Synopsis
Diagnose ADPKD accurately, manage renal and extrarenal complications, assess progression, and use tolvaptan through current specialist safeguards.
- ADPKD is a systemic inherited disorder causing progressive bilateral kidney cysts, hypertension, pain, haematuria, infection, stones and variable kidney failure.
- Diagnosis uses age- and family-history-sensitive imaging criteria or genomic testing; simple cysts become common with age and are not equivalent to ADPKD.
- Ask about intracranial aneurysm or subarachnoid haemorrhage, sudden death, kidney failure, liver cyst burden and relatives at potential reproductive risk.
Key red flags
Fever, inflammatory markers and focal renal or hepatic tenderness may occur even when routine urine culture is negative.
Investigation priorities
Assess bilateral cyst distribution, kidney size and alternative structural disease.
Management branches
Bilateral renal cysts or an affected relative raises possible ADPKD.
- Document family history, age, kidney function, blood pressure, cyst distribution and extrarenal features.
- Apply validated imaging criteria when appropriate; seek specialist radiology or genetic input for atypical morphology, absent family history or potential donation.
Key medicines
TolvaptanInitiate and titrate only in a specialist ADPKD service for adults meeting current NICE criteria; use split dosing, interaction adjustment and stopping decisions exactly as specified by the live UKKA pathway and SmPC.
ACE inhibitor or angiotensin-receptor blockerSelect one agent from the local CKD formulary and titrate to the agreed blood-pressure target with renal function and potassium monitoring.