title: Renal replacement therapy for autosomal dominant polycystic kidney disease (ADPKD) in Europe: prevalence and survival--an analysis of data from the ERA-EDTA Registry.
abstract: BACKGROUND
  Autosomal dominant polycystic kidney disease (ADPKD) is the fourth most common renal disease requiring renal replacement therapy (RRT). Still, there are few epidemiological data on the prevalence of, and survival on RRT for ADPKD.
  
  
  METHODS
  This study used data from the ERA-EDTA Registry on RRT prevalence and survival on RRT in 12 European countries with 208 million inhabitants. We studied four 5-year periods (1991-2010). Survival analysis was performed by the Kaplan-Meier method and by Cox proportional hazards regression.
  
  
  RESULTS
  From the first to the last study period, the prevalence of RRT for ADPKD increased from 56.8 to 91.1 per million population (pmp). The percentage of prevalent RRT patients with ADPKD remained fairly stable at 9.8%. Two-year survival of ADPKD patients on RRT (adjusted for age, sex and country) increased significantly from 89.0 to 92.8%, and was higher than for non-ADPKD subjects. Improved survival was noted for all RRT modalities: haemodialysis [adjusted hazard ratio for mortality during the last versus first time period 0.75 (95% confidence interval 0.61-0.91), peritoneal dialysis 0.55 (0.38-0.80) and transplantation 0.52 (0.32-0.74)]. Cardiovascular mortality as a proportion of total mortality on RRT decreased more in ADPKD patients (from 53 to 29%), than in non-ADPKD patients (from 44 to 35%). Of note, the incidence rate of RRT for ADPKD remained relatively stable at 7.6 versus 8.3 pmp from the first to the last study period, which will be discussed in detail in a separate study.
  
  
  CONCLUSIONS
  In ADPKD patients on RRT, survival has improved markedly, especially due to a decrease in cardiovascular mortality. This has led to a considerable increase in the number of ADPKD patients being treated with RRT.
authors.affiliation: Department of Nephrology, University Medical Center Groningen (UMCG), University of Groningen, Groningen, the Netherlands.
authors.forename: Edwin M
authors.initials: EM
authors.lastname: Spithoven
authors.affiliation: ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.
authors.forename: Anneke
authors.initials: A
authors.lastname: Kramer
authors.affiliation: Department of Nephrology, University Medical Center Groningen (UMCG), University of Groningen, Groningen, the Netherlands.
authors.forename: Esther
authors.initials: E
authors.lastname: Meijer
authors.affiliation: Division of Nephrology, Copenhagen University Hospital, Roskilde, Denmark.
authors.forename: Bjarne
authors.initials: B
authors.lastname: Orskov
authors.affiliation: Division of Nephrology, University Clinic, University of Würzburg, Würzburg, Germany.
authors.forename: Christoph
authors.initials: C
authors.lastname: Wanner
authors.affiliation: Departamento de Medicina Preventiva y Salud Pública, Universidad de Zaragoza, Zaragoza, Spain.
authors.forename: Jose M
authors.initials: JM
authors.lastname: Abad
authors.affiliation: Department of Nephrology, University Hospital Virgen Macarena, Seville, Spain.
authors.forename: Nuria
authors.initials: N
authors.lastname: Aresté
authors.affiliation: Public Health Directorate, Asturias, Spain.
authors.forename: Ramón Alonso
authors.initials: RA
authors.lastname: de la Torre
authors.affiliation: Richard Bright Renal Unit, Bristol, UK.
authors.forename: Fergus
authors.initials: F
authors.lastname: Caskey
authors.affiliation: REIN Registry, Agence de la Biomedecine, Saint Denis La Plaine, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Couchoud
authors.affiliation: Finnish Registry of Kidney Diseases, Helsinki, Finland.
authors.forename: Patrik
authors.initials: P
authors.lastname: Finne
authors.affiliation: Department of Nephrology, University of Copenhagen, Herlev Hospital, Herlev, Denmark.
authors.forename: James
authors.initials: J
authors.lastname: Heaf
authors.affiliation: Department of Nephrology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
authors.forename: Andries
authors.initials: A
authors.lastname: Hoitsma
authors.affiliation: Department of Nephrology, Dialysis and Hypertension, Nederlandstalige Belgische Vereniging voor Nefrologie (Dutch Speaking Belgium Renal Registry)-NBVN, AZ Nikolaas, Sint-Niklaas, Belgium.
authors.forename: Johan
authors.initials: J
authors.lastname: de Meester
authors.affiliation: Department of Nephrology, Hospital del Mar, Barcelona, Spain.
authors.forename: Julio
authors.initials: J
authors.lastname: Pascual
authors.affiliation: Clinical Epidemiology and Physiopathology of Renal Diseases and Hypertension, U.O.C. Nefrologia, Dialisi e Trapianto, Azienda Ospedaliera di Reggio Calabria and CNR-IBIM, Reggio Calabria, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Postorino
authors.affiliation: Department of Medicine and Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Pietro
authors.initials: P
authors.lastname: Ravani
authors.affiliation: Subirección General de Epidemiología y Vigilancia de la Salud, Conselleria de Sanitat, Generalitat C. Valenciana, Valencia, Spain Spanish Consortium of Epidemiology and Public Health Research (CIBERESP), Spain.
authors.forename: Oscar
authors.initials: O
authors.lastname: Zurriaga
authors.affiliation: ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.
authors.forename: Kitty J
authors.initials: KJ
authors.lastname: Jager
authors.affiliation: Department of Nephrology, University Medical Center Groningen (UMCG), University of Groningen, Groningen, the Netherlands.
authors.forename: Ron T
authors.initials: RT
authors.lastname: Gansevoort
mesh_terms: D000368:Aged; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016891:Polycystic Kidney, Autosomal Dominant / Q000401:mortality* / Q000628:therapy; D015995:Prevalence; D012042:Registries / Q000706:statistics & numerical data; D006435:Renal Dialysis / Q000401:mortality; D017582:Renal Replacement Therapy / Q000401:mortality*; D015996:Survival Rate; D013997:Time Factors
keywords: ADPKD; epidemiology; prevalence; renal replacement therapy; survival