Abrupt and durable remission of Henoch-Schönlein purpura nephritis with cyclosporine A

NDT Plus. 2008 Oct;1(5):300-2. doi: 10.1093/ndtplus/sfn105. Epub 2008 Aug 1.

Abstract

Henoch-Schönlein purpura glomerulonephritis (HSP-GN) is a common form of systemic small vessel vasculitis in children. Although prognosis is usually favourable, the disease is occasionally associated with a risk of renal insufficiency. Various immunosuppressive agents have been used in patients with severe HSP-GN, but none have shown convincing favourable effects. We report a case of biopsy-proven HSP-related GN in a 4-year-old girl that responded remarkably well to cyclosporine A (CsA), following failure to respond to other immunosuppressive agents. At 8 months post-CsA treatment, repeat renal biopsy findings were consistent with histological improvement. We conclude that CsA treatment not only exerts beneficial effects on resistant HSP-related GN but may also arrest progression of the disease.

Keywords: Henoch–Schönlein purpura; cyclosporine A; glomerulonephritis.