Citations

Polycystic kidney disease (PKD) is a genetic disorder characterized by progressive kidney cyst formation and eventual kidney failure. Emerging evidence suggests that plant-based diets may confer therapeutic benefits in patients with PKD by modulating mTOR and AMPK signaling, reducing oxidative stress and inflammation, while improving metabolic and cardiovascular profiles. These diets, which are low in animal protein and rich in antioxidants, may help lower uric acid levels and support blood pressure control, thereby potentially reducing fibrosis. However, careful planning is required to avoid nutritional deficiencies. Well-designed clinical trials are warranted to validate their role in ADPKD management.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disorder and a leading cause of kidney failure worldwide. Disease progression is driven by cyst expansion, tubular injury, and maladaptive metabolic and hemodynamic changes. Tolvaptan remains the only US Food and Drug Administration–approved disease-modifying therapy, however its tolerability and safety profile highlight the need for additional strategies. Sodium-glucose cotransporter 2 inhibitors (SGLT2is), initially developed for glycemic control, have demonstrated robust kidney-protective and cardioprotective effects across diverse patient populations, including those without diabetes. By lowering intraglomerular pressure, metabolic reprogramming, and attenuating hypoxic microenvironment and inflammation, SGLT2is target mechanisms that are relevant to ADPKD. However, concerns that SGLT2i may provoke osmotic diuresis and activate vasopressin led to systematic exclusion of patients with ADPKD from pivotal outcome trials. The 2025 Kidney Disease Improving Global Outcomes ADPKD guidelines explicitly advise against the use of SGLT2i for slowing kidney function decline in ADPKD. In this review, we examine mechanistic intersections between SGLT2i and ADPKD pathophysiology, summarize available preclinical and early clinical data, and discuss ongoing large trials designed to address safety and efficacy of SGLT2i in patients with ADPKD. We also highlight the importance of genetic heterogeneity, as most ongoing trials are enriched for rapid progressors carrying PKD1 or PKD2 pathogenic variants, while other genotypes such as DNAJB11 , ALG8 , and ALG9 , often associated with greater interstitial fibrosis, are underrepresented. Substratification of patients by genotype, risk of progression, and comorbidities will be essential to guide precision application of SGLT2i. Whether SGLT2i can ultimately be positioned as an adjunctive or standalone therapy for ADPKD remains unresolved.

Key Points BeWell360-PKD shows that integrating certified health and wellness coaches and augmenting patients' personal capacity as part of autosomal dominant polycystic kidney disease care is feasible and acceptable. Embedding BeWell360 coaches reduced care burden, improved caregiver self-efficacy, and enhanced care engagement by patients with autosomal dominant polycystic kidney disease and by their informal caregivers. This aligns with evolving care standards and guidelines promoting sustainable healthy lifestyle changes through tailored, integrative behavior-change centered interventions. Background Autosomal dominant polycystic kidney disease (ADPKD) imposes substantial care burden on patients and their informal caregivers (CGs), often contributing to nonadherence and impaired quality of life. Existing care models insufficiently address the behavioral and psychosocial dimensions of this burden. To evaluate the feasibility and effect of BeWell360-PKD , a person-centered intervention integrating Health and Wellness Coaching and capacity-workload support into ADPKD care, on care burden, adherence, self-efficacy, activation, and resilience. Methods In this 6-month, single-arm, mixed-methods pilot study, seven patient-CG dyads were enrolled from tertiary care polycystic kidney disease clinic. Board-certified Health and Wellness Coachings delivered individualized coaching within routine nephrology care, emphasizing capacity-building, goal setting, and care enactment support. Primary outcomes included changes in treatment burden (treatment burden questionnaire, ADPKD impact scale) and CG burden (burden scale for family CGs short version). Secondary outcomes included patient activation and resilience and CG self-efficacy. Semistructured interviews explored participant experiences. Results Patients experienced reduced treatment burden (treatment burden questionnaire mean change −9.3) and modest, domain-specific improvements in physical and fatigue-related ADPKD symptom burden. CG self-efficacy improved (+2.4), while CG burden increased (+14.8), and patient activation declined (−6.6). Qualitative themes reflected disease burden, emotional adaptation, and the perceived value of coaching in promoting behavior change and relational support. Conclusions BeWell360-PKD was feasible to implement and demonstrated early signals of benefit in reducing patient burden and improving CG self-efficacy. Increased CG burden and declining activation highlight the complexity of dyadic adaptation in ADPKD and the need for larger, controlled studies to refine and tailor coaching interventions.

