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.
Renal failure. 2025 Nov 09 [Epub]
Abdul Hamid Borghol, Masara Azooz, Marie Therese Bou Antoun, Levon Souvalian, Besher Shami, Jonathan Mina, Fadi George Munairdjy Debeh, Ahmad Ghanem, Hashem Sandouk, Vineetha Rangarajan, Dana Hanna, Christopher D Naranjo, Yelena Drexler, Sayna Norouzi, Anahita Noruzi, Mahdi Salih, Dane E Klett, Ivan E Porter, Neera K Dahl, Fouad T Chebib
Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Jacksonville, FL, USA., School of Osteopathic Medicine in Arizona (ATSU-SOMA), A.T. Still University, Mesa, AZ, USA., Department of Nephrology and Hypertension, Cleveland Clinic Foundation, Cleveland, OH, USA., Department of Medicine, Katz Family Division of Nephrology and Hypertension, University of Miami Miller School of Medicine, Miami, FL, USA., Department of Nephrology, Loma Linda University, Loma Linda, CA, USA., Department of Internal Medicine, Division of Nephrology and Transplantation, Erasmus MC Rotterdam, Rotterdam, The Netherlands., Department of Urology, Mayo Clinic, Jacksonville, FL, USA., Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA.