Autosomal dominant polycystic kidney disease: Clinical assessment of rapid progression

Mónica Furlano, Irene Loscos, Teresa Martí, Gemma Bullich, Nadia Ayasreh, Asunción Rius, Lourdes Roca, José Ballarín, Elisabet Ars, Roser Torra

Research output: Contribution to journalArticleResearch

5 Citations (Scopus)


© 2018 S. Karger AG, Basel. Background: Autosomal dominant polycystic kidney disease (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 algorithm from the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) Working Groups of Inherited Kidney Disorders and European Renal Best Practice (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 glomerular filtration rate (eGFR; ≥25 mL/min/1.73 m 2 ). Results: Historical 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. Overall, 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 identifying 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.
Original languageEnglish
Pages (from-to)308-317
JournalAmerican Journal of Nephrology
Publication statusPublished - 1 Nov 2018


  • Autosomal dominant polycystic kidney disease
  • Chronic kidney disease
  • Gene
  • Magnetic resonance imaging
  • Prediction
  • Rapid progression
  • Total kidney volume
  • Ultrasound


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