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. (P=0.003). Fractional uric acid excretion strongly correlated to fractional glucose excretion (r=0.35; P=0.02)
This article was prepared on behalf of the WGIKD, which is an official body of the ERA and the Working Group on
Autosomal Dominant Disorders of the ERKNet by members of these parties (WGIKD: G.C., E.C.-L.G., A.v.E., E.J.H., N.K., R.-U.M., T.N., J.S., A.S. and S.B.W.; ERKNet: O.D., D.M. and
F.S.) in cooperation with a number of external experts in the field of ADPKD (H.B., R.G., A.L.M., Y.L.M., A.O. and R.T.)
and PKDInternational (P.G., T.H. and U.K.). ERKNet is cofunded by the European Union within the framework of the
Third Health Programme ‘ERN-2016–Framework Partnership Agreement 2017–2021’. R.-U.M. is supported by the German
Research Foundation and the PKD Foundation.
© 2021 The Author(s) 2021. Published by Oxford University Press on behalf of the ERA.