title: Calcific uraemic arteriolopathy (calciphylaxis): data from a large nationwide registry.
abstract: Calcific uraemic arteriolopathy (CUA, calciphylaxis) is a rare disease predominantly in dialysis patients and associated with high mortality. Painful skin ulcerations and calcification of cutaneous arterioles characterize calciphylaxis.
  
  
  
  We established an observational, Internet-based registry allowing online notification for all German CUA cases. The registry recorded data about patient characteristics, biochemistry and therapies. Blood samples were stored in a central biobank.
  
  
  
  Between 2006 and 2015, 253 CUA patients were recorded: median age 70 [interquartile range (IQR) 61-76] years, 60% females and 86% ( n = 207) dialysis patients, translating into an estimated annual incidence rate of 0.04% in German dialysis patients. Fifty-two per cent received vitamin K antagonists (VKAs) prior to CUA. Skin lesions were localized in 71% on the legs or gluteal region. In dialysis CUA patients median total serum calcium was 2.20 (IQR 2.06-2.37) mmol/L, phosphorus 1.67 (IQR 1.35-2.03) mmol/L, intact parathyroid hormone 147 (IQR 72-276) pg/mL and fetuin-A 0.21 (IQR 0.16-0.26) g/L (normal range 0.35-0.95). Median sclerostin, osteoprotegerin, TRAP5b, bone-specific alkaline phosphatase and c-terminal FGF23 levels were all elevated. The most frequently recorded therapeutic procedures in dialysis CUA patients were as follows: wound debridement (29% of cases), stopping VKA (25%), lowering calcium supply (24%), sodium thiosulphate (22%), application of vitamin K (18%), increase of dialysis duration/frequency (17%) and stoping active vitamin D (16%).
  
  
  
  Approximately 50% of CUA patients used VKA. Our data suggest that uncontrolled hyperparathyroidism is not the key determinant of calciphylaxis. Therapeutic strategies were heterogeneous. The experience of the German registry will help substantially to initiate a large-scale multinational CUA registry.
authors.affiliation: Department of Cardiology, University Hospital of the RWTH Aachen, Aachen, Germany.|Center for Rare Diseases, University Hospital of the RWTH Aachen (ZSEA), Aachen, Germany.
authors.forename: Vincent M
authors.initials: VM
authors.lastname: Brandenburg
authors.affiliation: Center for Rare Diseases, University Hospital of the RWTH Aachen (ZSEA), Aachen, Germany.|Department of Nephrology, University Hospital of the RWTH Aachen, Aachen, Germany.
authors.forename: Rafael
authors.initials: R
authors.lastname: Kramann
authors.affiliation: Department of Nephrology, Klinikum Coburg, Coburg, Germany.
authors.forename: Hansjörg
authors.initials: H
authors.lastname: Rothe
authors.affiliation: Department of Nephrology, University Hospital of the RWTH Aachen, Aachen, Germany.
authors.forename: Nadine
authors.initials: N
authors.lastname: Kaesler
authors.affiliation: Clinical Trial Center-Aachen, RWTH Aachen, Aachen, Germany.
authors.forename: Joanna
authors.initials: J
authors.lastname: Korbiel
authors.affiliation: Department of Cardiology, University Hospital of the RWTH Aachen, Aachen, Germany.
authors.forename: Paula
authors.initials: P
authors.lastname: Specht
authors.affiliation: Department of General, Visceral and Transplantation Surgery, University Hospital of the RWTH Aachen, Aachen, Germany.
authors.forename: Sophia
authors.initials: S
authors.lastname: Schmitz
authors.affiliation: Department of Nephrology, University Hospital of the RWTH Aachen, Aachen, Germany.
authors.forename: Thilo
authors.initials: T
authors.lastname: Krüger
authors.affiliation: Center for Rare Diseases, University Hospital of the RWTH Aachen (ZSEA), Aachen, Germany.|Department of Nephrology, University Hospital of the RWTH Aachen, Aachen, Germany.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Floege
authors.affiliation: Department of Nephrology, Klinikum Coburg, Coburg, Germany.
authors.forename: Markus
authors.initials: M
authors.lastname: Ketteler
mesh_terms: D000368:Aged; D002115:Calciphylaxis / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D005260:Female; D000089703:Fibroblast Growth Factor-23; D006801:Humans; D007676:Kidney Failure, Chronic / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D012042:Registries / Q000706:statistics & numerical data*; D006435:Renal Dialysis / Q000009:adverse effects*; D014812:Vitamin K / Q000037:antagonists & inhibitors*
chemical_list: C000717427:FGF23 protein, human; D014812:Vitamin K; D000089703:Fibroblast Growth Factor-23
keywords: ESRD; anticoagulation; calcification; calciphylaxis; cardiovascular