KDIGO 2025 clinical practice guideline for the evaluation, management, and treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD)

The Kidney Disease: Improving Global Outcomes (KDIGO) Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) represents the first KDIGO guideline on this subject. The scope includes topics such as nomenclature, diagnosis, prognosis, and prevalence; kidney manifestations; chronic kidney disease management and progression, kidney failure, and kidney replacement therapy; therapies to delay progression of kidney disease; polycystic liver disease; intracranial aneurysms and other extrarenal manifestations; lifestyle and psychosocial aspects; pregnancy and reproductive issues; pediatric issues; and approaches to the management of people with ADPKD. The guideline has been developed with patient partners, healthcare providers, and researchers around the world, using robust methodology. The goal of the guideline is to generate a useful resource for healthcare providers and patients by providing actionable recommendations based on a rigorous, formal, systematic literature review. Another aim is to propose research recommendations for areas in which gaps in knowledge are present. The guideline targets a broad audience of healthcare providers treating ADPKD, while taking into account implications for policy and payment. The development of this guideline followed an explicit process of evidence review and appraisal. Treatment approaches and guideline recommendations are based on systematic reviews of relevant studies, appraisal of the certainty of the evidence, and the strength of recommendations following the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. The guideline also provides useful infographics and practice points that serve to direct clinical care or activities for which a systematic review was not conducted. Limitations of the evidence are discussed, and areas for future research are presented.