Clinical Characteristics and Disease Progression among Nondiabetic Patients with Apolipoprotein L1-Mediated Kidney Disease and Patients with Other CKD.

key pointsPatients with nondiabetic apolipoprotein L1-mediated kidney disease progressed to dialysis and transplant faster than patients with other forms of nondiabetic CKD. Although patients with apolipoprotein L1-mediated kidney disease were younger with fewer comorbidities, kidney decline was accelerated underscoring the aggressive nature of apolipoprotein L1-mediated kidney disease. These findings highlight the need of targeted therapies for apolipoprotein L1-mediated kidney disease and earlier identification through genetic testing to improve patient outcomes.

backgroundApolipoprotein L1-mediated kidney disease (AMKD) is an aggressive form of CKD. This study described characteristics of nondiabetic patients with AMKD and compared their disease progression with nondiabetic patients with other forms of CKD.

methodsThis retrospective study used clinical and biosample data from NashBio (NashBio contains electronic health records generated during clinical encounters at Vanderbilt University Medical Center). Nondiabetic patients with ≥1 CKD diagnosis code who were 10 years or older at index (date of first CKD diagnosis code in patient's medical record) were included. The AMKD and other forms of CKD cohorts were defined on the basis of the presence of apolipoprotein L1 risk alleles, and then their clinical and demographic characteristics were described. To assess progression patients were matched 1:1 on sex, presence of hypertension, and CKD stage at index and were followed until the last observation in the data. Progression outcomes including time from index to dialysis, kidney transplant, and diagnosis of CKD requiring KRT were assessed using Kaplan-Meier analysis, with log-rank tests used to compare matched cohorts.

resultsThe study included 645 patients with AMKD and 6749 patients with other forms of CKD. Patients with AMKD were significantly younger than patients with other CKD (median 44.0 versus 61.0 years; P < 0.001) and had significantly less comorbidity burden such as cardiovascular diseases (16.9% versus 36.6% P < 0.001). In addition, patients with AMKD had higher rates of CKD requiring KRT at index (59% versus 24%, P < 0.001). After 1:1 matching, patients with AMKD reached dialysis and kidney transplant significantly faster than patients with other CKD (both P < 0.001). Among patients who did not require KRT at index ( n =264 per cohort), patients with AMKD progressed to CKD requiring KRT significantly faster than patients with other CKD ( P < 0.01).

conclusionsNondiabetic patients with AMKD experienced more rapid disease progression than patients with other forms of CKD, despite being younger and having lower comorbidity burden.