complain of purplish red rash on both legs since approximately 1 week with painful knees and ankles that make the patient unable to walk. The patient was diagnosed with HSP and was treated with corticosteroid and analgesics. The patients only stayed for 2 nights at the hospital and discharged from the hospital with the ability to walk and experience no pain. CONCLUSION The role of corticosteroids in the treatment of HSP is still controversial. But from various research, we can conclude that the role of corticosteroid in HSP is as a symptom reliever (reduce abdominal pain and arthritis), but does not slow the progression of renal disease.
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Allen KL, Dein EJ, Ali OME, Gelber AC. Challenging to Treat: Fluctuating Abdominal and Joint Pain and Rash. Am J Med 2019; 132:579-582. [PMID: 30576632 DOI: 10.1016/j.amjmed.2018.11.044] [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: 11/28/2018] [Accepted: 11/29/2018] [Indexed: 10/27/2022]
Affiliation(s)
- Katherine L Allen
- Johns Hopkins Hospital and Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Md..
| | - Eric J Dein
- Johns Hopkins Hospital and Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Md
| | - Osama M E Ali
- Johns Hopkins Hospital and Department of Radiology, Johns Hopkins University School of Medicine, Baltimore, Md
| | - Allan C Gelber
- Johns Hopkins Hospital and Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Md
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Abstract
The causes of macroscopic and microscopic hematuria overlap; both are often caused by urinary tract infections or urethral/bladder irritation. Coexistent hypertension and proteinuria should prompt investigation for glomerular disease. The most common glomerulonephritis in children is postinfectious glomerulonephritis. In most patients, and especially with isolated microscopic hematuria, the diagnostic workup reveals no clear underlying cause. In those cases whereby a diagnosis is made, the most common causes of persistent microscopic hematuria are thin basement membrane nephropathy, immunoglobulin A nephropathy, or idiopathic hypercalciuria. Treatment and long-term prognosis varies with the underlying disease.
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Affiliation(s)
- Denver D Brown
- Pediatric Nephrology, Children's Hospital at Montefiore, Albert Einstein College of Medicine, 3415 Bainbridge Avenue, Bronx, NY 10467, USA
| | - Kimberly J Reidy
- Pediatric Nephrology, Children's Hospital at Montefiore, Albert Einstein College of Medicine, 3415 Bainbridge Avenue, Bronx, NY 10467, USA.
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Uehara E, Nagata C, Masuda H, Fujimori K, Kobayashi S, Kubota M, Ishiguro A. Risk factors of long hospital stay for immunoglobulin A vasculitis: Single-center study. Pediatr Int 2018; 60:918-922. [PMID: 30129988 DOI: 10.1111/ped.13685] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/22/2018] [Revised: 07/27/2018] [Accepted: 08/17/2018] [Indexed: 12/18/2022]
Abstract
BACKGROUND Immunoglobulin A (IgA) vasculitis is a common, systemic childhood disease that occasionally interferes with oral intake of food and necessitates hospitalization. In Japan, there are no reports on the length of hospitalization or factors related to long-term hospitalization in children with IgA vasculitis. In this study, we investigated the factors related to long-term hospitalization. METHODS We reviewed the medical records of children aged ≤15 years with IgA vasculitis who were admitted to the National Center for Child Health and Development (Tokyo, Japan) between March 2008 and April 2017. We reviewed their gender, age, previous episodes, digestive symptoms, fever, laboratory data, urine analysis, ultrasound, and use of glucocorticoid on admission day. We compared the long-stay (≥10 days) group (L) and the short-stay (≤9 days) group (S) on logistic regression analysis. RESULTS Of the 68 children included in the analysis, 34 were male, and the average age was 71.9 ± 26.4 months. The median period of hospitalization was 10.5 days (range, 0.5-75 days), and 36 children were allocated to group L. In the logistic regression model including age, gender, gastrointestinal (GI) bleeding, and use of glucocorticoid, male sex (OR: 4.2; 95%CI: 1.3-13.5) and GI bleeding (OR: 7.6; 95%CI: 1.4-41.5) were significantly associated with hospitalization ≥10 days. CONCLUSIONS In children with IgA vasculitis, male patients and those with GI bleeding were more likely to have a hospital stay ≥10 days.
