Original Report: Patient-Oriented, Translational Research Am J Nephrol 2018;48:308–317 Autosomal Dominant Polycystic Kidney Disease: Clinical Assessment of Rapid Progression Mónica Furlano a Irene Loscos b Teresa Martí c Gemma Bullich d Nadia Ayasreh a Asunción Rius a, e Lourdes Roca a, f José Ballarín g Elisabet Ars d Roser Torra a a Department of Nephrology, Inherited Renal Disorders, Fundació Puigvert, REDINREN, IIB Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; b Universitat Pompeu Fabra-Universitat Autònoma de Barcelona, Barcelona, Spain; c Department of Radiology, Fundació Puigvert, Barcelona, Spain; d Molecular Biology Laboratory, Fundació Puigvert, Instituto de Investigaciones Biomédicas Sant Pau (IIB Sant Pau), Universitat Autònoma de Barcelona, REDinREN, Instituto de Investigación Carlos III, Barcelona, Spain; e Department of Nephrology, Hospital General Universitario de Castellón, Castellón, Spain; f Hospital Universitario y Politécnico de La Fe, Valencia, Spain; g Department of Nephrology, Fundació Puigvert, REDinREN, IIB Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain Received: May 24, 2018 Accepted: August 17, 2018 Published online: October 22, 2018 Nephrolo gy American Journal of Prof. Roser Torra Department of Nephrology, Inherited Renal Disorders Fundació Puigvert, Carrer de Cartagena 340-350, ES–08025 Barcelona (Spain) E-Mail rtorra @fundacio-puigvert.es © 2018 S. Karger AG, Basel E-Mail karger@karger.com www.karger.com/ajn DOI: 10.1159/000493325 Keywords Autosomal dominant polycystic kidney disease · Chronic kidney disease · Rapid progression · Prediction · Magnetic resonance imaging · Ultrasound · Gene · Total kidney volume Abstract Background: Autosomal dominant polycystic kidney dis- ease (ADPKD) causes the development of renal cysts and leads to a decline in renal function. Limited guidance exists in clinical practice on the use of tolvaptan. A decision algo- rithm from the European Renal Association-European Dialy- sis and Transplant Association (ERA-EDTA) Working Groups of Inherited Kidney Disorders and European Renal Best Prac- tice (WGIKD/ERBP) has been proposed to identify candidates for tolvaptan treatment; however, this algorithm has not been assessed in clinical practice. Methods: Eighteen-month cross-sectional, unicenter, observational study assessing 305 consecutive ADPKD patients. The ERA-EDTA WGIKD/ ERBP algorithm with a stepwise approach was used to assess rapid progression (RP). Subsequently, expanded criteria based on the REPRISE trial were applied to evaluate the impact of extended age (≤55 years) and estimated glomeru- lar filtration rate (eGFR; ≥25 mL/min/1.73 m 2 ). Results: His- torical eGFR decline, indicative of RP, was fulfilled in 26% of 73 patients who were candidates for RP assessment, mostly aged 31–55 years. Further tests including ultrasound and MRI measurements of kidney volume plus genetic testing enabled the evaluation of the remaining patients. Over- all, 15.7% of patients met the criteria for rapid or likely RP using the algorithm, and the percentage increased to 27% when extending age and eGFR. Conclusions: The ERA-EDTA WGIKD/ERBP algorithm provides a valuable means of identi- fying in routine clinical practice patients who may be eligible for treatment with tolvaptan. The impact of a new threshold for age and eGFR may increase the percentage of patients to be treated. © 2018 S. Karger AG, Basel E.A. and R.T. have equally contributed to this work.