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Nephrol Dial Transplant. 2016 Mar;31(3):337-48. doi: 10.1093/ndt/gfv456. Epub 2016 Jan 29.

Recommendations for the use of tolvaptan in autosomal dominant polycystic kidney disease: a position statement on behalf of the ERA-EDTA Working Groups on Inherited Kidney Disorders and European Renal Best Practice.

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Department of Nephrology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Department of Nephrology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Department II of Internal Medicine and Centre for Molecular Medicine Cologne, University of Cologne, Cologne, Germany.
Department of Renal Medicine, Aarhus University Hospital, Aarhus, Denmark Department of Biomedicine, Aarhus University, Aarhus, Denmark.
Department of Nephrology, Second University of Naples, Naples, Italy.
Nephrology Clinic, Dialysis and Renal Transplant Center, 'C.I. PARHON' University Hospital, and 'Grigore T. Popa' University of Medicine, Iasi, Romania.
Institute of Physiology, University of Zurich, Zurich, Switzerland Division of Nephrology, UCL Medical School, Brussels, Belgium.
Renal Division, University of Würzburg, University Hospital, Wurzburg, Germany.
Department of Nephrology and Hypertension, Friedrich-Alexander University Erlangen-Nürnberg (FAU), Erlangen, Germany.
Department of Nephrology and Urology, Bambino Gesù Children's Hospital-IRCCS, Rome, Italy.
Department of Nephrology, Hôpital Necker, Paris Descartes University, Paris, France.
Service de Néphrologie, Hémodialyse et Transplantation Rénale, Hôpital La Cavale Blanche, Centre Hospitalier Régional Universitaire de Brest, Brest, France.
Division of Nephrology, Ambroise Paré Hospital, Assistance Publique Hôpitaux de Paris, Boulogne-Billancourt/Paris, France Inserm U-1018, Equipe 5, Villejuif, France University of Paris Saclay and Paris Ouest-Versailles-Saint-Quentin-en-Yvelines (UVSQ), France.
Academic Nephrology Unit, University of Sheffield Medical School, Sheffield, UK.
IIS-Fundacion Jimenez Diaz-UAM and REDINREN, Madrid, Spain.
Pediatric Nephrology Division, 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 REDINREN, Barcelona, Spain.
Renal Division, Ghent University Hospital, Ghent, Belgium.
Department of Nephrology, Transplantation and Internal Medicine, Medical University of Silesia in Katowice, Katowice, Poland.
CNR-IFC Clinical Epidemiology and Pathophysiology of Renal Diseases and Hypertension Unit, Reggio Calabria c/o Ospedali Riuniti, Reggio Calabria, Italy.


Recently, the European Medicines Agency approved the use of the vasopressin V2 receptor antagonist tolvaptan to slow the progression of cyst development and renal insufficiency of autosomal dominant polycystic kidney disease (ADPKD) in adult patients with chronic kidney disease stages 1-3 at initiation of treatment with evidence of rapidly progressing disease. In this paper, on behalf of the ERA-EDTA Working Groups of Inherited Kidney Disorders and European Renal Best Practice, we aim to provide guidance for making the decision as to which ADPKD patients to treat with tolvaptan. The present position statement includes a series of recommendations resulting in a hierarchical decision algorithm that encompasses a sequence of risk-factor assessments in a descending order of reliability. By examining the best-validated markers first, we aim to identify ADPKD patients who have documented rapid disease progression or are likely to have rapid disease progression. We believe that this procedure offers the best opportunity to select patients who are most likely to benefit from tolvaptan, thus improving the benefit-to-risk ratio and cost-effectiveness of this treatment. It is important to emphasize that the decision to initiate treatment requires the consideration of many factors besides eligibility, such as contraindications, potential adverse events, as well as patient motivation and lifestyle factors, and requires shared decision-making with the patient.


ADPKD; tolvaptan; vasopressin V2 receptor antagonist

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