Approval of the vasopressin V2 receptor antagonist tolvaptan-based on the landmark TEMPO 3:4 trial-marked a transformation in the management of autosomal dominant polycystic kidney disease (ADPKD). This development has advanced patient care in ADPKD from general measures to prevent progression of chronic kidney disease to targeting disease-specific mechanisms. However, considering the long-term nature of this treatment, as well as potential side effects, evidence-based approaches to initiate treatment only in patients with rapidly progressing disease are crucial. In 2016, the position statement issued by the European Renal Association (ERA) was the first society-based recommendation on the use of tolvaptan and has served as a widely used decision-making tool for nephrologists. Since then, considerable practical experience regarding the use of tolvaptan in ADPKD has accumulated. More importantly, additional data from REPRISE, a second randomized clinical trial (RCT) examining the use of tolvaptan in later-stage disease, have added important evidence to the field, as have post hoc studies of these RCTs. To incorporate this new knowledge, we provide an updated algorithm to guide patient selection for treatment with tolvaptan and add practical advice for its use.

Academic Nephrology Unit Department of Infection Immunity and Cardiovascular Disease University of Sheffield Medical School Sheffield UK

Association PolyKystose France Saclay France

Biogem Institute for Molecular Biology and Genetics Ariano Irpino Italy

CECAD University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany

Department 2 of Internal Medicine and Center for Molecular Medicine University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany

Department Genetics University Medical Centre Groningen Groningen The Netherlands

Department of Genetics University Medical Center Utrecht Utrecht The Netherlands

Department of Internal Medicine Division of Nephrology and Transplantation Erasmus Medical Center University Medical Center Rotterdam Rotterdam The Netherlands

Department of Nephrology 1st Faculty of Medicine General University Hospital Prague Czech Republic

Department of Nephrology Hemodialysis and Renal Transplantation CHU and University of Brest Brest France

Department of Nephrology Radboud Institute for Molecular Life Sciences Radboudumc Center of Expertise for Rare Kidney Disorders Radboud University Medical Center Nijmegen The Netherlands

Department of Nephrology University Medical Center Groningen University of Groningen Groningen The Netherlands

Department of Pediatric Nephrology and Organ Transplantation University Hospitals Leuven Leuven Belgium

Department of Renal Medicine Aarhus University Hospital Aarhus Denmark

Department of Renal Medicine University College London London UK

Department of Translational Medical Sciences Vanvitelli University Naples Italy

Departments of Clinical Medicine and Biomedicine Aarhus University Aarhus Denmark

Division of Nephrology UCL Medical School Brussels Belgium

Division of Pediatric Nephrology Center for Pediatrics and Adolescent Medicine Heidelberg University Hospital Heidelberg Germany

Inherited Kidney Diseases Nephrology Department Fundació Puigvert Instituto de Investigaciones Biomédicas Sant Pau Universitat Autònoma de Barcelona Barcelona Spain

Institute of Physiology University of Zurich Zurich Switzerland

Nephrology and Transplantation Department Necker University Hospital APHP Paris France

PKD Familiäre Zystennieren e 5 Bensheim Germany

PKD International Geneva Switzerland

PKD Research Group Laboratory of Pediatrics Department of Development and Regeneration KU Leuven Leuven Belgium

REDINREN Barcelona Spain

Sheffield Kidney Institute Sheffield Teaching Hospitals NHS Foundation Trust Sheffield UK

Translational and Clinical Research Institute Faculty of Medical Sciences Newcastle University Newcastle Upon Tyne UK

University Brest Inserm UMR 1078 GGB CHU Brest Brest France

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