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
- Idiopathic thrombocytopenic purpura (ITP)
- 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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