Leflunomide therapy for IgA vasculitis with nephritis in children

Abstract Background Henoch-Schönlein purpura (HSP), also called IgA vasculitis, is a systemic vasculitis characterized by deposits of immunoglobulin A in blood vessels. Renal impairment of these patients is the main determinant of prognosis. The optimal treatment of HSP nephritis (HSPN) in children remains controversial, but many clinicians administer an immunosuppressive agent with a corticosteroid. A previous study reported that leflunomide (LEF) with a corticosteroid was effective for adult patients with HSPN and nephrotic proteinuria. However, data on this treatment in pediatric patients is limited. Methods We described our experience at a single center on the use of LEF in 5 pediatric patients who had IgA vasculitis with proteinuria that was nearly 50 mg/kg (nephrotic range) and remained high despite administration of intravenous steroid, and biopsy-proven nephritis. All patients had class II to IIIb lesions based on the International Study of Kidney Disease in Children (ISKDC). Results We successfully treated all 5 children who had IgA vasculitis with nephritis using LEF with a corticosteroid. Four patients achieved a complete remission of proteinuria, and 1 patient had significantly reduced proteinuria. The children received LEF for 6 months to 12 months, and none of them had severe adverse events. Conclusions To our knowledge, this is the first case series to report successful treatment of pediatric HSPN with LEF in combination with a corticosteroid.

Download Full-text

A case of vasculitis triggered by infective endocarditis in a patient undergoing maintenance hemodialysis: a case report

BMC Nephrology ◽

10.1186/s12882-021-02647-w ◽

2022 ◽

Vol 23 (1) ◽

Author(s):

Hanui Park ◽

Miji Lee ◽

Jin Seon Jeong

Keyword(s):

Infective Endocarditis ◽

Clinical Features ◽

Systemic Vasculitis ◽

Renal Involvement ◽

Immunoglobulin A ◽

Maintenance Hemodialysis ◽

Iga Vasculitis ◽

Pharmacological Agents ◽

Case Presentation ◽

First Case

Abstract Background Immunoglobulin A vasculitis (IgA vasculitis) is one of the most common forms of vasculitis in children. It rarely occurs in adults. It is a systemic vasculitis with IgA deposition and is characterized by the classical tetrad of purpura, arthritis/arthralgia, gastrointestinal and renal involvement. Certain types of infections, and pharmacological agents have been reported to be associated with IgA vasculitis. Here, we describe a case of IgA vasculitis triggered by infective endocarditis in a patient undergoing maintenance hemodialysis. Case presentation A 70-year-old man undergoing hemodialysis was admitted because of skin purpura, abdominal pain, diarrhea, and lower back pain. We suspected him as IgA vasculitis based on the clinical features and skin biopsy findings. Transesophageal echocardiography revealed infective endocarditis, which predisposed him to IgA vasculitis. He was treated with antibiotics and low-dose corticosteroids, which led to resolution of vasculitis. Conclusions This is the first case of IgA vasculitis triggered by infective endocarditis in a patient undergoing hemodialysis. Patients undergoing hemodialysis are at a high risk of infection because of immune dysfunction and frequent venipuncture. The incidence of infective endocarditis associated with IgA vasculitis is very low, but it has been repeatedly reported. Therefore, it is necessary to consider infective endocarditis in patients with clinical features that indicate IgA vasculitis.

Download Full-text

Henoch Schonlein purpura in pediatric patients with inflammatory bowel disease: A case series

Pediatric Dermatology ◽

10.1111/pde.14818 ◽

2021 ◽

Author(s):

Anjani P. Sheth ◽

Karina L. Vivar ◽

Annette M. Wagner ◽

Lacey L. Kruse

Keyword(s):

Inflammatory Bowel Disease ◽

Bowel Disease ◽

Pediatric Patients ◽

Case Series ◽

Henoch Schonlein Purpura ◽

Inflammatory Bowel ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Download Full-text

Low Back Pain and Swelling as an Atypical Presentation of IgA Vasculitis

Clinical Practice and Cases in Emergency Medicine ◽

10.5811/cpcem.2019.11.44574 ◽

2020 ◽

Vol 4 (2) ◽

pp. 241-243

Author(s):

Clay Winkler ◽

Raymond Dobson ◽

Michael Tranovich

Keyword(s):

