Publication:

Clinical heterogeneity in biopsy-proven adult focal segmental glomerulosclerosis

Focal segmental glomerulosclerosis (FSGS) is a histopathological pattern of podocyte injury and a major cause of nephrotic syndrome in adults. Despite a common histopathological diagnosis, patients may exhibit considerable variability in clinical presentation, disease severity, therapeutic response, and renal outcomes. This case series highlights the diverse clinical spectrum of biopsy-proven adult FSGS encountered in routine clinical practice.We report three adult female patients with biopsy-proven focal segmental glomerulosclerosis managed at a rural tertiary care teaching hospital. The first patient presented with long-standing nephrotic syndrome complicated by severe hypoalbuminemia, generalized edema, gross ascites, liver cirrhosis, and poor treatment adherence. The second patient developed relapsing nephrotic syndrome with persistent nephrotic-range proteinuria despite corticosteroid therapy, requiring treatment escalation and emergency hospitalization. The third patient presented predominantly with progressive acute kidney injury and deteriorating renal function in the presence of multiple comorbidities, representing an atypical clinical presentation of FSGS. Renal biopsy established the diagnosis in all three patients and guided individualized management.This case series demonstrates the marked clinical heterogeneity of biopsy-proven adult FSGS despite a common histopathological diagnosis. The findings emphasize the importance of timely renal biopsy, comprehensive clinicopathological correlation, and individualized therapeutic strategies for accurate diagnosis and optimal management.