KDIGO 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD): executive summary
The KDIGO 2025 guideline is the first KDIGO clinical practice guideline dedicated to ADPKD, providing comprehensive GRADE-based recommendations across diagnosis, prognosis, kidney and extrarenal manifestations, and disease-modifying therapy.
Background
Autosomal dominant polycystic kidney disease is the most common inherited kidney disorder and a leading cause of kidney failure, with a heterogeneous course ranging from mild to rapidly progressive disease. The approval of the vasopressin V2-receptor antagonist tolvaptan as the first targeted, disease-modifying therapy created a need for structured, evidence-based guidance across the full spectrum of ADPKD care.
Key recommendations
The guideline was developed with patient partners, clinicians and researchers worldwide using a formal systematic literature review, with recommendation strength graded by the GRADE approach and practice points provided where systematic review was not feasible. Its scope spans nomenclature, diagnosis, prognosis and prevalence; kidney manifestations; CKD management and progression, kidney failure and kidney replacement therapy; therapies to delay progression; polycystic liver disease; intracranial aneurysms and other extrarenal manifestations; lifestyle and psychosocial aspects; pregnancy and reproductive issues; and pediatric issues.
Clinical implications
As the first KDIGO guideline on a genetic kidney disease, it offers actionable recommendations for a broad audience of healthcare providers and people living with ADPKD, emphasizes interdisciplinary management of systemic manifestations, and outlines research recommendations to address remaining gaps in knowledge alongside implications for policy and payment.
Category
KDIGO
Source
Kidney International
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