Autosomal dominant polycystic kidney disease (ADPKD) is one of the most frequent genetic diseases, affecting an estimated 12.5 million people worldwide. Despite its importance, approaches to the diagnosis, evaluation, prevention and treatment of the renal and extrarenal manifestations of ADPKD vary widely and no widely accepted practice guidelines exist for this disease. As such, KDIGO held a Controversies Conference on ADPKD in Edinburgh, United Kingdom on January 17-19, 2014. Drs. Vicente E. Torres (Mayo Clinic, USA) and Olivier Devuyst (University of Zurich, Switzerland) co-chaired this conference.

The aim of this KDIGO conference was to gather a global panel of multi-disciplinary clinical expertise that identified key issues relevant to the progression factors, mortality, co-morbidity, as well as patient support and health economical issues of ADPKD. The objective of this conference was to assess our current state of knowledge related to the evaluation, management and treatment of ADPKD, to summarize the outstanding knowledge gaps, and to propose a research agenda to resolve standing controversial issues. This conference served to inform whether there is sufficient evidence base for the development of a guideline on this topic and helped pave the way to harmonize and standardize the care of ADPKD patients.