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Affiliation(s)
- Erika Uehara
- Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan.,Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan
| | - Chie Nagata
- Department of Education for Clinical Research, National Center for Child Health and Development, Tokyo, Japan
| | - Hiroshi Masuda
- Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan
| | - Kentaro Fujimori
- Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan.,Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan
| | - Shinobu Kobayashi
- Department of Social Medicine, National Center for Child Health and Development, Tokyo, Japan
| | - Mitsuru Kubota
- Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan
| | - Akira Ishiguro
- Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan.,Department of Education for Clinical Research, National Center for Child Health and Development, Tokyo, Japan
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Henoch-Schönlein Purpura Presenting With Subcutaneous Edema: A Case Report and a Proposal to Include Subcutaneous Edema as a Diagnostic Criterion. J Clin Rheumatol 2018; 26:e65-e66. [PMID: 30142114 DOI: 10.1097/rhu.0000000000000811] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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45
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Shim JO, Han K, Park S, Kim GH, Ko JS, Chung JY. Ten-year Nationwide Population-based Survey on the Characteristics of Children with Henoch-Schӧnlein Purpura in Korea. J Korean Med Sci 2018; 33:e174. [PMID: 29915525 PMCID: PMC6000599 DOI: 10.3346/jkms.2018.33.e174] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/24/2018] [Accepted: 04/17/2018] [Indexed: 12/15/2022] Open
Abstract
BACKGROUND Henoch-Schӧnlein purpura (HSP) is a common vasculitis of childhood. Though HSP is usually self-limiting, severe complications can occur. The management for this condition has not been established yet. Thus, this nationwide study aimed at investigating epidemiological characteristics of children with HSP in Korea. The patterns of clinical practice with regard to the complications of the condition were also investigated. METHODS This is a national population-based study that used the National Health Insurance Database. Children below 18 years who were diagnosed with HSP in Korea between 2006 and 2015 were enrolled. Data, such as age, sex, yearly and monthly distribution of HSP, hospitalization, re-hospitalization, comorbidities, and interventions were obtained. The use of steroids was also analyzed. RESULTS A total of 56,841 children were enrolled. The annual incidence of HSP was 55.9 per 100,000 children. The peak age was 5 years. Spring was the most prevalent season. Sex (male) and young age (< 9 years) were risk factors of hospitalization. Younger children were more likely to be re-hospitalized and suspected with intussusception, arthritis, and nephritis. Only 4 children received laparotomy. In total, 57% were managed with steroids, and mean durations of medication were 4-5 days. Children who were hospitalized and those with comorbidities used steroids more frequently (P < 0.001). CONCLUSION The annual incidence of HSP is 55.9 per 100,000 children which is higher in Korea than that in other countries. Younger children can have a more severe clinical course. This nationwide survey provides valuable information to understand HSP in children and to inspire further research on HSP.
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Affiliation(s)
- Jung Ok Shim
- Department of Pediatrics, Korea University College of Medicine, Seoul, Korea
| | - Kyoungdo Han
- Department of Medical Statistics, The Catholic University of Korea, Seoul, Korea
| | - Sanghyun Park
- Department of Medical Statistics, The Catholic University of Korea, Seoul, Korea
| | - Gun-Ha Kim
- Department of Pediatrics, Korea University College of Medicine, Seoul, Korea
| | - Jae Sung Ko
- Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
| | - Ju-Young Chung
- Department of Pediatrics, Inje University College of Medicine, Seoul, Korea
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Brodie A, G N, Nitiahpapand R, Chowoo L. Unusual presentation of Henöch-Schonlein purpura. BMJ Case Rep 2018; 2018:bcr-2017-220129. [PMID: 29848517 DOI: 10.1136/bcr-2017-220129] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023] Open
Abstract
We present a rare case of a 4-year-old boy with newly diagnosed Henöch-Schonlein purpura (HSP) affecting the scrotum and penis. The patient presented to the emergency department with palpable purpura symmetrically distributed over the lower limbs. This was associated with arthritis of the right knee, abdominal pain and scrotal swelling. These symptoms were preceded by an upper respiratory tract infection (URTI). The patient was initially treated with empirical oral antibiotics for epididymitis and was discharged. He subsequently re-presented 12 days later with penile swelling, erythema and tenderness. An ultrasound scan of the penis revealed grossly oedematous subcutaneous tissue with normal penile architecture. His symptoms resolved spontaneously and the patient remains under close follow-up by the paediatric team for further sequelae of HSP.