Low Back Pain ◽

Back Pain ◽

Immunoglobulin A ◽

Lumbar Pain ◽

Low Back ◽

Iga Vasculitis ◽

Gastrointestinal Involvement ◽

Cutaneous Manifestations ◽

Schonlein Purpura ◽

Immunoglobulin A Vasculitis

Introduction: Immunoglobulin A vasculitis (IgA vasculitis), formerly Henoch-Schonlein purpura, is the most common vasculitis in children. Case Report: A 6-year-old female presented with low back pain and swelling, difficulty ambulating, and rash two weeks after a respiratory infection. She was approached with a broad differential and ultimately diagnosed with IgA vasculitis. Discussion: Cutaneous manifestations, arthralgias, renal and gastrointestinal involvement are the most common presenting signs of IgA vasculitis. Only two cases of IgA vasculitis associated with lumbar pain and swelling were identified in the literature. Conclusion: While rash and joint pain are common presenting signs of IgA vasculitis, practitioners should be aware it can present atypically.

Download Full-text

First case series of the transradial approach for neurointerventional procedures in pediatric patients

Journal of Neurosurgery Pediatrics ◽

10.3171/2019.12.peds19448 ◽

2020 ◽

Vol 25 (5) ◽

pp. 492-496 ◽

Cited By ~ 1

Author(s):

Neil Majmundar ◽

Pratit Patel ◽

Vincent Dodson ◽

Ivo Bach ◽

James K. Liu ◽

...

Keyword(s):

Ultrasound Guidance ◽

Pediatric Patients ◽

Pediatric Population ◽

Case Series ◽

Transfemoral Approach ◽

Safe Alternative ◽

Transradial Approach ◽

Clinical Events ◽

First Case ◽

Neurointerventional Procedures

OBJECTIVEThe transradial approach (TRA) has been widely adopted by interventional cardiologists but is only now being accepted by neurointerventionalists. The benefits of the TRA over the traditional transfemoral approach (TFA) include reduced risk of adverse clinical events and faster recovery. The authors assessed the safety and feasibility of the TRA for neurointerventional cases in the pediatric population.METHODSPediatric patients undergoing cerebrovascular interventions since implementation of the TRA at the authors’ institution were retrospectively reviewed. Pertinent patient information, procedure indications, vessels catheterized, fluoroscopy time, and complications were reviewed.RESULTSThere were 4 patients in this case series, and their ages ranged from 13 to 15 years. Each patient tolerated the procedure performed using the TRA without any postprocedural issues, and only 1 patient experienced radial artery spasm, which resolved with the administration of intraarterial verapamil. None of the patients required conversion to the TFA.CONCLUSIONSThe TRA can be considered a safe alternative to the TFA for neurointerventional procedures in the pediatric population and provides potential advantages. However, as pediatric patients require special consideration due to their smaller-caliber arteries, routine use of ultrasound guidance is advised when attempting the TRA.

Download Full-text

Rivaroxaban-related acute kidney injury in a patient with IgA vasculitis

BMJ Case Reports ◽

10.1136/bcr-2018-227756 ◽

2019 ◽

Vol 12 (1) ◽

pp. e227756 ◽

Cited By ~ 9

Author(s):

Yoshihiko Fujino ◽

Chisato Takahashi ◽

Kensuke Mitsumoto ◽

Takashi Uzu

Keyword(s):

Atrial Fibrillation ◽

Acute Kidney Injury ◽

Renal Function ◽

Systemic Vasculitis ◽

Kidney Injury ◽

Close Monitoring ◽

Iga Vasculitis ◽

Direct Oral Anticoagulant ◽

Macroscopic Haematuria ◽

First Case

Anticoagulants have recently been recognised as a cause of acute kidney injury (AKI). We describe the case of a 75-year-old man with IgA vasculitis and atrial fibrillation treated with rivaroxaban, who presented with macroscopic haematuria and an acute decline in renal function. Two months before referral, he noted palpable purpuric lesions and was diagnosed with IgA vasculitis based on skin biopsy findings; the skin lesion disappeared following treatment with a steroid external preparation. Renal biopsy revealed glomerular haemorrhage and red blood cell casts. Although rivaroxaban was withdrawn, his kidney function worsened and he was started on haemodialysis. His renal function did not recover. To the best of our knowledge, this is the first case of direct oral anticoagulant (DOAC)-related AKI in systemic vasculitis. During DOAC therapy, close monitoring of a patient’s urinalysis results and their renal function may be required for patients with systemic vasculitis to avoid AKI.

