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.
- Control blood pressure early and track eGFR slope, albuminuria and complications as standard kidney protection.
- Risk assessment for rapid progression may use serial eGFR, height-adjusted total kidney volume, Mayo imaging class, genotype and clinical events through a specialist clinic.
- NICE recommends tolvaptan for a defined adult CKD and rapid-progression population; current UKKA advice expands practical assessment but does not replace the appraisal or SmPC.
- Tolvaptan causes aquaresis and carries important liver-injury risk, requiring pre-treatment testing, monthly liver tests for 18 months and three-monthly tests thereafter.
- Intracranial aneurysm screening is selective and based on family history, previous aneurysm, high-risk occupation or major elective surgery and patient preference, not automatic for everyone.
Key red flags
Fever, inflammatory markers and focal renal or hepatic tenderness may occur even when routine urine culture is negative.
Thunderclap headache, meningism, collapse or focal deficit is a neurological emergency requiring the standard subarachnoid-haemorrhage pathway.
Inability to replace water losses, hypernatraemia symptoms or fatigue, anorexia, dark urine and jaundice require prompt treatment review.
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.
- Offer genetics-led cascade and reproductive counselling after confirmation, allowing at-risk adults to choose testing with informed consent.
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.Mandatory liver tests are required before treatment, monthly for 18 months and three-monthly thereafter. Counsel about thirst, polyuria, nocturia, dehydration, hypernatraemia, CYP3A interactions, pregnancy and withholding during acute illness.
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.Do not combine classes. Reassess during hypovolaemia, especially when aquaretic treatment is used, and follow pregnancy contraindications and sick-day advice.