IgA vasculitis (Henoch-Schonlein purpura)

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 11 Aug 2025

Last updated: 08 Apr 2025

IgA vasculitis (formerly known as Henoch-Schonlein purpura) is the most common vasculitis of childhood.

Rash of palpable purpura is present in all cases.

Most cases are self-limiting or resolve with symptomatic treatment. Immunosuppressive therapy is indicated for nephritis, orchitis, and other organ- or life-threatening manifestations.

Long-term complications are rare but there is a risk of chronic kidney disease.

Early, accurate diagnosis is important to initiate appropriate management.

Definition

Immunoglobulin A vasculitis (IgAV; formerly known as Henoch-Schonlein purpura [HSP]) is the most common vasculitis of childhood and presents with a purpuric rash, abdominal pain, arthritis/arthralgia, and glomerulonephritis.[1]

In 2012, the International Chapel Hill Consensus Conference Nomenclature of Vasculitides replaced the eponym Henoch-Schonlein purpura with IgAV to better reflect the pathophysiology.[2][3] However, the two terms are still sometimes used interchangeably in clinical practice. This topic focuses on the diagnosis and management of IgAV in children only. IgAV in adults is rare and beyond the scope of this topic.

História e exame físico

Principais fatores diagnósticos

rash
arthralgias
abdominal pain
signs of renal disease

Detalhes completos

Outros fatores diagnósticos

drug history
scrotal pain or swelling
headaches
seizures
pulmonary hemorrhage

Detalhes completos

Fatores de risco

genetics
allergy
male sex
age 2 to 10 years
increasing age at onset
winter season/prior infection
medication

Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

blood pressure
urinalysis
serum creatinine and electrolyte levels

Detalhes completos

Investigações a serem consideradas

serum IgA levels
CBC and coagulation studies
skin biopsy
renal biopsy
ultrasound, abdomen
ultrasound, testicles

Detalhes completos

Algoritmo de tratamento

AGUDA

without renal involvement

mild nephritis: normal GFR and mild or moderate proteinuria

moderate nephritis: <50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria

severe nephritis: >50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria

CONTÍNUA

relapsed/refractory disease

Colaboradores

Autores

Louise Oni, MBChB, MRCPCH, MA, PhD

Senior Lecturer in Paediatric Nephrology and Honorary Consultant Paediatric Nephrologist

University of Liverpool

Department of Paediatric Nephrology

Alder Hey Children's NHS Foundation Trust Hospital

Liverpool

Declarações

In the past 12 months LO has contributed to expert advisory roles for Boehringer Ingelheim and Biocryst pharmaceuticals. LO has received a travel grant from Sandoz. These are unrelated to this article and all monetary payments are placed into an institutional educational charity account. LO is an author of references cited in this topic.

Revisores

David Kershaw, MD

Professor of Pediatrics

University of Michigan

Ann Arbor

Declarações

DK is co-medical director for the board of directors of Nephcure.

Rajan Madhok, MD, FCRP

Consultant Physician and Rheumatologist

Centre for Rheumatic Diseases

Glasgow Royal Infirmary

Glasgow

Declarações

RM declares that he has no competing interests.

Richard A. Watts, MA, DM, FRCP

Consultant Rheumatologist

Ipswich Hospital NHS Trust

Ipswich

Declarações

RAW declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Ozen S, Marks SD, Brogan P, et al. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis - the SHARE initiative. Rheumatology (Oxford). 2019 Sep 1;58(9):1607-16.Texto completo Resumo

UK Kidney Association. The initial management of IgA vasculitis (Henoch Schönlein Purpura) in children and young people. Dec 2022 [internet publication].Texto completo

UK Kidney Association. The management of complications associated with IgA vasculitis (Henoch Schönlein Purpura) in children and young people. Dec 2022 [internet publication].Texto completo

Hahn D, Hodson EM, Craig JC. Interventions for preventing and treating kidney disease in IgA vasculitis. Cochrane Database Syst Rev. 2023 Feb 28;(2):CD005128.Texto completo Resumo

Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021 Oct;100(4S):S1-276.Texto completo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

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Diretrizes
- The initial management of IgA vasculitis (Henoch Schönlein Purpura) in children and young people
- The management of complications associated with IgA vasculitis (Henoch Schönlein Purpura) in children and young people
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IgA vasculitis (Henoch-Schonlein purpura)

Summary

Definition

História e exame físico

Principais fatores diagnósticos

Outros fatores diagnósticos

Fatores de risco

Investigações diagnósticas

Primeiras investigações a serem solicitadas

Investigações a serem consideradas

Algoritmo de tratamento

without renal involvement

mild nephritis: normal GFR and mild or moderate proteinuria

moderate nephritis: <50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria

severe nephritis: >50% crescents on renal biopsy and impaired GFR or severe persistent proteinuria

relapsed/refractory disease

Colaboradores

Autores

Louise Oni, MBChB, MRCPCH, MA, PhD

Declarações

Revisores

David Kershaw, MD

Declarações

Rajan Madhok, MD, FCRP

Declarações

Richard A. Watts, MA, DM, FRCP

Declarações

Peer reviewer acknowledgements

Disclosures

Referências

Principais artigos

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais

Diretrizes

Videos

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se para acessar todo o BMJ Best Practice