IgA vasculitis (Henoch-Schonlein purpura)
Summary
Definition
History and exam
Key diagnostic factors
- presence of risk factors
- rash
- arthralgias
- abdominal pain
- signs of renal disease
Other diagnostic factors
- drug history
- scrotal pain or swelling
- headaches
- seizures
- pulmonary haemorrhage
Risk factors
- genetics
- male sex
- age 2 to 10 years
- increasing age at onset
- winter season/prior infection
- medication
Diagnostic investigations
1st investigations to order
- blood pressure
- urinalysis
- serum creatinine and electrolyte levels
Investigations to consider
- serum IgA levels
- FBC and coagulation studies
- skin biopsy
- renal biopsy
- ultrasound, abdomen
- ultrasound, testicles
Treatment algorithm
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 persistent proteinuria
persistent proteinuria
Contributors
Authors
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
UK
Disclosures
LO is an author of references cited in this topic.
Peer reviewers
R. John Presutti, DO
Consultant
Department of Family Medicine
Mayo Clinic
Jacksonville
FL
Disclosures
RJP declares that he has no competing interests.
Rajan Madhok, MD, FCRP
Consultant Physician and Rheumatologist
Centre for Rheumatic Diseases
Glasgow Royal Infirmary
Glasgow
UK
Disclosures
RM declares that he has no competing interests.
Richard A. Watts, MA, DM, FRCP
Consultant Rheumatologist
Ipswich Hospital NHS Trust
Ipswich
UK
Disclosures
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.
References
Key articles
Ozen S, Pistorio A, Iusan SM, et al; Paediatric Rheumatology International Trials Organisation (PRINTO). EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria. Ann Rheum Dis. 2010 May;69(5):798-806.Full text Abstract
Mills JA, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Henoch-Schonlein purpura. Arthritis Rheum. 1990 Aug;33(8):1114-21. Abstract
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.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Idiopathic thrombocytopenic purpura (ITP)
- Hypersensitivity vasculitis
- Granulomatosis with polyangiitis
More DifferentialsGuidelines
- Henoch-Schönlein purpura
- European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis - the SHARE initiative
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