Clinical heterogeneity in biopsy-proven adult focal segmental glomerulosclerosis
DOI:
https://doi.org/10.18203/2320-6012.ijrms20263552Keywords:
Focal segmental glomerulosclerosis , Nephrotic syndrome, Proteinuria, Renal Biopsy, Case seriesAbstract
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.
References
Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021;100(4S):S1-S276.
D'Agati VD, Kaskel FJ, Falk RJ. Focal segmental glomerulosclerosis. N Engl J Med. 2011;365(25):2398-411.
De Vriese AS, Wetzels JF, Glassock RJ, Sethi S, Fervenza FC. Therapeutic trials in adult FSGS: lessons learned and the road forward. Nat Rev Nephrol. 2021;17(9):619-30.
Rosenberg AZ, Kopp JB. Focal Segmental Glomerulosclerosis. Clin J Am Soc Nephrol. 2017;12(3):502-517.
D'Agati VD, Fogo AB, Bruijn JA, Jennette JC. Pathologic classification of focal segmental glomerulosclerosis: a working proposal. Am J Kidney Dis. 2004;43(2):368-82.
Sethi S, Glassock RJ, Fervenza FC. Focal segmental glomerulosclerosis: towards a better understanding for the practicing nephrologist. Nephrol Dial Transplant. 2015;30(3):375-84.
Gipson DS, Troost JP, Spino C, Attalla S, Tarnoff J, Massengill S, et al. Comparing kidney health outcomes in children, adolescents, and adults with focal segmental glomerulosclerosis. JAMA Netw Open. 2022;5(8):e2228701.
Cattran DC, Troyanov S, Wall CA, et al. Complete remission in focal segmental glomerulosclerosis predicts improved kidney survival. Clin J Am Soc Nephrol. 2017;12(5):683-691.
Fogo AB. Causes and pathogenesis of focal segmental glomerulosclerosis. Nat Rev Nephrol. 2015;11(2):76-87.
Reiser J, Altintas MM. Podocytes. F1000Res. 2016;5:F1000 Faculty Rev-114.
Korbet SM. Treatment of primary focal segmental glomerulosclerosis. Kidney Int. 2002;62(6):2301-10.
Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group. KDIGO clinical practice guideline for glomerulonephritis. Kidney Int Suppl. 2012;2(2):139-274.
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Copyright (c) 2026 Kazi Ammara, Aasem Khan, Malhar Lonikar, Tauseef Ahmed Abdul Gani

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