Idiopathic focal segmental glomerulosclerosis: a favourable prognosis in untreated patients?
| Autor: | Deegens, J. K. J., Assmann, K. J. M., Steenbergen, E. J., Luuk Hilbrands, Gerlag, P. G. G., Jansen, J. L. J., Wetzels, J. F. M. |
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| Rok vydání: | 2005 |
| Předmět: | |
| Zdroj: | Netherlands Journal of Medicine, 63, 10, pp. 393-8 Netherlands Journal of Medicine, 63, 393-8 ResearcherID |
| ISSN: | 0300-2977 |
| Popis: | Contains fulltext : 47572.pdf (Publisher’s version ) (Closed access) BACKGROUND: Patients with focal segmental glomerulosclerosis (FSGS) are considered to have a poor prognosis and spontaneous remissions are seldom reported. However, FSGS is not a single disease entity. Our aim was to describe the clinical course in initially untreated patients with recently diagnosed idiopathic FSGS. METHODS: This was a retrospective study of patients with a diagnosis of FSGS by histology, who fulfilled the following criteria: proteinuria >3.5 g/day, normal renal function, duration of proteinuria or hypertension of less than one year, normal-sized kidneys, no underlying renal disease, and a negative family history. Renal biopsies were reviewed without knowledge of the clinical course. RESULTS: Twenty patients (13 male, 7 female) fulfilled the study criteria. Median age was 49.3 (range 21.8 to 73.0) years, serum creatinine 90 +/- 20 micromol/l, proteinuria 10.0 +/- 5.5 g/day and serum albumin 24 +/- 6 g/l. After a median follow-up of 9.4 (2.1-18.6) years, 13 patients (65%) were in remission of proteinuria. Renal function deterioration occurred in seven patients, and prompted treatment in four of them. The ten-year death-censored renal survival was 89%. Renal function deterioration and remission rate could be predicted by selectivity index, serum albumin at three months after renal biopsy and the percentage of glomeruli with segmental sclerosis. CONCLUSION: Focal glomerulosclerosis is not a single disease. Case definition using strict clinical criteria identifies a subgroup of patients with idiopathic FSGS who have a good prognosis. In the majority of these patients immunosuppressive therapy is not warranted. |
| Databáze: | OpenAIRE |
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