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Affiliation(s)
- Andrew Brodie
- Department of Urology, Lister Hospital, Stevenage, UK
| | - Natasha G
- Department of Urology, Lister Hospital, Stevenage, UK
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Su HW, Chen CY, Chiou YH. Hemorrhagic bullous lesions in Henoch-Schönlein purpura: a case report and review of the literature. BMC Pediatr 2018; 18:157. [PMID: 29747613 PMCID: PMC5944150 DOI: 10.1186/s12887-018-1117-8] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/25/2016] [Accepted: 04/18/2018] [Indexed: 11/10/2022] Open
Abstract
Background Henoch-Schönlein purpura (HSP) is a common vasculitis in childhood characterized by purpura, arthritis, abdominal pain and renal involvement. However, bullous HSP is a rare cutaneous manifestation, and a few cases have been reported. Case presentation Herein, we report a 15-year-old male with bullous HSP who presented with severe abdominal pain and hemorrhagic bullous lesions over his lower extremities. He was treated with corticosteroid, after which the symptoms improved dramatically. No recurrence was noted after follow-up, though scarring was present. We also reviewed the literature related to bullous HSP and identified 39 cases, most of whom were treated with corticosteroids. Conclusion Clinicians should be aware of the atypical types of HSP, including bullous HSP. Most patients with bullous HSP have a good prognosis.
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Affiliation(s)
- Hung-Wen Su
- Division of Pediatric Allergy, Immunology and Rheumatology, Department of Pediatrics, Kaohsiung Veterans General Hospital, No. 386, Dazhong 1st Road, Zuoying District, Kaohsiung City, 813, Taiwan
| | - Chiu-Yu Chen
- Division of Pediatric Allergy, Immunology and Rheumatology, Department of Pediatrics, Kaohsiung Veterans General Hospital, No. 386, Dazhong 1st Road, Zuoying District, Kaohsiung City, 813, Taiwan
| | - Yee-Hsuan Chiou
- Division of Pediatric Allergy, Immunology and Rheumatology, Department of Pediatrics, Kaohsiung Veterans General Hospital, No. 386, Dazhong 1st Road, Zuoying District, Kaohsiung City, 813, Taiwan. .,School of Medicine, National Yang-Ming University, Taipei, Taiwan.
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48
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Lei WT, Tsai PL, Chu SH, Kao YH, Lin CY, Fang LC, Shyur SD, Lin YW, Wu SI. Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database. Pediatr Rheumatol Online J 2018; 16:25. [PMID: 29661187 PMCID: PMC5902957 DOI: 10.1186/s12969-018-0247-8] [Citation(s) in RCA: 48] [Impact Index Per Article: 6.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/29/2018] [Accepted: 04/11/2018] [Indexed: 01/10/2023] Open
Abstract
BACKGROUND The recurrence rate of Henoch-Schönlein purpura (HSP) is 2.7%-30%, with varied average intervals between the first and second episodes. Few studies have explored the incidence and risk factors for recurrent HSP. METHODS We used a 16-year nationwide database to analyze the incidence of recurrent HSP. Patients with HSP were identified, and risk factors for recurrent HSP were explored. Kaplan-Meier and Cox regression model analyses were performed, and covariates were adjusted in the multivariate model. RESULTS From January 1, 1997 to December 31, 2012, among 2,886,836 individuals in the National Health Insurance Research Database, 1002 HSP patients aged < 18 years were identified. Among them, 164 had ≥2 HSP episodes (recurrence rate, 16.4%; incidence of recurrent HSP, 7.05 per 100 person-years); 83.6% patients with one HSP episode remained free of secondary HSP. The average time intervals between the first and second and second and third HSP episodes were 9.2 and 6.4 months, respectively. After adjusting for demographic parameters, comorbidities, and socioeconomic status, recurrent HSP was found to occur more frequently in patients who had renal involvement (adjusted hazard ratio, 2.41; 95% confidence interval [CI], 1.64-3.54; p < 0.001), were receiving steroid therapy for > 10 days (adjusted hazard ratio, 8.13; 95%CI, 2.51-26.36; p < 0.001), and had allergic rhinitis (adjusted hazard ratio, 1.63; 95%CI, 1.06-2.50; p = 0.026). CONCLUSIONS The annual incidence of recurrent HSP was low. However, children who had underlying allergic rhinitis, presented with renal involvement, and received steroid treatment for > 10 days should be notified regarding the possibility of recurrence.