Download Full-text

Tubulointerstitial Infiltration of M2 Macrophages in Henoch-Schönlein Purpura Nephritis Indicates the Presence of Glomerular Crescents and Bad Clinical Parameters

BioMed Research International ◽

10.1155/2019/8579619 ◽

2019 ◽

Vol 2019 ◽

pp. 1-10 ◽

Cited By ~ 2

Author(s):

Jisup Kim ◽

Sung-Eun Choi ◽

Keum Hwa Lee ◽

Hyeon Joo Jeong ◽

Jae Il Shin ◽

...

Keyword(s):

Systemic Vasculitis ◽

Renal Involvement ◽

International Study ◽

Clinical Parameters ◽

M2 Macrophages ◽

Henoch Schonlein Purpura ◽

Severe Manifestation ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura ◽

Purpura Nephritis

Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in children, and renal involvement (HSP nephritis, HSPN) is a severe manifestation. HSPN is histologically classified by the International Study of Kidney Disease in Children (ISKDC) based on mesangial hypercellularity and the extent of glomerular crescents. Macrophages, categorized as M1 or M2, frequently infiltrate in various glomerular and tubulointerstitial diseases and infiltration of specific subtypes is associated with disease progression. Therefore, to identify whether infiltration of M1 or M2 macrophages has clinical significance, we quantified the subtypes of macrophages in 49 HSPN specimens and correlated the counts with histologic features and clinical parameters. Higher tubulointerstitial M2 counts were associated with chronic renal failure (CRF), ISKDC classes III-IV, and crescents (P<0.001, 0.002, 0.001). Glomerular M2 counts were significantly related to ISKDC classes III-IV and crescents (area under curve, AUC 0.804, 0.833). Tubulointerstitial M2 counts were associated with CRF, ISKDC classes III-IV, and crescents (AUC 0.872, 0.778, 0.830). Tubulointerstitial M2 counts also revealed higher AUC than tubulointerstitial M1 counts for CRF (P=0.036) and ISKDC classes III-IV (P=0.047). Glomerular M2 counts revealed higher AUC than glomerular M1 counts for ISKDC classes III–IV (P=0.024). Tubulointerstitial M2 counts were the most powerful parameter for CRF (AUC 0.872) and revealed even higher AUC than ISKDC classification (AUC 0.716) with borderline significance (P=0.086) for CRF. In summary, tubulointerstitial M2 counts were a superior parameter to tubulointerstitial M1 counts and even to ISKDC classification indicating the presence of CRF.

Download Full-text

Successful Outcome of a Corticodependent Henoch-Schönlein Purpura Adult with Rituximab

Case Reports in Medicine ◽

10.1155/2014/619218 ◽

2014 ◽

Vol 2014 ◽

pp. 1-4 ◽

Cited By ~ 17

Author(s):

Taylor Pindi Sala ◽

Jean-Marie Michot ◽

Renaud Snanoudj ◽

Marion Dollat ◽

Emmanuel Estève ◽

...

Keyword(s):

Systemic Vasculitis ◽

Transplant Rejection ◽

Immunoglobulin A ◽

Corticosteroid Treatment ◽

Successful Outcome ◽

Henoch Schonlein Purpura ◽

Small Vessels ◽

History Of ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a systemic vasculitis involving small vessels with deposition of immunoglobulin A (IgA) complexes, usually affecting children. Compared with children, HSP in adults is more severe and frequently associated with cancer. We report the case of a 49-year-old woman with medical history of kidney transplantation for segmental glomerular hyalinosis. Eight years after the transplantation, while taking combined immunosuppressive therapy with tacrolimus and azathioprine indicated for the prevention against transplant rejection, she developed a Henoch-Schönlein purpura. Vasculitis involves skin and sciatic peroneal nerve and she received systemic corticosteroid treatment. Because of four relapses and corticosteroid dependence, the patient was treated with rituximab (two intravenous infusions of 1000 mg given two weeks apart). Successful outcome was observed along two years of follow-up. This new case of successful use of rituximab in HSP promotes more investigations of this treatment in clinical trials.

