Jafry NH, Sarwar S, Waqar T, Mubarak M. Clinical course and outcome of adult patients with primary focal segmental glomerulosclerosis with kidney function loss on presentation. World J Nephrol 2024; 13(4): 98932 [DOI: 10.5527/wjn.v13.i4.98932]
Corresponding Author of This Article
Muhammed Mubarak, MD, Full Professor, Department of Histopathology, Sindh Institute of Urology and Transplantation, Chand Bibi Road, Karachi 74200, Sindh, Pakistan. drmubaraksiut@yahoo.com
Research Domain of This Article
Urology & Nephrology
Article-Type of This Article
Observational Study
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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: http://creativecommons.org/licenses/by-nc/4.0/
World J Nephrol. Dec 25, 2024; 13(4): 98932 Published online Dec 25, 2024. doi: 10.5527/wjn.v13.i4.98932
Clinical course and outcome of adult patients with primary focal segmental glomerulosclerosis with kidney function loss on presentation
Nazarul Hassan Jafry, Sarfraz Sarwar, Tajammul Waqar, Muhammed Mubarak
Nazarul Hassan Jafry, Sarfraz Sarwar, Tajammul Waqar, Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi 74200, Sindh, Pakistan
Muhammed Mubarak, Department of Histopathology, Sindh Institute of Urology and Transplantation, Karachi 74200, Sindh, Pakistan
Author contributions: Jafry NH and Sarwar S conceived and designed the study; Jafry NH, Sarwar S, Waqar T, and Mubarak M contributed significantly and equally to the preparation of the manuscript, participated in primary and final drafting, and performed the research; Mubarak M critically reviewed and finalized the draft; All of the authors read and approved the final version of the manuscript to be published.
Institutional review board statement: The study was reviewed and approved by the Sindh Institute of Urology and Transplantation (Pakistan).
Informed consent statement: All study participants or their legal guardians provided informed written consent prior to study enrollment.
Conflict-of-interest statement: There are no conflicts of interest to report.
Data sharing statement: The dataset and related documents are available from the first author at drjafry12@gmail.com.
STROBE statement: The authors have read the STROBE Statement-checklist of items, and the manuscript was prepared and revised according to the STROBE Statement-checklist of items.
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, Full Professor, Department of Histopathology, Sindh Institute of Urology and Transplantation, Chand Bibi Road, Karachi 74200, Sindh, Pakistan. drmubaraksiut@yahoo.com
Received: July 9, 2024 Revised: September 28, 2024 Accepted: October 21, 2024 Published online: December 25, 2024 Processing time: 120 Days and 20.8 Hours
Abstract
BACKGROUND
Kidney function loss or renal insufficiency indicated by elevated creatinine levels and/or an estimated glomerular filtration rate (eGFR) < 60 mL/minute/1.73 m² at presentation in patients with primary focal segmental glomerulosclerosis (FSGS) is commonly seen as a poor prognostic marker for kidney survival. However, a pre>vious study from our center suggested this may be due to hemodynamic factors.
AIM
To observe the clinical and biochemical parameters, treatment response, kidney survival, and overall outcomes of adult patients with primary FSGS presenting with kidney function insufficiency.
METHODS
This retrospective observational study was conducted at the Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan, from January 1995 to December 2017. During this period, 401 biopsy-proven primary FSGS patients were identified, of which 98 (24.4%) presented with kidney function loss or renal insufficiency defined as eGFR < 60 mL/minute/1.73 m² at presentation and were studied in detail.
RESULTS
Among the 98 patients with renal function loss on presentation, the mean age was 30.9 years ± 13.6 years with a male-to-female ratio of 2.5:1. The mean serum creatinine level was 2.2 mg/dL ± 1.3 mg/dL and mean eGFR 37.1 mL/minute/1.73 m2 ± 12.8 mL/minute/1.73 m2. The mean 24-hour urinary protein excretion was 5.9 g/day ± 4.0 g/day, and the mean serum albumin was 2.1 g/dL ± 1.0 g/dL (median: 1.5 g/dL). The mean systolic blood pressure (BP) was 132.7 mmHg ± 19.8 mmHg, and the mean diastolic BP was 87.4 mmHg ± 12.7 mmHg. Steroid treatment was given to 81 (82.6%) of 98 patients for an average duration of 19.9 weeks ± 14.4 weeks, with a mean total steroid dose of 4.4 g ± 1.5 g. Treatment response showed that 20 (24.6%) patients achieved complete remission, 9 (11.1%) achieved partial remission, and 52 (64.1%) did not respond. The baseline eGFR was significantly lower in the non-responsive group (P = 0.006). The distribution of FSGS variants was also significantly different among steroid-responsive and non-responsive groups (P = 0.012).
CONCLUSION
Renal function loss in FSGS patients at presentation does not necessarily indicate irreversible kidney function loss and a significant number of patients respond to appropriate treatment of the underlying disease.
Core Tip: Focal segmental glomerulosclerosis is a common glomerular disease that typically presents with nephrotic syndrome. It often presents with kidney function loss and generally signifies a poor prognosis. We previously showed that kidney function loss at presentation is not necessarily associated with poor outcomes. This study further corroborates these findings and suggests that treatment of such patients can improve outcomes.
