Adult minimal change disease: Clinicopathologic characteristics, treatment response and outcome at a single center in Pakistan

Abstract

BACKGROUND

Minimal change disease (MCD) is a significant cause of idiopathic nephrotic syndrome (INS) in adults, representing approximately 10%-15% of INS cases. The data is scanty on clinicopathological features, treatment responses, and long-term outcomes of MCD in adults.

AIM

To determine the clinicopathologic characteristics, treatment responses, and medium-term outcomes of adult patients with MCD in Pakistan.

METHODS

This retrospective cohort study included all adult patients with biopsy-proven MCD treated at the adult nephrology clinic, Sindh institute of urology and transplantation, between January 2010 and December 2020. The data was retrieved from the original renal biopsy request forms in the histopathology archives and the case files. Data on demographics, clinical presentation, laboratory findings, treatment regimens, and outcomes were collected and analyzed. Complete remission (CR), partial remission (PR), relapse, and steroid resistance were defined according to standard criteria. Statistical analyses were performed using statistical product and service solutions, Version 22.

RESULTS

The study cohort included 23 adults [15 (65.2% males), mean age 26.34 ± 10.28 years]. Hypertension was found in 7 (30.4%) and microscopic hematuria in 10 (43.4%) of participants. Laboratory findings revealed a mean serum creatinine of 1.03 ± 1.00 mg/dL, mean serum albumin of 1.94 ± 0.90 g/dL and mean 24-hour urinary proteins of 4.53 ± 2.43 g. The mean follow-up time was 38.09 ± 22.3 months. Treatment with steroids was effective in 16/18 (88.8%) of patients, with 10/16 (62.5%) achieving CR and 6/16 (37.5%) achieving PR. Two patients were resistant to steroids and required second-line immunosuppressive therapy. Relapse occurred in 4/20 (19.04%) of patients, with a mean time to first relapse of 6.5 ± 3.31 months. At the last follow-up, 18/20 (85.7%) of patients were in remission, and 16/20 (76.1%) maintained normal renal function. No patients progressed to end-stage renal disease or died.

CONCLUSION

MCD in adults shows a favorable response to steroid therapy, with a majority achieving remission. However, relapses are common, necessitating second-line immunosuppressive treatments in some cases. The study highlights the need for standardized treatment guidelines for adult MCD to optimize outcomes.

Keywords: Minimal change disease; Nephrotic syndrome; Adults; Steroid therapy; Immunosuppression; Clinical outcomes; Pakistan

Core Tip: Minimal change disease (MCD) constitutes the third most common cause of nephrotic syndrome in adults. The information is scanty on the clinicopathological features, treatment responses, and long-term outcomes of MCD in adults, particularly from developing countries. This study revealed that MCD in adults shows a favorable response to steroid therapy, with a majority achieving remission. However, relapses are common, necessitating second-line immunosuppressive treatments in some cases. The study highlights the need for standardized treatment guidelines for adult MCD to optimize outcomes.