NDT Plus Advance Access originally published online on August 1, 2008
NDT Plus 2008 1(5):300-302; doi:10.1093/ndtplus/sfn105
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Abrupt and durable remission of Henoch–Schönlein purpura nephritis with cyclosporine A
1 Department of Paediatric Nephrology
2 Department of Paediatrics, University of Athens, Aghia Sophia Children's Hospital, Athens 11527
3 Nephropathology Department, Evangelismos Hospital, Athens, Greece
Correspondence: Correspondence and offprint requests to: Eleni Georgaki-Angelaki, Department of Paediatric Nephrology, Aghia Sophia Children's Hospital Athens, Thivon and Levadias Street, Athens 11527, Greece. Tel: +30-2107467097; Fax: +30-2107467097; E-mail: pednephr{at}paidon-agiasofia.gr
| Abstract |
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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.
Key Words: cyclosporine A glomerulonephritis Henoch–Schönlein purpura
Received for publication April 15, 2008. Accepted for publication June 26, 2008.