Raveendran R. et al., 2025: A Review of the Management of Nephrolithiasis in Autosomal Dominant Polycystic Kidney Disease.

Rakhul Raveendran, Vinodh Murali, Herman Fernando 
Cureus. 2025 Oct 15;17(10):e94642. doi: 10.7759/cureus.94642. FREE FULL TEXT

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is a chronic hereditary disorder and a major cause of end-stage renal disease (ESRD). Urolithiasis is a frequent complication in ADPKD and may contribute to the accelerated decline in renal function. The presence of complex calyceal anatomy, impaired renal function, and associated comorbidities makes stone management particularly challenging in this population. We conducted a narrative review of the literature to explore the underlying reasons for the high incidence of nephrolithiasis in ADPKD, as well as current approaches to management. A targeted literature search was performed in MEDLINE, EMBASE, Google Scholar, and Web of Science for articles published up to February 2025 using the keywords "ADPKD", "nephrolithiasis", "kidney stones," "urolithiasis," "management", and "treatment". Only English-language articles involving human subjects were considered. Original studies, meta-analyses, narrative, and systematic reviews relevant to nephrolithiasis in ADPKD were included. Conference abstracts, editorials, and non-peer-reviewed sources were excluded. Both medical and surgical treatment modalities were evaluated, with attention to reported success rates and complication profiles. Although this is a narrative review and no formal risk of bias assessment was performed, emphasis was placed on including high-quality studies and widely cited literature. Key findings were synthesized thematically to provide an overview of current understanding and management of nephrolithiasis in ADPKD. Small sample sizes and significant heterogeneity in study design, patient selection, and treatment protocols limit available evidence. While the general principles of stone management are similar to those in non-ADPKD patients, therapeutic decisions must be tailored to stone size, location, composition, renal function, and anatomical considerations. Based on the literature, we propose a treatment algorithm to support clinical decision-making. Standardization of management strategies in ADPKD remains an unmet need. Larger, well-designed studies are required to establish evidence-based guidelines for optimal care.

Comment Peter Alken

A comprehensive review of this entity; good information for those dealing with such patients. However, I have some opposing views and completely disagree with their “Algorithm for managing nephrolithiasis in ADPKD patients”
These principally few patients will or should always be under care of doctors devoted to their problem and special conditions.

“Diagnostics: “
“Ultrasonography: Ultrasound is a useful initial screening test, with a specificity of about 90%, but its
sensitivity is as low as 24% [17]. It cannot reliably differentiate stones from calcifications and is highly
operator-dependent. Multiple cysts often obscure visualization of calculi and distort acoustic windows [16]. While it may be helpful in experienced hands, variability and confounding factors such as cyst calcification reduce its reliability.
Computed tomography (CT) scan: CT offers the highest sensitivity and specificity for stone detection, both in the general population [16] and in ADPKD.”
1. It is correct that CT better detects stones than Sonographie. However, he/she who takes care should have a continuously updated road map of the individual kidneys from each sonographic examination documenting the whole kidney and eventual changes. CT might be added whenever in doubt. This should save radiation. The argument that Sonography might be difficult is only valid for the unexperienced examiner.

“Therapy”
“… the common metabolic abnormalities observed in non-PKD stone formers, such as
hypercalciuria, hyperoxaluria, and hyperuricosuria, are uncommon in this group [5,11]. The metabolic profile in ADPKD explains the typical stone types found: uric acid stones (primarily due to low urinary pH rather than hyperuricemia or increased uric acid excretion) and calcium oxalate monohydrate stones (linked to hypocitraturia).”

“Medical management, prevention, and follow-up of nephrolithiasis in ADPKD
Since most stones in ADPKD are composed of uric acid, and the dominant metabolic abnormalities include low urinary pH and hypocitraturia, may be considered. Dissolution therapy can be effective for small, asymptomatic stones, though the precise threshold stone size for intervention is not clearly defined.
In modern practice, potassium citrate is best used both as a preventive measure and as a treatment for small, non-obstructing uric acid calculi in cystic kidneys with preserved renal function. “
I have good experience with oral dissolution of even large uric acid staghorn stones (1)

Their “Algorithm for managing nephrolithiasis in ADPKD patients” includes only SWL, URS, and PNL or expectant treatment. The first step in every patient should be a trial of oral chemolysis.

1 Schärfe T, Alken P, Klose KJ, Hohenfellner R. Slightly radiopaque uric acid calculi: impact upon therapeutic considerations? Urol Int. 1989;44(5):292-7. doi: 10.1159/000281525. PMID: 2800064.

Peter Alken