IgA vasculitis (Henoch-Schonlein purpura)

Обзор 
Теория 
Диагноз 
Лечение 
Последующее наблюдение 
Источники 

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Последний просмотренный: 9 Aug 2026

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.

Определение

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.

Анамнез и осмотр

Ключевые диагностические факторы

rash
arthralgias
abdominal pain
signs of renal disease

Полная информация

Другие диагностические факторы

drug history
scrotal pain or swelling
headaches
seizures
pulmonary hemorrhage

Полная информация

Факторы риска

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

Полная информация

Диагностические исследования

Исследования, которые показаны в первую очередь

blood pressure
urinalysis
serum creatinine and electrolyte levels

Полная информация

Исследования, проведение которых нужно рассмотреть

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

Полная информация

Алгоритм лечения

Острый

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

ПРОДОЛЖЕНИЕ

relapsed/refractory disease

Составители

Авторы

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

Раскрытие информации

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.

Рецензенты

David Kershaw, MD

Professor of Pediatrics

University of Michigan

Ann Arbor

Раскрытие информации

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

Раскрытие информации

RM declares that he has no competing interests.

Richard A. Watts, MA, DM, FRCP

Consultant Rheumatologist

Ipswich Hospital NHS Trust

Ipswich

Раскрытие информации

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.

Список литературы

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Основные статьи

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.Полный текст Аннотация

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

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

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.Полный текст Аннотация

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.Полный текст

Статьи, указанные как источники

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Отличия
- Idiopathic thrombocytopenic purpura (ITP)
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Рекомендации
- 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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Использование этого контента попадает под действие нашего заявления об отказе от ответственности

IgA vasculitis (Henoch-Schonlein purpura)

Резюме

Определение

Анамнез и осмотр

Ключевые диагностические факторы

Другие диагностические факторы

Факторы риска

Диагностические исследования

Исследования, которые показаны в первую очередь

Исследования, проведение которых нужно рассмотреть

Алгоритм лечения

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

Составители

Авторы

Louise Oni, MBChB, MRCPCH, MA, PhD

Раскрытие информации

Рецензенты

David Kershaw, MD

Раскрытие информации

Rajan Madhok, MD, FCRP

Раскрытие информации

Richard A. Watts, MA, DM, FRCP

Раскрытие информации

Peer reviewer acknowledgements

Disclosures

Список литературы

Основные статьи

Статьи, указанные как источники

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Отличия

Рекомендации

Videos

Войдите в учетную запись или оформите подписку, чтобы получить полноценный доступ к BMJ Best Practice

Войдите в учетную запись или оформите подписку, чтобы получить полноценный доступ к BMJ Best Practice

Войдите в учетную запись, чтобы получить полноценный доступ к BMJ Best Practice