The KDIGO 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) was published online in January 2025. The guideline and Executive Summary will be published in the February issue of Kidney International Supplements. The guideline was co-chaired by Olivier Devuyst, MD, PhD (Switzerland), and Vicente Torres, MD, PhD (United States).
ADPKD affects up to 12 million people worldwide and is the most prevalent monogenic kidney disease associated with kidney failure. The ADPKD Guideline, the first guideline from KDIGO on a rare kidney disease, provides healthcare providers, people living with ADPKD, and stakeholders with practical tools to improve diagnosis, care, and treatment, and address the complex challenges of managing this widespread inherited kidney disorder. The guideline spans topics such as disease nomenclature, diagnosis, prognosis, kidney and extrarenal manifestations, treatment strategies, and pregnancy and reproductive issues. The guideline also includes a dedicated chapter for issues related to children.
Treatment approaches and actionable guideline recommendations were based on a systematic review of relevant studies and appraisal of the certainty of the evidence and the strength of recommendations, following the “Grading of Recommendations Assessment, Development, and Evaluation” (GRADE) approach. The guideline provides practice points for which a systematic review was not conducted to direct clinical care or aid in the implementation of graded recommendations, as well as a series of useful infographics summarizing key guideline messages.
The guideline introduces a new disease nomenclature and offers updated recommendations on therapies to slow disease progression, including the use of tolvaptan, while stressing the importance of shared decision-making and individualized care. The guideline also emphasizes patient-centered approaches, addressing resource disparities and cultural differences in healthcare delivery. It details the steps for attaining an accurate diagnosis and prognosis assessment using tools like the Mayo Imaging Classification (MIC) and/or Predicting Renal Outcome in Polycystic Kidney Disease (PROPKD) score for risk stratification and includes recommendations and practice points for managing ADPKD-associated conditions.
“This comprehensive guideline reflects the significant progress in ADPKD research over the last decade, from genetic insights to advanced therapies,” said Dr. Torres. “By addressing the complexities of diagnosis, treatment, and management, we hope this guideline will standardize care, enhance clinical practices, and help reduce the global burden of ADPKD. Our goal is that this resource will not only empower clinical decision-making but also inspire hope for patients and their families, demonstrating that meaningful progress is achievable for this challenging disease.”
“The publication of this guideline represents a pivotal step forward in the care of ADPKD and people with rare genetic disorders worldwide,” said Dr. Devuyst. “It is the result of an extraordinary global collaboration among experts, researchers, and patient advocates, bringing together the best available evidence to guide care across diverse settings. This guideline is not just important for individuals with ADPKD. It also serves as a crucial template for the development of guidance for other rare genetic conditions. By providing a roadmap for consistent, evidence-based care, it addresses critical knowledge and practice gaps worldwide, offering a model for improving the lives of those with rare diseases globally.”
DISCLAIMER: USE OF THE CLINICAL PRACTICE GUIDELINES
This Clinical Practice Guideline is based upon the best information available at the time of publication. The recommendations are designed to provide information and assist decision-making. They are not intended to define a standard of care, and should not be construed as one. Neither should they be interpreted as prescribing an exclusive course of management. Variations in practice will inevitably and appropriately occur when clinicians take into account the needs of individual patients, available resources, and limitations unique to an institution or type of practice. Every health care professional making use of this Guideline is responsible for evaluating the appropriateness of applying them in the setting of any particular clinical situation. The recommendations for research contained within this document are general and do not imply a specific protocol.
