KDIGO 2025 Clinical Practice Guideline - Autosomal Dominant Polycystic Kidney Disease (ADPKD)

Sep 18, 2026

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KDIGO 2025 provides a comprehensive clinical practice guideline supplement for evaluating, managing, and treating autosomal dominant polycystic kidney disease (ADPKD). The supplement spans nomenclature, diagnosis, prognosis, and epidemiology, then kidney manifestations, CKD management and progression, kidney failure, and kidney replacement therapy. It includes therapies to delay disease progression, extrarenal involvement such as polycystic liver disease and intracranial aneurysms, and addresses lifestyle, psychosocial factors, pregnancy, reproductive issues, and pediatric considerations.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages240

Reading Time10 h 5 min

AccessFree to download

Table list, figure list, and supplementary material

KDIGO Executive Committee

Reference keys

Current chronic kidney disease (CKD) nomenclature used by KDIGO

Conversion factors of conventional units to SI units

Equivalent albuminuria categories in CKD

Abbreviations and acronyms

Notice

Foreword

Dedication

Work Group membership

Abstract

Introduction from the Guideline Co-Chairs

Summary of recommendation statements and practice points

Chapter 1: Nomenclature, diagnosis, prognosis, and prevalence

Chapter 2: Kidney manifestations

Chapter 3: Chronic kidney disease (CKD) management and progression, kidney failure, and kidney replacement therapy (KRT)

Chapter 4: Therapies to delay the progression of kidney disease

Chapter 5: Polycystic liver disease

Chapter 6: Intracranial aneurysms and other extrarenal manifestations

Chapter 7: Lifestyle and psychosocial aspects

Chapter 8: Pregnancy and reproductive issues

Chapter 9: Pediatric issues

Chapter 10: Approaches to the management of people with ADPKD

Methods for guideline development

Appendix 1: Patient-reported outcome measures (PROMs) in ADPKD care

Appendix 2: Recommended checklist of issues to discuss at the beginning of the patient journey

Appendix 3: Patient organizations dealing with ADPKD or kidney disease in general, and other useful resources

SUPPLEMENT TO KDIGO 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) VOLUME 107 | ISSUE 2S | FEBRUARY 2025 [www.kidney-international.org](www.kidney-international.org) KDIGO 2025 CLINICAL PRACTICE GUIDELINE FOR THE EVALUATION, MANAGEMENT, AND TREATMENT OF AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE (ADPKD) Kidney International (2025) 107 (Suppl 2S), S1–S239 S1 VOL 107 | ISSUE 2S | FEBRUARY 2025 KDIGO 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) S3 Table list, ﬁgure list, and supplementary material S7 KDIGO Executive Committee S8 Reference keys S9 Current chronic kidney disease (CKD) nomenclature used by KDIGO S10 Conversion factors of conventional units to SI units S10 Equivalent albuminuria categories in CKD S11 Abbreviations and acronyms S13 Notice S14 Foreword S15 Dedication S17 Work Group membership S19 Abstract S20 Introduction from the Guideline Co-Chairs S22 Summary of recommendation statements and practice points S56 Chapter 1: Nomenclature, diagnosis, prognosis, and prevalence S85 Chapter 2: Kidney manifestations S99 Chapter 3: Chronic kidney disease (CKD) management and progression, kidney failure, and kidney replacement therapy (KRT) S112 Chapter 4: Therapies to delay the progression of kidney disease S134 Chapter 5: Polycystic liver disease S146 Chapter 6: Intracranial aneurysms and other extrarenal manifestations S158 Chapter 7: Lifestyle and psychosocial aspects S168 Chapter 8: Pregnancy and reproductive issues S176 Chapter 9: Pediatric issues S187 Chapter 10: Approaches to the management of people with ADPKDS191 Methods for guideline development S205 Biographic and disclosure information S216 Acknowledgments S217 References S236 Appendix 1: Patient-reported outcome measures (PROMs) in ADPKD care S238 Appendix 2: Recommended checklist of issues to discuss at the beginning of the patient journey S239 Appendix 3: Patient organizations dealing with ADPKD or kidney disease in general, and other useful resources for patients and healthcare providers This article is published as part of a supplement sponsored by Kidney Disease: Improving Global Outcomes (KDIGO) . S2 Kidney International (2025) 107 (Suppl 2S), S1–S239 TABLES S57 Table 1. Genes associated with the ADPKD spectrum, designations, and phenotype S58 Table 2. Genes associated with the ADPLD spectrum, designations, and phenotype S64 Table 3. Factors to consider when testing (by imaging and/or genetics) people at risk for ADPKD S25 Table 4. Situations in which genetic testing can clarify the diagnosis and aid in determining a prognosis S26 Table 5. Other disorders that present with kidney cysts S72 Table 6. Genetic testing methods for screening for ADPKD and ADPLD S73 Table 7. Consequences of genetic testing by tNGS for people with typical or atypical ADPKD S113 Table 8. Approaches to reduce AVP activity in ADPKD S119 Table 9. Checklist of contraindications to initiating and/or maintaining tolvaptan use S124 Table 10. Relative contraindications for increasing water intake S126 Table 11. Factors to be considered before advising increased water intake S135 Table 12. Classiﬁcations for PLD S39 Table 13. Methods to assess sarcopenia and malnutrition S139 Table 14. Treatment options in PLDS149 Table 15. Risk factors for ICAs or SAH S42 Table 16. Advantages and limitations of screening for ICAs S155 Table 17. Extrarenal manifestations S44 Table 18. Nutrition guidance for people with ADPKD and CKD G1–G4 S163 Table 19. Recommendations on use of common lifestyle products in people with ADPKDS165 Table 20. Key concerns of people with ADPKD and their family members, relating to psychosocial issues S50 Table 21. Deﬁnitions of phenotypical entities in children with ADPKD S192 Table 22. Clinica

FAQs

What conditions and topics does the KDIGO 2025 ADPKD guideline supplement cover?

It covers evaluation and management of ADPKD across diagnosis, prognosis, and prevalence, kidney manifestations, CKD progression, kidney failure and kidney replacement therapy, and therapies intended to delay progression. It also addresses extrarenal manifestations including polycystic liver disease and intracranial aneurysms, plus lifestyle, psychosocial aspects, pregnancy and reproductive issues, and pediatric issues.

Which clinical chapters focus on delaying progression and related therapies?

The supplement includes Chapter 4 on therapies to delay progression of kidney disease and also includes content on approaches to delay disease progression and reduce relevant biological activity, along with related contraindication and consideration tables.

Does the supplement include appendices for patient-centered outcomes and patient journey checklists?

Yes. Appendix 1 lists patient-reported outcome measures (PROMs) in ADPKD care, Appendix 2 provides a recommended checklist of issues to discuss at the beginning of the patient journey, and Appendix 3 lists patient organizations and other useful resources.

Sep 18, 2026

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