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Renal & Urology

Polycystic Kidney Disease

Autosomal dominant polycystic kidney disease (ADPKD) is an inherited cystic kidney disorder causing enlarged kidneys, hypertension and progressive CKD, with important renal, hepatic, vascular, reproductive and family implications.

In a nutshell

ADPKD causes progressive bilateral renal cysts, hypertension and CKD, with liver cysts and vascular complications. Confirm the phenotype and stage BP, ACR and eGFR; use renal-led risk assessment for tolvaptan, follow NICE UK eligibility, and investigate fever, haematuria, stones and thunderclap headache promptly.

Classic presentation

A young adult with a first-degree family history has hypertension, bilateral cystic kidneys and a slowly falling eGFR; a separate patient with ADPKD develops fever and focal loin pain or a sudden thunderclap headache.

Key points

  • ADPKD is systemic: renal cysts are accompanied by hypertension, liver cysts, pain, infection, stones and vascular risk.
  • Use serial eGFR and specialist imaging-based risk assessment to identify rapid progression; one scan or one creatinine is not enough.
  • NICE TA358 restricts NHS tolvaptan eligibility to adults with CKD stage 2 or 3 at treatment start and evidence of rapid progression.
  • Do not screen every asymptomatic person for intracranial aneurysm automatically; use a risk-led, shared decision-making pathway.
  • Fever with focal loin pain needs cyst infection consideration and specialist antimicrobial advice.
  • A thunderclap headache is a subarachnoid-haemorrhage emergency, not a routine PKD follow-up problem.

First-line investigation

Renal ultrasound plus BP, urine ACR, urinalysis, eGFR and family-history assessment; use specialist MRI/genetic testing when ultrasound is equivocal.

Management

Identify complications needing emergency care

  • Send thunderclap headache through the urgent subarachnoid-haemorrhage pathway; admit and investigate fever with focal loin pain, haemodynamic haematuria or acute kidney deterioration.7,1,5

Confirm and stage the disease

  • Use family history, renal imaging, BP, ACR, urinalysis and serial eGFR; involve renal/genetics services when imaging is equivocal or family testing is requested.5,2,1

Slow progression and manage complications

  • Treat hypertension through NICE CKD guidance, consider tolvaptan only in the specialist UK pathway, and use cause-specific infection, stone, haematuria, aneurysm and CKD management.5,3,4,1

Plan family and kidney-failure care

  • Continue BP, ACR, eGFR and treatment-toxicity surveillance, offer genetic/reproductive counselling, discuss selective aneurysm imaging and plan transplant, dialysis or conservative care before kidney failure.5,1,2,8

Exam traps

  • Do not diagnose ADPKD from a few simple cysts without age, family-history and phenotype context.
  • NICE TA358 UK tolvaptan eligibility is not the same as every international rapid-progression criterion.
  • Do not promise that high water intake prevents progression; give individualised renal advice and avoid dehydration.
  • Cyst infection may need prolonged lipid-soluble antibiotic treatment and is not the same as uncomplicated cystitis.
  • Routine aneurysm screening is not mandatory for every asymptomatic patient; risk-led shared decision-making is current.
  • Thunderclap headache requires the NICE subarachnoid-haemorrhage pathway even when ADPKD is already known.

Illustrations

Polycystic kidneys on CT or ultrasoundMultiplanar CT showing bilaterally enlarged kidneys containing numerous cysts of varying size in autosomal dominant polycystic kidney disease.Hg6996, Wikimedia Commons · CC-BY-SA-3.0
Intracranial berry aneurysmAn illustration or angiogram of a berry aneurysm at a circle of Willis branch point, the feared extrarenal complication causing subarachnoid haemorrhage.Nicholas Zaorsky, M.D, Wikimedia Commons · CC-BY-SA-3.0

Key sources

  1. KDIGO 2025 Clinical Practice Guideline for ADPKD (Current international ADPKD guideline used for updated progression assessment, kidney/liver complications, cyst infection, intracranial aneurysm risk and tolvaptan safety where no newer UK disease-specific adult guidance was identified; published January 2025)Published 21 Jan 2025
  2. UK Kidney Association, Monitoring children and young people with or at risk of ADPKD (UK paediatric ADPKD monitoring and shared-decision guidance; published 1 November 2018, review date 1 November 2023)Published 1 Nov 2018
  3. NICE TA358, Tolvaptan for treating autosomal dominant polycystic kidney disease (Current UK NHS technology-appraisal eligibility: adults with CKD stage 2 or 3 at treatment start and evidence of rapidly progressing ADPKD; published 28 October 2015)Published 28 Oct 2015
  4. UK Kidney Association, Commentary on tolvaptan for ADPKD (Current UK Kidney Association commentary on implementing updated ADPKD and tolvaptan evidence in UK practice; published 1 March 2024, review date 1 March 2027)Published 1 Mar 2024
  5. NICE NG203, Chronic kidney disease: assessment and management (Current UK CKD pathway for ACR, BP, ACE/ARB treatment, monitoring and referral; published 2021 and accessed 4 August 2026)Published 23 Nov 2021
  6. BNF, ACE inhibitors and angiotensin-receptor blockers (Current UK prescribing, renal-function, potassium, pregnancy and monitoring information for renin–angiotensin system blockers)
  7. NICE NG228, Subarachnoid haemorrhage caused by a ruptured aneurysm (Current UK emergency assessment pathway for thunderclap headache, CT head, lumbar puncture timing and specialist transfer; published 23 November 2022)Published 23 Nov 2022
  8. BNF, Tolvaptan and renal prescribing (Current UK prescribing information for tolvaptan, liver safety, renal dosing, interactions, pregnancy and monitoring; direct monograph access may require NHS login)
  9. NICE CKS, Autosomal dominant polycystic kidney disease (NICE CKS diagnostic and management topic anchor; access may require an NHS or institutional login, so specific claims are anchored to directly accessible NICE, UKKA and KDIGO sources)
  10. BNF, antimicrobials and renal dosing (Current UK antimicrobial choice, renal adjustment, interaction and safety information; use local microbiology guidance for cyst infection)
  11. NICE NG136, Hypertension in adults: diagnosis and management (Current NICE adult hypertension pathway used when ADPKD-specific treatment does not add a separate recommendation)Published 28 Aug 2019

This page is exam revision material, not medical advice, and must not be used for patient care. Always check drug doses against the BNF and current guidance. Full disclaimer.