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QJM Advance Access published online on May 6, 2005
QJM, doi:10.1093/qjmed/hci072
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© The Author 2005. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org
Received November 19, 2004
Revised March 11, 2005

Original article

Treatment and outcome of adult patients with primary focal segmental glomerulosclerosis in five UK renal units

C.M. Stirling 1*, P. Mathieson 2, J.M. Boulton-Jones 1, J. Feehally 3, D. Jayne 4, H.M. Murray 5, and D. Adu 6

1 From the Glasgow Royal Infirmary, Glasgow, UK
2 From the Southmead Hospital, Bristol, UK
3 From the Leicester General Hospital, Leicester, UK
4 From the St Helier Hospital, Carshalton, UK
5 From the Robertson Centre for Biostatistics, University of Glasgow, Glasgow, UK
6 From the Queen Elizabeth Hospital, Birmingham, UK

* To whom correspondence should be addressed.
C.M. Stirling, E-mail: cathstirling{at}hotmail.com


  Abstract

Background: Focal segmental glomerulosclerosis (FSGS) is the least studied of the causes of idiopathic nephrotic syndrome, and there are few specific guidelines for treatment.

Aim: To review data from five UK renal units to investigate whether adult patients with FSGS were treated uniformly, and to examine the effect of treatment on proteinuria and survival.

Design: Retrospective record review.

Methods: We examined electronic records of patients with idiopathic FSGS for information on baseline clinical parameters, treatment regimens and outcomes.

Results: Of 136 patients with primary FSGS and nephrotic range proteinuria, 76 (56%) were treated with prednisolone and of this group, 59% were treated with additional immunosuppression. Among the treated patients, the total remission rate (complete and partial) was 67%, and one hospital achieved a remission rate of 80%. Treated patients had a significantly higher remission rate than those who were not treated. Remission was associated with a 5-year survival off dialysis of 94%, compared with 53% if remission was not achieved. Baseline serum creatinine and remission were independently associated with survival off dialysis in a multivariate Cox proportional hazards model.

Discussion: Patients with primary FSGS and nephrotic range proteinuria, who are treated with corticosteroids, are more likely to enter remission than those who are not treated. Remission rates of up to 80% can be achieved with prolonged treatment, and remission is an independent predictor of survival off dialysis. Patients who do not achieve remission have a poor prognosis. Further clarification of optimal treatment regimens requires additional, prospective studies.


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O. Moranne, L. Watier, J. Rossert, B. Stengel, and The GN-Progress Study Group
Primary glomerulonephritis: an update on renal survival and determinants of progression
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[Abstract] [Full Text] [PDF]


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