NDT Advance Access originally published online on July 27, 2004
Nephrology Dialysis Transplantation 2004 19(10):2437-2444; doi:10.1093/ndt/gfh320
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Nephrol Dial Transplant Vol. 19 No. 10 © ERA-EDTA 2004; all rights reserved
Editorial Review
Nephrotic focal segmental glomerulosclerosis in 2004: an update
Hôpitaux Broussais et HEGP, Paris, Nephrology and INSERM U430 F-75015 Paris, France
Correspondence and offprint requests to: Alain Meyrier, Service de Néphrologie & INSERM U 430, University Hospitals Broussais & HEGP, 20 rue Leblanc, F-75015 Paris, France. Email: alain.meyrier@brs.ap-hop-paris.fr
Keywords: focal segmental glomerulonephritis (FSGS); genetics of FSGS; idiopathic nephrotic syndrome; immunosuppressive therapy; podocyte
| The first 150 words of the full text of this article appear below. |
Writing on the treatment of primary focal segmental glomerulosclerosis (FSGS) is a challenge. The jaded nephrologist is tempted to consider that the issue could be summarized as Treatment of a disease which is not a disease but a lesion of obscure pathophysiology, with drugs the mechanism of action of which is poorly understood and flinch from the task. However, the knowledge on this topic has so tremendously progressed over recent years that obsolescence looms with each passing year. Let us try to analyse the subject using a Cartesian approach, that is, dissecting the problem step by step. This review will focus on the nephrotic forms of this nephropathy, as mildly proteinuric FSGS is probably a different entity in terms of pathophysiology, prognosis and treatment.
FSGS is not a disease but a lesion with no definite prognostic value
The denomination of the lesion is misleading, as it is not always focal, or segmental, or even sclerotic. Hence, there is a multiplicity of terms, none
The common denominator of all FSGS variants is a podocyte disease
FSGS, an immunological podocyte disease
FSGS, a viral podocyte disease
FSGS, a toxic podocyte disease
FSGS, an inherited podocyte disease
Treatment of FSGS with corticosteroids
Treatment of FSGS with alkylating agents
Treatment of FSGS with CsA
Treatment of FSGS with other immunophillin modulators
Tacrolimus (FK-506)
Sirolimus (rapamycin)
Treatment of FSGS with mycophenolate mofetil
Relapse on transplanted kidney
Conclusion
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