title: Calciphylaxis: Beyond CKD-MBD.
abstract: BACKGROUND
  Calcific uraemic arteriolopathy (CUA), also called calciphylaxis, is a rare but potentially fatal vascular disorder that almost exclusively affects patients with chronic renal failure. The objective of this study was to analyse various risk factors for developing CUA and its subsequent clinical course according to the treatment received.
  
  
  METHODS
  A retrospective study that included patients diagnosed with CUA from December 1999 to December 2015. Various risk factors, clinical course and treatment options were analysed.
  
  
  RESULTS
  A total of 28 patients (53.6% females) with a mean age of 67.2±11.8 (38-88) years were included. At the time of diagnosis, 53.6% were on haemodialysis, 25% were kidney transplant patients and 21.4% had normal renal function. The use of steroids (100%, P=.001) was the main risk factor in renal transplant patients. Skin lesions resolved in 60.7% (especially in those receiving multitargeted therapy). Patient survival at 12 months was 29% in transplant patients, 57% in haemodialysis patients and 100% in normal renal function patients (log-rank 6.88, P=.032). Chronic renal failure (P=.03) and hypoalbuminaemia (P=.02) were the main risk factor for CUA mortality.
  
  
  CONCLUSIONS
  Although the incidence of CUA remains low, CUA mortality is very high, Special attention to its occurrence in kidney transplant patients and «non-renal» CUA forms is required. Oral anticoagulants and steroids appear to be the main risk factors, CUA is a challenge; a registry of patients and determining standard therapy are required.
authors.affiliation: Servicio de Nefrología, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: María
authors.initials: M
authors.lastname: Fernández
authors.affiliation: Servicio de Nefrología, Hospital Universitario 12 de Octubre, Madrid, España. Electronic address: emoralesr@senefro.org.
authors.forename: Enrique
authors.initials: E
authors.lastname: Morales
authors.affiliation: Servicio de Nefrología, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Gutierrez
authors.affiliation: Servicio de Nefrología, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Natalia
authors.initials: N
authors.lastname: Polanco
authors.affiliation: Servicio de Nefrología, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Hernández
authors.affiliation: Servicio de Nefrología, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Eva
authors.initials: E
authors.lastname: Mérida
authors.affiliation: Servicio de Nefrología, Hospital Universitario 12 de Octubre, Madrid, España.
authors.forename: Manuel
authors.initials: M
authors.lastname: Praga
mesh_terms: D000305:Adrenal Cortex Hormones / Q000009:adverse effects; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000671:Amputation, Surgical; D002115:Calciphylaxis / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D015913:Chelation Therapy; D000069449:Cinacalcet / Q000627:therapeutic use; D003131:Combined Modality Therapy; D004164:Diphosphonates / Q000627:therapeutic use; D005260:Female; D006801:Humans; D034141:Hypoalbuminemia / Q000150:complications; D053208:Kaplan-Meier Estimate; D007676:Kidney Failure, Chronic / Q000150:complications / Q000601:surgery / Q000628:therapy; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D010431:Pentoxifylline / Q000627:therapeutic use; D011183:Postoperative Complications / Q000208:ethnology / Q000209:etiology; D006435:Renal Dialysis; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D004164:Diphosphonates; D010431:Pentoxifylline; D000069449:Cinacalcet
keywords: Anticoagulantes orales; Calcifilaxis; Calciphylaxis; Chronic renal failure; Formas no renales; Insuficiencia renal crónica; Mortalidad; Mortality; Non-renal forms; Oral anticoagulants