Autosomal Dominant Polycystic Kidney Disease: Core Curriculum 2025

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of kidney failure. Scientific advances have improved the approach to diagnosis, prognosis, and management of renal and extrarenal manifestations. The combination of total kidney volume, kidney function, and the genetic mutation (if known), predicts risk for progression to kidney failure, thereby identifying patients in whom disease modifying therapy is recommended. Currently there is one therapy approved by the US Food and Drug Administration (FDA) for slowing ADPKD progression, the V2 receptor antagonist, tolvaptan. Other therapies are under active investigation for ADPKD. This Core Curriculum discusses diagnosis and management of the renal and extrarenal manifestations seen in ADPKD including acute and chronic pain and cyst infection and polycystic liver disease and intracranial aneurysm. Management of hypertension and women’s health and pregnancy management in ADPKD are covered. This review was aligned with the findings of the recently published Kidney Disease Improving Global Outcomes (KDIGO) clinical practice guideline for the Evaluation, Management, and Treatment of ADPKD.