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
, 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
,contents www.kidney-international.org
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 ADPKD
S191 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
, www.kidney-international.org contents
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 PLD
S149 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 ADPKD
S165 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. Clinical questions and SR topics in PICOD format
S203 Table 23. GRADE system for grading the certainty of evidence
S203 Table 24. KDIGO nomenclature and description for grading recommendations
S203 Table 25. Determinants of the strength of recommendation
S204 Table 26. Classification for the grade of the certainty of evidence
FIGURES
S24 Figure 1. Diagnosis algorithm in at-risk adults (positive family history) for autosomal dominant polycystic kidney
disease (ADPKD)
S63 Figure 2. Diagnosis algorithm in adults with incidentally detected kidney and/or liver cysts in absence of known
family history of autosomal dominant polycystic kidney disease (ADPKD)
S23 Figure 3. Ultrasound criteria by age group to diagnose autosomal dominant polycystic kidney disease (ADPKD)
in people with a positive family history based on a positive predictive value of the test
S23 Figure 4. Ultrasound criteria by age group to exclude autosomal dominant polycystic kidney disease (ADPKD) in
people with a positive family history based on a negative predictive value of the test
S23 Figure 5. Magnetic resonance imaging (MRI) criteria for ages 16–40 years in people with a positive family history
S75 Figure 6. Factors associated with the rate of disease progression in autosomal dominant polycystic kidney
disease (ADPKD)
S77 Figure 7. Methods to assess the rate of kidney disease progression in autosomal dominant polycystic kidney
disease (ADPKD)
S79 Figure 8. The Mayo Imaging Classification divides height-adjusted total kidney volume (htTKV)/age into 5
different classes
S80 Figure 9. The Mayo Imaging Classification (MIC) of autosomal dominant polycystic kidney disease (ADPKD; left
panel) with examples (right panel) of (a,b) MIC subclass 1A and 1E, (c–f) MIC subclass 2A, and (g,h) MIC
subclass 2B
Kidney International (2025) 107 (Suppl 2S), S1–S239 S3
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
, 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
,contents www.kidney-international.org
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 ADPKD
S191 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
, www.kidney-international.org contents
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 PLD
S149 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 ADPKD
S165 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. Clinical questions and SR topics in PICOD format
S203 Table 23. GRADE system for grading the certainty of evidence
S203 Table 24. KDIGO nomenclature and description for grading recommendations
S203 Table 25. Determinants of the strength of recommendation
S204 Table 26. Classification for the grade of the certainty of evidence
FIGURES
S24 Figure 1. Diagnosis algorithm in at-risk adults (positive family history) for autosomal dominant polycystic kidney
disease (ADPKD)
S63 Figure 2. Diagnosis algorithm in adults with incidentally detected kidney and/or liver cysts in absence of known
family history of autosomal dominant polycystic kidney disease (ADPKD)
S23 Figure 3. Ultrasound criteria by age group to diagnose autosomal dominant polycystic kidney disease (ADPKD)
in people with a positive family history based on a positive predictive value of the test
S23 Figure 4. Ultrasound criteria by age group to exclude autosomal dominant polycystic kidney disease (ADPKD) in
people with a positive family history based on a negative predictive value of the test
S23 Figure 5. Magnetic resonance imaging (MRI) criteria for ages 16–40 years in people with a positive family history
S75 Figure 6. Factors associated with the rate of disease progression in autosomal dominant polycystic kidney
disease (ADPKD)
S77 Figure 7. Methods to assess the rate of kidney disease progression in autosomal dominant polycystic kidney
disease (ADPKD)
S79 Figure 8. The Mayo Imaging Classification divides height-adjusted total kidney volume (htTKV)/age into 5
different classes
S80 Figure 9. The Mayo Imaging Classification (MIC) of autosomal dominant polycystic kidney disease (ADPKD; left
panel) with examples (right panel) of (a,b) MIC subclass 1A and 1E, (c–f) MIC subclass 2A, and (g,h) MIC
subclass 2B
Kidney International (2025) 107 (Suppl 2S), S1–S239 S3
