Immunoglobulin A vasculitis

Immunoglobulin A vasculitis (IgA vasculitis), formerly known as Henoch-Schönlein purpura (HSP), is the most common systemic vasculitis of childhood. It is characterized by inflammation of small blood vessels (small-vessel vasculitis) with deposition of IgA-containing immune complexes in affected tissues. The disease primarily affects the skin, gastrointestinal tract, joints, and kidneys. The hallmark clinical feature is palpable purpura — a raised, non-blanching rash typically distributed over the lower extremities and buttocks. Other key manifestations include abdominal pain (often colicky, sometimes accompanied by gastrointestinal bleeding), arthritis or arthralgia (particularly affecting the knees and ankles), and renal involvement ranging from mild hematuria and proteinuria to, in a minority of cases, progressive glomerulonephritis that can lead to chronic kidney disease. IgA vasculitis frequently follows an upper respiratory tract infection, and various infectious agents, medications, and environmental triggers have been implicated. The condition predominantly affects children between the ages of 3 and 10 years, though it can occur at any age. Adults tend to have more severe disease, particularly with regard to renal complications. The disease is generally self-limiting in children, with most cases resolving within weeks to months. Treatment is primarily supportive, including adequate hydration, rest, and nonsteroidal anti-inflammatory drugs (NSAIDs) for joint pain. Corticosteroids may be used for severe gastrointestinal symptoms or significant renal involvement. In cases of progressive nephritis, immunosuppressive agents such as cyclophosphamide, azathioprine, or mycophenolate mofetil may be considered. Long-term follow-up is recommended to monitor for renal complications, which represent the most important determinant of long-term prognosis.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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