Cyclosporin therapy in patients with Alport syndrome

Alport syndrome (AS) is a hereditary disorder of type IV collagen characterized by the association of progressive hematuric nephritis and sensorineural hearing loss. An increase in proteinuria is linked with progressive renal failure. Preliminary data have shown that cyclosporin therapy reduces prot...

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Published in:Pediatric nephrology (Berlin, West) West), 2007-01, Vol.22 (1), p.57-63
Main Authors: Charbit, Marina, Gubler, Marie-Claire, Dechaux, Michèle, Gagnadoux, Marie-France, Grünfeld, Jean-Pierre, Niaudet, Patrick
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biopsy
Child
Creatinine - blood
Cyclosporine - adverse effects
Cyclosporine - therapeutic use
Female
Glomerular Filtration Rate - drug effects
Glomerular Filtration Rate - physiology
Humans
Immunosuppressive Agents - adverse effects
Immunosuppressive Agents - therapeutic use
Inulin - blood
Kidney - drug effects
Kidney - pathology
Kidney Failure, Chronic - etiology
Male
Nephritis, Hereditary - drug therapy
Nephritis, Hereditary - physiopathology
Proteinuria - complications
Proteinuria - prevention & control
Treatment Outcome
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Summary:Alport syndrome (AS) is a hereditary disorder of type IV collagen characterized by the association of progressive hematuric nephritis and sensorineural hearing loss. An increase in proteinuria is linked with progressive renal failure. Preliminary data have shown that cyclosporin therapy reduces proteinuria, thereby suggesting that it may also slow the progression of AS nephropathy. We treated nine AS patients manifesting proteinuria >1 g/m(2)/day and a glomerular filtration rate (GFR) >50 ml/min/1.73 m(2) with cyclosporin for at least 6 months. At the end of this 6-month period, mean proteinuria had decreased from 2+/-1.06 to 0.65+/-0.73 g/day, and mean albuminemia had increased from 29+/-5.2 to 35+/-6.5 g/l. Mean inulin clearance had decreased from 102+/-29 to 74+/-16.3 ml/min/1.73 m(2). Cyclosporin treatment was stopped in four patients because of inefficacy or adverse effects and continued in the remaining five patients for an additional 14-42 months. At the end of this second treatment period, control renal biopsies revealed significant lesions of cyclosporin nephrotoxicity in three patients. Based on these results we conclude that while cyclosporin therapy can decrease proteinuria in most patients with AS, it may be associated with nephrotoxicity, thereby precluding its long-term use.
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-006-0227-y