Kidney stones (nephrolithiasis/urolithiasis) are a clinically significant yet underrecognized complication of autosomal dominant polycystic kidney disease (ADPKD), with reported prevalence ranging from 3% to 59% due to differences in diagnostic criteria and study design. Patients with ADPKD are predisposed to uric acid and calcium oxalate stones, driven by metabolic abnormalities, such as low urine pH, hypocitraturia, hyperuricosuria, as well as structural factors including cyst-induced distortion of the collecting system and impaired urinary drainage. These anatomical changes complicate both diagnosis and intervention, posing challenges, such as reduced stone-free rates, longer operative times, increased need for repeat procedures, and higher complication risk. While non-contrast computed tomography (CT) remains the diagnostic gold standard, low-dose CT is preferred to minimize cumulative radiation exposure. Management generally aligns with that of the broader population and includes aggressive hydration, correction of metabolic derangements, dietary modification, and individualized urologic intervention. Tolvaptan, a vasopressin V2 receptor antagonist, may have additional benefit by increasing urine volume, reducing supersaturation, and potentially mitigating stone risk. This review integrates nephrology, urology, and imaging perspectives to summarize the current understanding of nephrolithiasis in ADPKD, including its pathophysiology, clinical manifestations, diagnostic challenges, and management. We propose an ADPKD-specific diagnostic and management algorithm to optimize prevention, evaluation, and treatment of stone disease in this complex population.
Keywords: ADPKD; nephrolithiasis; stone management algorithm; tolvaptan; uric acid stones; urolithiasis.