Predicting the Risk of Progression in Indian ADPKD Cohort using PROPKD Score - A Single-Center Retrospective Study

Background: With the variable genotype-phenotype expression of autosomal dominant polycystic kidney disease (ADPKD) and availability of novel targeted therapies, it is important to find predictors for rapid progression. The PROPKD score, consisting of genetic and clinical parameters like sex, hypertension, and urological events, is a useful tool in predicting the risk of progression. This study was aimed to determine the risk of ADPKD progression in Indian patients using the PROPKD score. Materials and Methods: A retrospective study was done from 2006 to 2021. ADPKD patients with ESRD were included in the study. Scoring was done as per the PROPKD score as follows: male sex: 1, onset of hypertension before 35 years: 2, first urological event before 35 years: 2, PKD1 truncating mutation: 4, PKD1 non-truncating mutation: 2, and PKD2 mutation: 0. Two types of risk classifications were done as follows: (a) considering the clinical variables in all 73 patients (male sex, onset of hypertension before 35 years, and first urological event before 35 years), they were classified into three risk groups: low-risk group (0-1), intermediate-risk group (2-3), and high-risk group (4-5) and (b) considering the clinical variables and type of mutation in 39 patients, they were classified into three risk groups: low-risk group (0-3), intermediate-risk group (4-6), and high-risk group (7-9). Results: Total number of patients included was 73, with the median age at ESRD being 54 years. High-risk group of clinical variables with hazard ratio (HR) of 4.570 (2.302-9.075, P < 0.001) and high-risk group of the PROPKD score with HR of 6.594 (1.868-23.284, P = 0.003) were associated with early ESRD. High-risk groups of both classifications were associated with early ESRD. Conclusion: High-risk groups based on the PROPKD scoring and clinical variables were associated with early progression to ESRD.