Kidney Week Educational Symposia-Autosomal Dominant Polycystic Kidney Disease: Risk Assessment and Management

Video Transcription

Video Summary

The session reviews risk assessment and management of autosomal dominant polycystic kidney disease (ADPKD), a common inherited cause of kidney failure due to progressive cyst growth. ADPKD is usually caused by PKD1 (more severe) or PKD2 (milder); other genes (e.g., GANAB) can cause milder disease with liver involvement. Diagnosis is primarily imaging-based: ultrasound for screening, and CT/MRI for sensitivity and prognosis. Total kidney volume (TKV) is central for risk stratification; the Mayo Imaging Classification (classes 1A–1E for typical, bilaterally distributed disease) estimates kidney growth rate and predicts faster GFR decline, with classes 1C–1E indicating higher risk.<br /><br />Speakers emphasize distinguishing “risk” of rapid progression (early, based on TKV/Mayo class) from “evidence” (later, based on measured TKV growth >5%/year or eGFR decline >2.5 mL/min/1.73m²/year). Genetics and PRO-PKD scoring add information but have limited individual predictive power; emerging biomarkers (urinary MCP-1, β2-microglobulin, and blood copeptin) may improve prediction but are not yet routine.<br /><br />Management includes optimized supportive care: blood pressure control (ACE/ARB preferred; intensive targets for younger patients with preserved GFR), sodium restriction, high water intake to suppress vasopressin when safe, weight control, and CKD risk reduction. Tolvaptan slows TKV growth and eGFR decline in eligible rapid progressors but requires REMS liver-function monitoring and counseling on aquaresis. Extrarenal care includes screening high-risk patients for intracranial aneurysms and addressing symptomatic polycystic liver disease.

Asset Subtitle

Fouad Chebib, Ron Gansevoort, Ronald Perrone
Support is provided by an educational grant from Otsuka America Pharmaceutical, Inc.

Keywords

autosomal dominant polycystic kidney disease (ADPKD)

PKD1 and PKD2 genetics

imaging diagnosis (ultrasound, CT, MRI)

total kidney volume (TKV)

Mayo Imaging Classification 1A–1E

rapid progression risk stratification

eGFR decline and TKV growth rate

tolvaptan therapy and REMS liver monitoring

intracranial aneurysm screening and polycystic liver disease