KDIGO 2025 clinical practice guideline for the evaluation, management, and treatment of autosomal dominant polycystic kidney disease (ADPKD): executive summary

Vicente E Torres; Curie Ahn; Thijs R M Barten; Godela Brosnahan; Melissa A Cadnapaphornchai; Arlene B Chapman; Emilie Cornec-Le Gall; Joost P H Drenth; Ron T Gansevoort; Peter C Harris; Tess Harris; Shigeo Horie; Max C Liebau; Michele Liew; Andrew J Mallett; Changlin Mei; Djalila Mekahli; Dwight Odland; Albert C M Ong; Luiz F Onuchic; York P-C Pei; Ronald D Perrone; Gopala K Rangan; Brian Rayner; Roser Torra; Ethan M Balk; Craig E Gordon; Amy Earley; Reem A Mustafa; Olivier Devuyst

doi:10.1016/j.kint.2024.07.010

KDIGO 2025 clinical practice guideline for the evaluation, management, and treatment of autosomal dominant polycystic kidney disease (ADPKD): executive summary

Kidney Int. 2025 Feb;107(2):234-254. doi: 10.1016/j.kint.2024.07.010.

Authors

Vicente E Torres¹, Curie Ahn², Thijs R M Barten³, Godela Brosnahan⁴, Melissa A Cadnapaphornchai⁵, Arlene B Chapman⁶, Emilie Cornec-Le Gall⁷, Joost P H Drenth³, Ron T Gansevoort⁸, Peter C Harris⁹, Tess Harris¹⁰, Shigeo Horie¹¹, Max C Liebau¹², Michele Liew¹³, Andrew J Mallett¹⁴, Changlin Mei¹⁵, Djalila Mekahli¹⁶, Dwight Odland¹⁷, Albert C M Ong¹⁸, Luiz F Onuchic¹⁹, York P-C Pei²⁰, Ronald D Perrone²¹, Gopala K Rangan²², Brian Rayner²³, Roser Torra²⁴, Ethan M Balk²⁵, Craig E Gordon²⁶, Amy Earley²⁷, Reem A Mustafa²⁸, Olivier Devuyst²⁹

Affiliations

¹ Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota, USA. Electronic address: [email protected].
² Laboratory of Transplantation Immunology, Seoul National University, Seoul, South Korea.
³ Department of Gastroenterology and Hepatology, Radboud University Medical Center, Nijmegen, The Netherlands.
⁴ Department of Medicine, University of Colorado, Aurora, Colorado, USA.
⁵ Division of Nephrology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA; Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA.
⁶ Section of Nephrology, University of Chicago, Chicago, Illinois, USA.
⁷ Department of Nephrology, Centre de reference MAladies Rénales Héréditaires de l'Enfant et de l'Adulte (MARHEA), University Hospital of Brest, Brest, France; Division of Nephrology, Inserm UMR1078, Brest, France.
⁸ Department of Nephrology, University of Groningen, Groningen, The Netherlands.
⁹ Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota, USA.
¹⁰ Polycystic Kidney Disease Charity UK, London, UK.
¹¹ Department of Urology, Juntendo University, Tokyo, Japan.
¹² Department of Pediatrics, Center for Rare Diseases, Medical Faculty, Cologne, Germany; Department of Pediatrics, Center for Family Health, Medical Faculty, Cologne, Germany; Department of Pediatrics, Center for Molecular Medicine, Medical Faculty, Cologne, Germany; University Hospital Cologne, Cologne, Germany.
¹³ Patient partner, Hong Kong, China.
¹⁴ Department of Renal Medicine, Townsville University Hospital, Townsville, Queensland, Australia.
¹⁵ Department of Nephrology, Changzheng Hospital, Shanghai, China.
¹⁶ Department of Cellular and Molecular Medicine, KU Leuven, Leuven, Belgium; Department of Pediatric Nephrology, University Hospital Leuven, Leuven, Belgium.
¹⁷ PKD Foundation, Los Angeles, California, USA.
¹⁸ Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.
¹⁹ Division of Nephrology, Universidade de São Paulo, São Paulo, Brazil.
²⁰ Division of Nephrology, University of Toronto, Toronto, Ontario, Canada.
²¹ Division of Nephrology, Tufts University School of Medicine, Boston, Massachusetts, USA.
²² Department of Renal Medicine, Westmead Institute for Medical Research, The University of Sydney, Sydney, New South Wales, Australia.
²³ Division of Nephrology and Hypertension, Groote Schuur Hospital, University of Cape Town, Cape Town, South Africa.
²⁴ Department of Nephrology, Fundació Puigvert, IR Sant Pau, Barcelona, Spain.
²⁵ Center for Evidence Synthesis in Health, Brown University School of Public Health, Providence, Rhode Island, USA.
²⁶ Division of Nephrology, Tufts University School of Medicine, Boston, Massachusetts, USA; Center for Evidence Synthesis in Health, Brown University School of Public Health, Providence, Rhode Island, USA.
²⁷ KDIGO, Brussels, Belgium.
²⁸ Department of Internal Medicine, Division of Nephrology and Hypertension, University of Kansas, Kansas City, Kansas, USA.
²⁹ Institute of Physiology, University of Zurich, Zurich, Switzerland; Division of Nephrology, Cliniques universitaires Saint-Luc, UCLouvain Medical School, Brussels, Belgium. Electronic address: [email protected].

PMID: 39848746
DOI: 10.1016/j.kint.2024.07.010

Abstract

The Kidney Disease: Improving Global Outcomes (KDIGO) 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) represents the first KDIGO guideline on this subject. Its scope includes nomenclature, diagnosis, prognosis, and prevalence; kidney manifestations; chronic kidney disease (CKD) management and progression, kidney failure, and kidney replacement therapy; therapies to delay progression of kidney disease; polycystic liver disease; intracranial aneurysms and other extrarenal manifestations; lifestyle and psychosocial aspects; pregnancy and reproductive issues; pediatric issues; and approaches to the management of people with ADPKD. The guideline has been developed with patient partners, clinicians, and researchers around the world, with the goal to generate a useful resource for healthcare providers and patients by providing actionable recommendations. The development of this guideline followed an explicit process of evidence review and appraisal, based on a rigorous, formal systematic literature review. The strength of recommendations follows the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach. The guideline also provides practice points serving to direct clinical care or activities relating to areas for which a systematic review was not conducted. Limitations of the evidence are discussed. Research recommendations to address gaps in knowledge, and implications for policy and payment, are provided. The guideline targets a broad audience of healthcare providers, people living with ADPKD, and stakeholders involved in the various aspects of ADPKD care.

Keywords: ADPKD; evidence-based; guideline; monogenic disease; nomenclature.

Publication types

Practice Guideline

MeSH terms

Disease Progression
Humans
Nephrology / standards
Polycystic Kidney, Autosomal Dominant* / diagnosis
Polycystic Kidney, Autosomal Dominant* / therapy
Renal Insufficiency, Chronic / diagnosis
Renal Insufficiency, Chronic / etiology
Renal Insufficiency, Chronic / therapy
Renal Replacement Therapy / standards