Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review

Henoch-Schönlein purpura, now called immunoglobulin A (IgA) vasculitis, is a systemic, immune complex-mediated, small-vessel leukocytoclastic vasculitis characterized by nonthrombocytopenic palpable purpura, arthritis, and abdominal pain. It is the most common vasculitis in children but can also occur in adults. Diagnostic testing is required only to exclude other etiologies of purpura, to identify renal involvement, and, if indicated, to determine its extent with biopsy. Imaging or endoscopy may be needed to assess organ complications. IgA vasculitis spontaneously resolves in 94% of children and 89% of adults, making supportive treatment the primary management strategy. However, a subset of patients experience renal involvement that can persist and relapse years later. Additional complications can include gastrointestinal bleeding, orchitis, and central nervous system involvement. Systematic reviews have shown that steroids do not prevent complications and should not be used prophylactically. However, randomized trials have demonstrated success with high-dose steroids, cyclosporine, and mycophenolate in treating glomerulonephritis and other complications. Long-term prognosis depends on the extent of renal involvement. Six months of follow-up is prudent to assess for disease relapse or remission.