Download Full-text

Predicting renal outcomes in immunoglobulin A vasculitis nephritis; from ISKDC classification to Oxford MEST-C classification

Journal of Renal Injury Prevention ◽

10.15171/jrip.2020.08 ◽

2019 ◽

Vol 9 (1) ◽

pp. 08-08 ◽

Cited By ~ 1

Author(s):

Mohsen Akhavan Sepahi ◽

Muhammed Mubarak

Keyword(s):

Kidney Disease ◽

Renal Disease ◽

Iga Nephropathy ◽

Immunoglobulin A ◽

Iga Vasculitis ◽

Oxford Classification ◽

Schonlein Purpura ◽

Immunoglobulin A Vasculitis ◽

Purpura Nephritis

Immunoglobulin A vasculitis nephritis (IgAVN) or Henoch-Schönlein purpura nephritis (HSPN) is the most common type of secondary IgA nephropathy (IgAN), particularly in children. Overall, its prognosis is good, but in a significant number of cases, it does lead to chronic kidney disease (CKD) and end-stage renal disease (ESRD). Various classifications exist to predict the long-term outcome of kidney disease in these diseases, but none is universally accepted. We searched PubMed, Web of Science Embase, EBSCO, Scopus and directory of open access journals (DOAJ) with keywords of Henoch-Schönlein purpura nephritis, immunoglobulin A vasculitis nephritis, immunoglobulin A nephropathy, IgA vasculitis, extracapillary proliferation, IgA nephropathy, leukocytoclastic vasculitis, endstage renal disease, Oxford classification, crescent and childhood IgA vasculitis. IgAVN in children presents most often with crescents and endocapillary proliferation, with relapsing and remitting course clinically. Due to morphological resemblance of IgAVN and IgAN, the Oxford MEST-C scores could be applied for determining the long-term outcomes in the former disease. A critical concern in applying Oxford classification for IgAVN is that limited number of children with IgAVN exists in any one center and also a relatively short period of follow-up. Hence, further work in this regard is necessary. Preliminary evidence suggests that Oxford MEST-C classification is valid in predicting long-term kidney outcomes in children with IgAVN and the classification can also be used in adults. However, further, large scale, multicenter, international collaborative studies are needed to address the unmet issues.

Download Full-text

IgA Vasculitis and COVID-19 in Children: A Systematic Review

10.21203/rs.3.rs-1043944/v1 ◽

2021 ◽

Author(s):

Eva Maria Glenn Lecea ◽

Daniela Aguilar Abisad ◽

Ana Paredes ◽

Arielle Hay

Keyword(s):

Systematic Review ◽

Causality Assessment ◽

Case Reports ◽

Meta Analysis ◽

Renal Involvement ◽

Immunoglobulin A ◽

Case Series ◽

Iga Vasculitis ◽

Female Ratio ◽

Inflammatory Conditions

Abstract Background: Immunoglobulin A vasculitis is the most common form of vasculitis in children. The diagnosis is made clinically and patients will present with a rash, together with gastrointestinal, musculoskeletal, and renal system involvement. Progress in the classification of the systemic vasculitides has facilitated better understanding of the pathogenesis underlying these inflammatory conditions. Over the past year, several cases of IgA vasculitis have been reported in both children and adults in association with SARS-CoV2 infection, raising the question of whether there is any causal or even a synergistic association.Methods: This systematic review was performed following the guidelines of Meta-analysis of Observational Studies in Epidemiology. A literature search was conducted using MEDLINE, SciELO and Google Scholar using the search terms “COVID-19” or “SARS-CoV-2" in combination with “IgA vasculitis”, or “Henoch-Schonlein Purpura”. We considered articles to be eligible for inclusion if they reported a case report or series of cases of IgA vasculitis associated with proven COVID-19 infection. We excluded cases from further review if the case reported was a patient older than 18 years. WHO causality assessment categories were used to standardize case causality. Results: After reviewing the complete article and applying our exclusion criteria, 12 articles describing 12 cases of COVID-19 associated IgA vasculitis in children were included. In 83% of the cases the diagnosis of COVID-19 was made on presentation of IgA symptoms or on presentation to seek medical care. In 17% of cases the SARS Cov-2 test was positive before IgA vasculitis symptoms presentation. The mean age of the patients was 7.3 years of age (SD ±4.8). Male to female ratio was 3:1. Lower extremity purpura was present in all 12 patients. Gastrointestinal manifestations were present in 7 patients. Oligoarthritis was present in 7 patients. Three patients presented renal involvement with hematuria/proteinuria. Conclusions: During the pandemic, several autoimmune phenomena have been described to co-occur with or following COVID-19. The exact role of COVID-19 in the development of these IgA-related diseases is still being explored. Our review of case series and case reports with standardized causality assessment identified 12 cases of IgA vasculitis associated with COVID-19 infection in children.

Download Full-text