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Affiliation(s)
- Wei-Te Lei
- 0000 0004 0573 007Xgrid.413593.9Division of Allergy, Immunology, Rheumatology Disease, Department of Pediatrics, Mackay Memorial Hospital, Hsinchu, Taiwan
| | - Po-Li Tsai
- Division of Colorectal Surgery, Department of Surgery, Mackey Memorial Hospital, Taipei, Taiwan
| | - Szu-Hung Chu
- 0000 0004 0573 007Xgrid.413593.9Division of Allergy, Immunology, Rheumatology Disease, Department of Pediatrics, Mackay Memorial Hospital, Hsinchu, Taiwan
| | - Yu-Hsuan Kao
- 0000 0004 0573 007Xgrid.413593.9Division of Allergy, Immunology, Rheumatology Disease, Department of Pediatrics, Mackay Memorial Hospital, Hsinchu, Taiwan
| | - Chien-Yu Lin
- 0000 0004 0573 007Xgrid.413593.9Department of Pediatrics, Mackay Memorial Hospital, Hsinchu, Taiwan
| | - Li-Ching Fang
- 0000 0004 0573 007Xgrid.413593.9Division of Allergy, Immunology, Rheumatology Disease, Department of Pediatrics, Mackay Memorial Hospital, Hsinchu, Taiwan
| | - Shyh-Dar Shyur
- 0000 0004 0573 007Xgrid.413593.9Division of Allergy, Immunology, Rheumatology Disease, Department of Pediatrics, Mackay Memorial Hospital, Hsinchu, Taiwan
| | - Yu-Wen Lin
- 0000 0004 0573 007Xgrid.413593.9Department of Medical Research, Mackay Memorial Hospital, Taipei, Taiwan
| | - Shu-I Wu
- Department of Medicine, Mackay Medical College, No.45, Minsheng Rd., Tamsui Dist., New Taipei City, 25160, Taiwan. .,Audiology and Speech Language Pathology, Mackay Medical College, No.45, Minsheng Rd., Tamsui Dist., New Taipei City, 25160, Taiwan. .,Department of Psychiatry, Mackay Memorial Hospital, No.45, Minsheng Rd., Tamsui Dist., New Taipei City, 25160, Taiwan.
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Hackl A, Becker JU, Körner LM, Ehren R, Habbig S, Nüsken E, Nüsken KD, Ebner K, Liebau MC, Müller C, Pohl M, Weber LT. Mycophenolate mofetil following glucocorticoid treatment in Henoch-Schönlein purpura nephritis: the role of early initiation and therapeutic drug monitoring. Pediatr Nephrol 2018; 33:619-629. [PMID: 29177628 DOI: 10.1007/s00467-017-3846-6] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/29/2017] [Revised: 11/02/2017] [Accepted: 11/03/2017] [Indexed: 12/24/2022]
Abstract
BACKGROUND Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood and traditionally considered as a self-limiting disease. However, renal involvement can unfavorably determine long-term prognosis. The reported regimens to treat HSP nephritis (HSPN) are diverse, indicating that the most effective treatment remains controversial. METHODS This retrospective, single-center study involved 18 patients presenting with HSPN and nephrotic-range proteinuria. We aimed to investigate the efficacy and safety of mycophenolate mofetil (MMF) and identify a cut-off level for estimated mycophenolic acid area under the curve (eMPA-AUC0-12h) values, which can predict complete remission with high sensitivity. RESULTS Despite prior insufficient therapeutic response to corticosteroids, 89% of patients showed a significant decrease in proteinuria after 1 month of MMF treatment. None of them relapsed during treatment; however, two children relapsed after discontinuation. Based on results of a receiver operating characteristic (ROC) analysis, an eMPA-AUC0-12h >56.4 mg*h/l was a predictor for complete remission within 3 months (80% sensitivity, 83.3% specificity, p = 0.035). During MMF administration, we encountered no adverse event requiring discontinuation of treatment. CONCLUSION Our study demonstrates that MMF is a safe and potentially effective secondary treatment option for children with HSPN to achieve and maintain long-term remission without serious side effects. To achieve complete remission within 3 months, resolve severe inflammatory glomerular lesions, and avoid progression to chronic kidney disease, we propose timely diagnosis and early initiation of MMF with an eMPA-AUC0-12h value of 56.4 mg*h/l.
