Kidney outcomes in children with primary focal segmental glomerulosclerosis from a low- and middle- income country

Kolluri Priyanka; Bobbity Deepthi; Sudarsan Krishnasamy; Rajesh Nachiappa Ganesh; Madhileti Sravani; Sriram Krishnamurthy

doi:10.1007/s00467-024-06382-w

Kidney outcomes in children with primary focal segmental glomerulosclerosis from a low- and middle- income country

Pediatr Nephrol. 2024 Apr 23. doi: 10.1007/s00467-024-06382-w. Online ahead of print.

Authors

Kolluri Priyanka¹, Bobbity Deepthi², Sudarsan Krishnasamy¹, Rajesh Nachiappa Ganesh³, Madhileti Sravani¹, Sriram Krishnamurthy¹

Affiliations

¹ Pediatric Nephrology Services, Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, 605006, India.
² Pediatric Nephrology Services, Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, 605006, India. deepu.reddy222@gmail.com.
³ Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, 605006, India.

PMID: 38652137
DOI: 10.1007/s00467-024-06382-w

Abstract

Background: Limited data exists regarding the clinical course and outcomes of children with primary focal segmental glomerulosclerosis (FSGS) from low- and middle- income countries.

Methods: Children aged 1-18 years with biopsy-proven primary FSGS followed from January 2010-June 2023 in a tertiary-care center were enrolled and their clinical profile, histological characteristics, kidney outcomes, and predictors of adverse outcomes were determined.

Results: Over 13 years, 73 (54.8% boys) children with median (IQR) age at FSGS diagnosis 6.7 (3,10) years were recruited and followed up for median 4 (2.5,8) years. FSGS-not otherwise specified (NOS) was the most common histological subtype, in 64 (87.6%) children, followed by collapsing variant in 5 (6.8%) children. At last follow-up, 43 (58.9%), 2 (2.7%) and 28 (38.3%) children were in complete remission (CR), partial remission (PR), and no remission (NR) respectively. Calcineurin inhibitors led to CR or PR in 39 (62%) children. Overall, 21 (28.7%) children progressed to chronic kidney disease (CKD) stage 2-5 (19 from NR vs. 2 from PR group; p = 0.03); with 41% of those NR at 12 months progressing to CKD 4-5 by last follow-up. On multivariable analysis, collapsing variant [adjusted HR 2.5 (95%CI 1.5, 4.17), p = 0.001] and segmental sclerosis > 25% [aHR 9.9 (95%CI 2.2, 45.2), p = 0.003] predicted kidney disease progression.

Conclusions: In children with FSGS, response to immunosuppression predicts kidney survival as evidenced by nil to lower progression to CKD 2-5 by median follow-up of 4 (2.5,8) years in children with CR and PR, compared to those with no remission at 12 months from diagnosis. Segmental sclerosis > 25% and collapsing variant predicted progression to advanced CKD.

Keywords: CKD progression; Collapsing FSGS; Focal segmental glomerulosclerosis; Kidney survival; Steroid resistant nephrotic syndrome.