Purpose: To investigate the prevalence of urolithiasis in cystic fibrosis (CF) and to summarize the available clinical features within this unique population. Methods: Studies reporting the prevalence of urolithiasis in CF patients were identified by a systematic search of the literature from inception to July 31, 2020 on three databases: Ovid Medline, Ovid Embase, and Web of Science. Data were extracted on a predetermined standardized form by two independent authors. Results: A total of 596 publications were retrieved and screened, 15 of which met the eligibility criteria. The publications were published between 1993 and 2019 and were all observational in design. There was a total of 2982 patients with CF included in this review. The overall prevalence of stone formation in the CF population was 4.6% (137/2982). The mean age of diagnosis was 25.1 ± 9.6 and ranged from 0.25 to 47. Ultrasound was the most common imaging modality for kidney stone diagnosis. There was no apparent sex difference, with a female to male ratio of 1:1. Surgical intervention was required in 37.8% (34/90) of cases. Stone recurrence was reported in 42.9% (33/77) of stone formers. Conclusions: This review provides the most recent update for the prevalence of urolithiasis in CF patients and summarizes the available clinical data. Our findings suggest that patients with CF could be at risk for developing stones at a younger age and require interventional management strategies at higher rates compared with the general population. Given the heterogeneity of the literature for urolithiasis in CF, larger population-based studies reporting the epidemiology, clinical features, and management strategies are required to further our understanding of urolithiasis in CF.
Keywords: cystic fibrosis; epidemiology; nephrolithiasis; prevalence; renal calculi.