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.
Long-term complications are rare but there is a risk of chronic kidney disease.
Early, accurate diagnosis is important to initiate appropriate management.
IgA vasculitis (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.
In 2012, the International Chapel Hill Consensus Conference Nomenclature of Vasculitides replaced the eponym Henoch-Schonlein purpura with immunoglobulin A vasculitis (IgAV) to better reflect the pathophysiology. However, the two terms are still used interchangeably in clinical practice.
History and exam
Key diagnostic factors
- abdominal pain
- signs of renal disease
Other diagnostic factors
- drug history
- scrotal pain or swelling
- pulmonary hemorrhage
- male sex
- age 2 to 10 years
- increasing age at onset
- winter season/prior infection
1st investigations to order
- blood pressure
- serum creatinine and electrolyte levels
Investigations to consider
- serum IgA levels
- CBC and coagulation studies
- skin biopsy
- renal biopsy
- ultrasound, abdomen
- ultrasound, testicles
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
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
LO is an author of references cited in this topic.
R. John Presutti, DO
Department of Family Medicine
RJP declares that he has no competing interests.
Rajan Madhok, MD, FCRP
Consultant Physician and Rheumatologist
Centre for Rheumatic Diseases
Glasgow Royal Infirmary
RM declares that he has no competing interests.
Richard A. Watts, MA, DM, FRCP
Ipswich Hospital NHS Trust
RAW declares that he has no competing interests.
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- Hypersensitivity vasculitis
- Granulomatosis with polyangiitis
- Henoch-Schönlein purpura
- European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis - the SHARE initiative
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