Abstract: FR-PO317

Assessing Rapid ADPKD Progression in Clinical Practice in the Era of Tolvaptan

Session Information

Category: Genetic Diseases of the Kidney

  • 801 Cystic Kidney Diseases


  • Furlano, Monica, Fundacio Puigvert, Barcelona, Spain
  • Marti, Teresa, Fundació Puigvert, Barcelona, Spain
  • Loscos giménez, Irene, Fundacio Puigvert, Barcelona, Spain
  • Bullich vilanova, Gemma, Fundacio Puigvert, Barcelona, Spain
  • Ballarin, Jose, Fundacio Puigvert, Barcelona, Spain
  • Ars, Elisabet, Fundacio Puigvert, Barcelona, Spain
  • Torra, Roser, Fundacio Puigvert, Barcelona, Spain

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent inherited kidney disease. The progressive cyst growth, together with interstitial damage causes progressive kidney failure but the severity of the diseases varies a lot among affected individuals. The EMA has approved tolvaptan for adults with CKD stage 1-3 at baseline who are rapid progressors (RP). Being the mean age in Europe of onset of ESRD for ADPKD patients 58 years, the ERA-EDTA WGKID/EBPG recommended to consider RP those patients predicted to reach ESRD before 58 years of age.


297 ADPKD patients followed up in an outpatient clinic for inherited kidney diseases were assessed for rapid progression according to the EDTA-ERA WGIKD/EBPG recommendations. Only patients between 18-50 years of age were considered. Assessment was only indicated when eGFR was over 45ml/min and eGFR for patients 30-40 years old was below 90ml/min and for those 40-50 years old was below 60 ml/min. If the patients met these eGFR according to age, retrospective eGFR decline was assessed; if it was > 5 ml/year or >2.5 ml/year for 3 consecutive years patients were considered RP. For those who didn’t met the retrospective eGFR criteria ultrasound (US) diameter was assessed. Patients younger than 45 with a renal diameter >16.5 cm were considered RP. For those not fulfilling any of the above criteria total kidney volume (TKV) by MRI was measured and the Mayo ADPKD calculator was applied. Patients class 1C,D,E were considered RP. Finally for patients younger than 35 with hypertension or urinary symptoms than did not met the above criteria genetic testing was performed and the PROPKD score was applied.


The step by step process of RP assessment based on the EDTA-ERA/EBPG recommendations proved to be cost-effective and sensitive to identify RP.
RP was identified in 16.5% of patients with CKD stage 1, 29% in CKD2 and 34.3% in CKD3a.
53.8% of patients aged 18-30 were rapid progressors while this number decreased with age: 30.9% from 31-40 years and 13.5% from 41-50 years.


The multi step algorithm provided by the EDTA-ERA/EBPG is useful to identify RP that would benefit from tolvaptan treatment. The use of the algorithm is cost-effective and fairly easy to incorporate into clinical practice.


  • Government Support - Non-U.S.