CLINICAL OUTCOMES OF FSGS WITH NEPHROTIC SYNDROME AT 1-YEAR: FINDINGS FROM THE ITANGIBLE REGISTRY

Introduction:

Focal Segmental Glomerulosclerosis (FSGS) is a common cause of nephrotic syndrome in adults, necessitating long-term monitoring to evaluate disease progression and the effectiveness of treatment. Steroid responsiveness remains the most significant predictor of long-term outcomes. The clinical manifestation of FSGS varies globally. There is no data on the natural history of FSGS in South Asia. We present the 1-year outcomes of patients with FSGS enrolled in a pan-India glomerulonephritis (GN) registry.

Methods:

I-TANGIBLE is an ICMR funded collaborative registry of adult patients with biopsy-proven GN representing 13 large centres in India. A total of 399 FSGS cases were recruited between June 2022 and June 2024. 1-year follow-up data were available for 67 patients with FSGS, all but one patients had nephrotic syndrome. Baseline data comprised demographic details, clinical presentation, laboratory findings, and histopathological characteristics. Treatment details were systematically documented. Follow-up evaluations at 3, 6, and 12 months included assessments of proteinuria (24-hour urine protein excretion), serum creatinine, estimated glomerular filtration rate (eGFR), blood pressure, and serum albumin levels. Remission was defined according to KDIGO guidelines.

Results:

The mean age was 39.35 ± 16.38 years, comprising 32 males and 35 females. The BMI was 22.45 ± 4.13 kg/m². At presentation, 09 (13.43%) patients had diabetes mellitus, and 34 (50.74%) had hypertension. The proteinuria, serum albumin, and serum creatinine at baseline were 6.00 (4.20,8.10) g/day, 2.33± 0.76(1.5,3.2) g/dL, and 1.10 (0.80,1.67) mg/dL, respectively. The average duration of symptoms was 4.03±0.37 months. Sixty-three (94.02%) patients were treated with oral prednisolone. Fifty-eight patients received ACEi/ARBs. Others were treated with ACEi/ARB. At the 1-year follow-up, the median proteinuria, serum albumin, and serum creatinine were 0.52 (0.27,1.65) g/day, 3.74± 0.87 g/dL, and 0.9 (0.7,1.1) mg/dL, respectively. At the end of 12 months, 54 (87.09%) patients achieved remission (18 complete remission and 36 partial remission). One patient progressed to end stage kidney disease. Six patients received rituximab for frequently relapsing/steroid dependent nephrotic syndrome. One patient received tacrolimus therapy as steroid-sparing therapy due to adverse effect.

Conclusions:

This is the first study documenting the natural course and short-term outcomes of adult patients with FSGS and nephrotic syndrome in India. Over 80% of patients with FSGS and nephrotic syndrome achieved clinical remission after treatment with RAAS inhibitors and steroids. Continued follow-up of the subjects will provide valuable data on long-term outcomes.

I have no potential conflict of interest to disclose.

I did not use generative AI and AI-assisted technologies in the writing process.