Renal survival and treatment of adult patients with Primary Focal Segmental glomerulosclerosis: A historical cohort study of the National Greek Registry

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BACKGROUND/OBJECTIVE: Primary Focal and Segmental glomerulosclerosis (FSGS) is one of the most common causes of idiopathic nephrotic syndrome. Our aim was to describe a large cohort of patients with primary FSGS, identify risk factors associated with worse renal survival and assess the impact of different immunosuppressive regiments on renal survival. METHODS: This was a historical cohort study of adults who were diagnosed with primary FSGS from March 26, 1982, to September 16, 2020. The primary outcome was progression to ESRD. RESULTS: We included 579 patients. The mean age was 46 (±15) years of age, with 378 (65%) males and median 24-hour proteinuria was 3.8 (2-6) g. In multivariable analysis only eGFR (HR: 0.97 per ml/min increase, 95% CIs 0.95-0.98) and remission status (complete remission (HR: 0.03, 95% CIs 0.003-0.22) and partial remission (HR: 0.28, 95% CIs 0.13-0.61) compared to no remission) were associated with renal survival. Among patients who received immunosuppression compared to those that did not, there was a higher percentage of complete remission (121 (41%) vs. 40 (24%), p<0.001), and higher percentage of relapses (135 (64%) vs. 27 (33%), p<0.001). Immunosuppression and its type (glucocorticoids vs. cyclosporine ± glucocorticoids) were not associated with renal survival. CONCLUSION: In primary FSGS, complete and partial remission were associated with improved renal survival. Further randomized studies are needed to assess the efficacy of different therapeutic agents and guide treatment.

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