Retrospective Study
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Nephrol. Mar 25, 2024; 13(1): 88028
Published online Mar 25, 2024. doi: 10.5527/wjn.v13.i1.88028
Clinicopathological features and medium-term outcomes of histologic variants of primary focal segmental glomerulosclerosis in adults: A retrospective study
Nazarul Hassan Jafry, Shumaila Manan, Rahma Rashid, Muhammed Mubarak
Nazarul Hassan Jafry, Shumaila Manan, Department of Nephrology, Sindh Institute of Urology and Transplantation, Sindh, Karachi 74200, Pakistan
Rahma Rashid, Muhammed Mubarak, Department of Pathology, Sindh Institute of Urology and Transplantation, Sindh, Karachi 74200, Pakistan
Author contributions: Jafry NH and Mubarak M conceived and designed the study; Jafry NH, Manan S, Rashid R, and Mubarak M performed the research; all four participated in primary and final drafting; all read and approved the final manuscript; all four authors contributed significantly and equally to preparation of the manuscript.
Institutional review board statement: This study was reviewed and approved by the Ethics Committee of Sindh Institute of Urology and Transplantation.
Informed consent statement: All study participants or their legal guardian provided informed written consent regarding personal and medical data collection prior to study enrolment.
Conflict-of-interest statement: We have no financial relationships to disclose.
Data sharing statement: Data are available from the first author and can be shared on reasonable request.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Muhammed Mubarak, MD, Professor, Department of Pathology, Sindh Institute of Urology and Transplantation, Chand Bibi Road, DFMC, Sindh, Karachi 74200, Pakistan. drmubaraksiut@yahoo.com
Received: September 6, 2023
Peer-review started: September 6, 2023
First decision: November 21, 2023
Revised: November 30, 2023
Accepted: January 11, 2024
Article in press: January 11, 2024
Published online: March 25, 2024
Processing time: 197 Days and 14.7 Hours
Abstract
BACKGROUND

The Columbia classification identified five histological variants of focal segmental glomerulosclerosis (FSGS). The prognostic significance of these variants remains controversial.

AIM

To evaluate the relative frequency, clinicopathologic characteristics, and medium-term outcomes of FSGS variants at a single center in Pakistan.

METHODS

This retrospective study was conducted at the Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan on all consecutive adults (≥ 16 years) with biopsy-proven primary FSGS from January 1995 to December 2017. Studied subjects were treated with steroids as a first-line therapy. The response rates, doubling of serum creatinine, and kidney failure (KF) with replacement therapy were compared between histological variants using ANOVA or Kruskal Wallis, and Chi-square tests as appropriate. Data were analyzed by SPSS version 22.0. P-value ≤ 0.05 was considered significant.

RESULTS

A total of 401 patients were diagnosed with primary FSGS during the study period. Among these, 352 (87.7%) had a designated histological variant. The not otherwise specified (NOS) variant was the commonest, being found in 185 (53.9%) patients, followed by the tip variant in 100 (29.1%) patients. Collapsing (COL), cellular (CEL), and perihilar (PHI) variants were seen in 58 (16.9%), 6 (1.5%), and 3 (0.7%) patients, respectively. CEL and PHI variants were excluded from further analysis due to small patient numbers. The mean follow-up period was 36.5 ± 29.2 months. Regarding response rates of variants, patients with TIP lesions achieved remission more frequently (59.5%) than patients with NOS (41.8%) and COL (24.52%) variants (P < 0.001). The hazard ratio of complete response among patients with the COL variant was 0.163 [95% confidence interval (CI): 0.039-0.67] as compared to patients with NOS. The TIP variant showed a hazard ratio of 2.5 (95%CI: 1.61-3.89) for complete remission compared to the NOS variant. Overall, progressive KF was observed more frequently in patients with the COL variant, 43.4% (P < 0.001). Among these, 24.53% of patients required kidney replacement therapy (P < 0.001). The hazard ratio of doubling of serum creatinine among patients with the COL variant was 14.57 (95%CI: 1.87-113.49) as compared to patients with the TIP variant.

CONCLUSION

In conclusion, histological variants of FSGS are predictive of response to treatment with immunosuppressants and progressive KF in adults in our setup.

Keywords: Adults; Columbia classification; Focal segmental glomerulosclerosis; Histological variants; Kidney failure; Kidney failure with replacement therapy

Core Tip: Focal segmental glomerulosclerosis (FSGS) is one of the most common glomerular diseases and a leading cause of kidney failure. FSGS is a heterogeneous disorder with many causes, varying pathogenesis and clinical courses. Columbia classification identified five histological variants of FSGS. The prognostic significance of these has remained controversial. Early studies found a good correlation of the variants with clinical presentation, treatment responses, and final outcomes. However, a more recent Japanese study found no prognostic value of the variants. The present study aimed to determine the clinical significance of these variants in a large sample of the Pakistani adult FSGS population.