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Affiliation(s)
- Agnes Hackl
- Pediatric Nephrology, Children's and Adolescents' Hospital, University Hospital of Cologne, Kerpener Street 62, 50937, Cologne, Germany.
| | - Jan U Becker
- Institute of Pathology, University Hospital of Cologne, Cologne, Germany
| | - Lisa M Körner
- Pediatric Nephrology, Children's and Adolescents' Hospital, University Hospital of Cologne, Kerpener Street 62, 50937, Cologne, Germany
| | - Rasmus Ehren
- Pediatric Nephrology, Children's and Adolescents' Hospital, University Hospital of Cologne, Kerpener Street 62, 50937, Cologne, Germany
| | - Sandra Habbig
- Pediatric Nephrology, Children's and Adolescents' Hospital, University Hospital of Cologne, Kerpener Street 62, 50937, Cologne, Germany
| | - Eva Nüsken
- Pediatric Nephrology, Children's and Adolescents' Hospital, University Hospital of Cologne, Kerpener Street 62, 50937, Cologne, Germany
| | - Kai-Dietrich Nüsken
- Pediatric Nephrology, Children's and Adolescents' Hospital, University Hospital of Cologne, Kerpener Street 62, 50937, Cologne, Germany
| | - Kathrin Ebner
- Pediatric Nephrology, Children's and Adolescents' Hospital, University Hospital of Cologne, Kerpener Street 62, 50937, Cologne, Germany
| | - Max C Liebau
- Pediatric Nephrology, Children's and Adolescents' Hospital, University Hospital of Cologne, Kerpener Street 62, 50937, Cologne, Germany.,Nephrology Research Laboratory, Department II of Internal Medicine, University Hospital of Cologne, Cologne, Germany.,Center for Molecular Medicine, University Hospital of Cologne, Cologne, Germany
| | - Carsten Müller
- Department of Therapeutic Drug Monitoring, Centre of Pharmacology, University Hospital of Cologne, Cologne, Germany
| | - Martin Pohl
- Department of General Pediatrics, Adolescent Medicine and Neonatology, Medical Center - Faculty of Medicine, University of Freiburg Germany, Freiburg, Germany
| | - Lutz T Weber
- Pediatric Nephrology, Children's and Adolescents' Hospital, University Hospital of Cologne, Kerpener Street 62, 50937, Cologne, Germany
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Nothhaft M, Klepper J, Kneitz H, Meyer T, Hamm H, Morbach H. Hemorrhagic Bullous Henoch-Schönlein Purpura: Case Report and Review of the Literature. Front Pediatr 2018; 6:413. [PMID: 30723709 PMCID: PMC6349767 DOI: 10.3389/fped.2018.00413] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/30/2018] [Accepted: 12/11/2018] [Indexed: 12/27/2022] Open
Abstract
Henoch-Schönlein Purpura (HSP) or IgA vasculitis is the most common systemic vasculitis of childhood and may affect skin, joints, gastrointestinal tract, and kidneys. Skin manifestations of HSP are characteristic and include a non-thrombocytopenic palpable purpura of the lower extremities and buttocks. Rarely, HSP may initially present as or evolve into hemorrhagic vesicles and bullae. We present an otherwise healthy 5-year-old boy with an acute papulovesicular rash of both legs and intermittent abdominal pain. After a few days the skin lesions rapidly evolved into palpable purpura and hemorrhagic bullous lesions of variable size and severe hemorrhagic HSP was suspected. A histological examination of a skin biopsy showed signs of a small vessel leukocytoclastic vasculitis limited to the upper dermis and direct immunofluorescence analysis revealed IgA deposits in vessel walls, compatible with HSP. To further characterize the clinical picture and treatment options of bullous HSP we performed an extensive literature research and identified 41 additional pediatric patients with bullous HSP. Two thirds of the reported patients were treated with systemic corticosteroids, however, up to 25% of the reported patients developed skin sequelae such as hyperpigmentation and/or scarring. The early use of systemic corticosteroids has been discussed controversially and suggested in some case series to be beneficial by reducing the extent of lesions and minimizing sequelae of disease. Our patient was treated with systemic corticosteroids tapered over 5 weeks. Fading of inflammation resulted in healing of most erosions, however, a deep necrosis developing from a large blister at the dorsum of the right foot persisted so that autologous skin transplantation was performed. Re-examination 11 months after disease onset showed complete clinical remission with re-epithelialization but also scarring of some affected areas.
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Affiliation(s)
- Matthias Nothhaft
- Department of Pediatrics, University Hospital Würzburg, Würzburg, Germany
| | - Joerg Klepper
- Department of Pediatrics, Klinikum Aschaffenburg-Alzenau, Aschaffenburg, Germany
| | - Hermann Kneitz
- Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Würzburg, Germany
| | - Thomas Meyer
- Department of Pediatric Surgery, Pediatric Traumatology and Pediatric Urology, University Hospital Würzburg, Würzburg, Germany
| | - Henning Hamm
- Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Würzburg, Germany
| | - Henner Morbach
- Department of Pediatrics, University Hospital Würzburg, Würzburg, Germany
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