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Polycystic kidney disease

Last reviewed: 2 Apr 2025
Last updated: 10 Jan 2025

Summary

Definition

History and exam

Key diagnostic factors

  • family history of autosomal-dominant PKD (ADPKD) or end-stage renal disease
  • family history of cerebrovascular event
  • renal cysts
  • hypertension
  • abdominal/flank pain
  • hematuria
  • palpable kidneys/abdominal mass
  • headaches
  • dysuria, suprapubic pain, fever
Full details

Other diagnostic factors

  • cardiac murmur
  • abdominal hernia
  • hepatomegaly
  • chest pain
Full details

Risk factors

  • family history of autosomal-dominant PKD (ADPKD)
  • family history of cerebrovascular event
Full details

Diagnostic tests

1st tests to order

  • renal ultrasound
  • CT abdomen/pelvis
  • MRI abdomen/pelvis
  • urinalysis/Gram stain and urine culture
  • serum electrolytes, BUN, creatinine
  • fasting lipid profile
  • ECG
  • CT brain
Full details

Tests to consider

  • genetic testing
  • echocardiogram
  • 24-hour urine collection
  • dual-energy CT of abdomen/pelvis
  • lumbar puncture and cerebrospinal fluid analysis
  • C-reactive protein
  • PET scan
Full details

Treatment algorithm

ACUTE

confirmed autosomal-dominant polycystic disease

ONGOING

end-stage renal disease

Contributors

Authors

Marie C. Hogan, MD, PhD

Consultant

Division of Nephrology

Professor of Medicine

College of Medicine

Mayo Clinic

Rochester

MN

Disclosures

MCH receives research funding from Novartis, and was an investigator participating in tolvaptan clinical trials. MCH is also an author of several references cited in this topic.

Maria Irazabel Mira, MD, PhD

Associate Professor of Medicine, Associate Consultant

Division of Nephrology and Hypertension

Department of Internal Medicine

Mayo Clinic

Rochester

MN

Disclosures

MIM is an author of several references cited in this topic.

Acknowledgements

Dr Marie C Hogan and Dr Maria Irazabal Mira would like to gratefully acknowledge Dr Vicente Torres, a previous contributor to this topic.

Disclosures: VT is an author of several references cited in this topic.

Peer reviewers

Richard Sandford, PhD, FRCP

Wellcome Trust Senior Fellow in Clinical Research

University Reader in Real Genetics

Honorary Consultant in Medical Genetics

Cambridge

UK

Disclosures

RS declares that he has no competing interests.

Arlene Chapman, MD

Professor of Medicine

Renal Division

Emory University

School of Medicine

Atlanta

GA

Disclosures

Not disclosed.

References

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Key articles

Bergmann C, Guay-Woodford LM, Harris PC, et al. Polycystic kidney disease. Nat Rev Dis Primers. 2018 Dec 6;4(1):50.Full text  Abstract

Cornec-Le Gall E, Alam A, Perrone RD. Autosomal dominant polycystic kidney disease. Lancet. 2019 Mar 2;393(10174):919-35. Abstract

Pei Y, Obaji J, Dupuis A, et al. Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 2009 Jan;20(1):205-12.Full text  Abstract

Torres VE, Chapman AB, Devuyst O, et al; TEMPO 3:4 Trial Investigators. Tolvaptan in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2012 Dec 20;367(25):2407-18.Full text  Abstract

Schrier RW, Abebe KZ, Perrone RD, et al; HALT-PKD Trial Investigators. Blood pressure in early autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11;371(24):2255-66. Abstract

Torres VE, Abebe KZ, Chapman AB, et al; HALT-PKD Trial Investigators. Angiotensin blockade in late autosomal dominant polycystic kidney disease. N Engl J Med. 2014 Dec 11;371(24):2267-76. Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Polycystic kidney disease images
  • Differentials

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    • Simple cyst
    • Tuberous sclerosis complex
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  • Guidelines

    • Autosomal-dominant polycystic kidney disease (ADPKD): executive summary
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  • Patient information

    Kidney infection

    Kidney stones

    More Patient information
  • Calculators

    Glomerular Filtration Rate Estimate by the IDMS-Traceable MDRD Study Equation

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  • Videos

    Diagnostic lumbar puncture in adults: animated demonstration

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