Abrupt and durable remission of Henoch-Schonlein purpura nephritis with cyclosporine A
E. Georgaki-Angelaki, Stavroula Kostaridou, Athanasia Lourida, Constantina D. Petraki, Evagelia Lagona
Abstract
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E. Georgaki-Angelaki, Stavroula Kostaridou, Athanasia Lourida, Constantina D. Petraki, Evagelia Lagona
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.
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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 concepts: Medicine, Henoch-Schonlein purpura, Nephritis, Purpura (gastropod), Renal biopsy, Biopsy, Gastroenterology, Vasculitis