Chapter 509 Henoch-Schönlein Purpura Nephritis
Henoch-Schönlein purpura (HSP) is the most common small vessel vasculitis in childhood. It is characterized by a purpuric rash and commonly accompanied by arthritis and abdominal pain (Chapter 161.1). Approximately 50% of patients with HSP develop renal manifestations, which vary from asymptomatic microscopic hematuria to severe, progressive glomerulonephritis.
Pathogenesis and Pathology
The pathogenesis of HSP nephritis appears to be mediated by the deposition of polymeric immunoglobulin A (IgA) in glomeruli. This is analogous to the same type of IgA deposits seen in systemic small vessels, primarily those of the skin and intestine. The glomerular findings can be indistinguishable from those of IgA nephropathy. IgA deposits are present by immunofluorescence, and a broad spectrum of glomerular lesions that can range from mild proliferation to necrotic and crescentic changes can be seen.
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