#2645 Clinical features and outcomes of steroid resistant focal sclerosing glomerulosclerosis (FSGS) in a single tertiary nephrology centre in Malaysia

Abstract

Abstract Background and Aims Steroid resistant FSGS is defined as persistence of proteinuria with <50% reduction from baseline despite prednisolone 1 mg/kg/d for at least 16 weeks. We aim to study the clinical presentations, treatment, treatment response and kidney survival among steroid resistant FSGS patients. Method This retrospective study included all patients who had undergone renal biopsy in HSIS and had documented diagnosis of steroid resistant FSGS from January 2007 until December 2022. Data was collected and analysed using SPSS version 17. Results are expressed in mean ± standard deviation (SD) unless stated otherwise. Results A total of 74 patients had primary FSGS out of which 15 were identified as steroid resistant. Median age was 33 years old with 53% being female. Median weight at diagnosis was 73.7kg with BMI of 28kg/m2. One third had no co-morbid disease while 4 patients had underlying diagnosis of minimal change disease. Proteinuria on presentation was 7.6g with albumin levels of 16 g/L. No difference was seen between steroid resistant and steroid sensitive cohort with regards to age, proteinuria, GFR and laboratory parameters. Median duration of steroid was 38.8 months with duration of high dose steroids were 5 months and complications often set in by 2 months of therapy. Steroid toxicity occurred in 13 patients with most common complication seen was Cushingoid features (8/13) followed by acne (2/13) and diabetes mellitus (2/13). There were more steroid complications in steroid resistant cohort compared to the steroid sensitive. Second line was initiated in all patients but one with cyclosporin A being the most common choice (11/14) followed by Azathioprine (2/14) and cyclophosphamide (3/14). The median duration of second line agent was 22 months with CNI specifically being for 18 months. 3 patients developed CNI toxicity and was given cyclophosphamide while one patient developed CNI resistance and was given IV Rituximab. 4 (%) patients went on to achieve complete remission, 8 had partial remission while the rest did not achieve remission. 9 patients had normal renal function, while 3 developed chronic kidney disease. One patient went on to become end stage renal disease while 2 patients died. There was no difference in terms of renal or patient outcome between steroid resistant and steroid sensitive FSGS. Conclusion Steroid resistant FSGS is a challenging entity of FSGS that warrants close monitoring for complications and management. The outcomes were not different between steroid sensitive and steroid resistant FSGS however, the steroid complications were more common in steroid resistant FSGS.