Key Points Automated cyst segmentation in autosomal dominant polycystic kidney disease could transform care by enabling precise monitoring of cyst progression with minimal manual effort. This technology can facilitate more consistent assessments of cyst burden across patients. Translating these segmentation models from magnetic resonance imaging to computed tomography is crucial for providing comprehensive, modality-independent evaluations. Background Total kidney and liver volumes are key image-based biomarkers to predict the severity of kidney and liver phenotype in autosomal dominant polycystic kidney disease (ADPKD). However, magnetic resonance (MR) imaging-based advanced biomarkers like total cyst number and cyst parenchyma surface area have been shown to more accurately assess cyst burden and improve the prediction of disease progression. The main aim of this study was to extend the calculation of advanced biomarkers to other imaging modalities; thus, we propose a fully automated model to segment kidney and liver cysts in computed tomography (CT) images. Methods Abdominal CTs of patients with ADPKD were gathered retrospectively between 2001 and 2018. A three-dimensional deep learning method using the nnU-Net architecture was trained to learn cyst edges-cores and the noncystic kidney/liver parenchyma. Separate segmentation models were trained for kidney cysts in contrast-enhanced CTs and liver cysts in noncontrast CTs using an active learning approach. Two experienced research fellows manually generated the reference standard segmentation, which were reviewed by an expert radiologist for accuracy. Results Two-hundred CT scans from 148 patients (mean age, 51.2±14.1 years; 48% male) were used for model training (80%) and testing (20%). In the test set, both models showed good agreement with the reference standard segmentations, similar to the agreement between two independent human readers (model versus reader: TCN kidney/liver r =0.96/0.97 and CPSA kidney r =0.98, inter-reader: TCN kidney/liver r =0.96/0.98 and CPSA kidney r =0.99). Conclusions Our study demonstrates that automated models can segment kidney and liver cysts accurately in CT scans of patients with ADPKD.

Key Points This study demonstrates that peritoneal dialysis is a safe and feasible option for patients with autosomal dominant polycystic kidney disease, even with high cystic organ volumes. Larger organ volumes, including height-adjusted cumulative kidney and liver volume, were not associated with increased peritoneal dialysis–related complications. Background Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent genetic kidney disorder and the fourth leading cause of kidney failure. Peritoneal dialysis (PD), preferred for its home-based convenience and cost-effectiveness, is often underutilized in ADPKD because of concerns over enlarged kidneys and heightened risk of complications. Methods This retrospective cohort study used data from the Mayo Clinic Polycystic Kidney Disease Database to evaluate individuals with ADPKD undergoing PD. We analyzed demographics, clinical parameters, and PD-related parameters. Complications were correlated with kidney and liver volumes derived from pre-kidney failure imaging. Results A total of 155 individuals with ADPKD on PD were included, of whom 45.1% were male. The mean age at PD initiation was 54.3±12.8 years, and the mean body mass index was 28.0±7.0 kg/m 2 . The median duration of PD was 24.3 months (interquartile range [IQR], 10.7–43.1), with 21.9% transitioning to hemodialysis. The most common complications were abdominal hernias (30.3%) and peritonitis (23.9%), with a peritonitis rate of 0.11 episodes per patient-year. Imaging analyses performed on a subset of 50 patients showed a median height-adjusted total kidney liver volume of 2731.9 ml/m (IQR, 2102.5–3131.1) and a median height-adjusted total kidney volume of 1303.5 ml/m (IQR, 733.1–1829.4). Kaplan–Meier analysis demonstrated no differences in complications rates on the basis of height-adjusted total kidney liver volume or height-adjusted total kidney volume (above versus below median values) or body mass index categories. Multivariate Cox regression analysis revealed that higher height-adjusted cumulative organ volume was associated with a lower risk of PD-related complications (hazard ratio=0.56, P = 0.026). Conclusions PD is a safe and feasible treatment option for patients with ADPKD, with no increased risk of PD-related complications associated with larger height-adjusted cumulative organ volumes. Infectious complication rates in this cohort were within International Society of Peritoneal Dialysis guideline thresholds, further supporting the safety of PD in this population.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disorder. It is primarily caused by pathogenic variants in the PKD1 or PKD2 genes. This leads to the development of numerous kidney cysts, which can result in kidney enlargement and progression to kidney failure. Pain is a common symptom in ADPKD and can negatively affect quality of life (QOL). This pain is often due to the continuous growth of kidney and liver cysts or associated cystic complications. We present a case of a 29-year-old woman with ADPKD who experienced chronic, refractory right-sided flank pain that significantly affected her QOL. Her pain persisted despite taking daily multimodal analgesics and undergoing multiple invasive interventions. She had an unusual asymmetric disease with the right kidney accounting for only 24% of her kidney function. After exhausting all other pain control strategies, she underwent right nephrectomy and partial hepatectomy, which led to substantial improvement in pain and QOL. This review describes the causes, manifestations, and management strategies for abdominal and/or flank pain in ADPKD, including a practical stepwise algorithm to guide clinicians in managing pain and improve QOL of patients with ADPKD. Pain in ADPKD can either be acute or chronic and can lead to significant physical and psychologic distress. Effective pain management in ADPKD requires a multidisciplinary approach, incorporating both nonpharmacologic and pharmacologic interventions such as gabapentin or tolvaptan in select cases. Interventions considered in ADPKD pain control include cyst aspiration with sclerotherapy, celiac plexus blockade, spinal cord stimulation, renal denervation, or nephrectomy.