title: Survival, Risk Factors, and Effect of Treatment in 101 Patients With Calciphylaxis.
abstract: OBJECTIVE
  To report on the survival and the associations of treatments upon survival of patients with calciphylaxis seen at a single center.
  
  
  METHODS
  Using the International Classification of Diseases, Ninth Revision diagnosis code of 275.49 and the keyword "calciphylaxis" in the dismissal narrative, we retrospectively identified 101 patients with calciphylaxis seen at our institution between January 1, 1999, through September 20, 2014, using a predefined, consensus-developed classification scheme.
  
  
  RESULTS
  The average age of patients was 60 years: 81 (80.2%) were women; 68 (68.0%) were obese; 19 (18.8%) had stage 0 to 2 chronic kidney disease (CKD), 19 (18.9%) had stage 3 or 4 CKD; 63 (62.4%) had stage 5 or 5D (dialysis) CKD. Seventy-five patients died during follow-up. Six-month survival was 57%. Lack of surgical debridement was associated with insignificantly lower 6-month survival (hazard ratio [HR]=1.99; 95% CI, 0.96-4.15; P=.07) and significantly poorer survival for the entire duration of follow-up (HR=1.98; 95% CI, 1.15-3.41; P=.01), which was most pronounced in stage 5 or 5D CKD (HR=1.91; 95% CI, 1.03-3.56; P=.04). Among patients with stage 5/5D CKD, subtotal parathyroidectomy (performed only in patients with hyperparathyroidism) was associated with better 6-month (HR=0.12; 95% CI, 0.02-0.90; P=.04) and overall survival (HR= 0.37; 95% CI, 0.15-0.87; P=.02).
  
  
  CONCLUSIONS
  Calciphylaxis is associated with a high mortality rate. Significantly effective treatments included surgical debridement and subtotal parathyroidectomy in patients with stage 5/5D CKD with hyperparathyroidism. Treatments with tissue-plasminogen activator, sodium thiosulfate, and hyperbaric oxygen therapy were not associated with higher mortality.
authors.affiliation: Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN. Electronic address: mccarthy.james@mayo.edu.
authors.forename: James T
authors.initials: JT
authors.lastname: McCarthy
authors.affiliation: Department of Dermatology, Mayo Clinic, Rochester, MN.
authors.forename: Rokea A
authors.initials: RA
authors.lastname: El-Azhary
authors.affiliation: Mayo Medical School, Mayo Graduate School of Medicine, Mayo Clinic, Rochester, MN.
authors.forename: Michelle T
authors.initials: MT
authors.lastname: Patzelt
authors.affiliation: Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN.
authors.forename: Amy L
authors.initials: AL
authors.lastname: Weaver
authors.affiliation: Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN.
authors.forename: Robert C
authors.initials: RC
authors.lastname: Albright
authors.affiliation: Department of Dermatology, Mayo Clinic, Rochester, MN.
authors.forename: Alina D
authors.initials: AD
authors.lastname: Bridges
authors.affiliation: Department of Medicine, Division of Hyperbaric Medicine in Preventive, Occupational and Aerospace Medicine, Mayo Clinic, Rochester, MN.
authors.forename: Paul L
authors.initials: PL
authors.lastname: Claus
authors.affiliation: Department of Dermatology, Mayo Clinic, Rochester, MN.
authors.forename: Mark D P
authors.initials: MD
authors.lastname: Davis
authors.affiliation: Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN.
authors.forename: John J
authors.initials: JJ
authors.lastname: Dillon
authors.affiliation: Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN.
authors.forename: Ziad M
authors.initials: ZM
authors.lastname: El-Zoghby
authors.affiliation: Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN.
authors.forename: LaTonya J
authors.initials: LJ
authors.lastname: Hickson
authors.affiliation: Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN.
authors.forename: Rajiv
authors.initials: R
authors.lastname: Kumar
authors.affiliation: Department of Cardiovascular Disease, Mayo Clinic, Rochester, MN.
authors.forename: Robert D
authors.initials: RD
authors.lastname: McBane
authors.affiliation: Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN.
authors.forename: Kathleen A M
authors.initials: KA
authors.lastname: McCarthy-Fruin
authors.affiliation: Department of Dermatology, Mayo Clinic, Rochester, MN.
authors.forename: Marian T
authors.initials: MT
authors.lastname: McEvoy
authors.affiliation: Department of Dermatology, Mayo Clinic, Rochester, MN.
authors.forename: Mark R
authors.initials: MR
authors.lastname: Pittelkow
authors.affiliation: Department of Dermatology, Mayo Clinic, Rochester, MN.
authors.forename: David A
authors.initials: DA
authors.lastname: Wetter
authors.affiliation: Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN.
authors.forename: Amy W
authors.initials: AW
authors.lastname: Williams
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002115:Calciphylaxis / Q000150:complications / Q000401:mortality* / Q000628:therapy*; D003646:Debridement; D003920:Diabetes Mellitus; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D006931:Hyperbaric Oxygenation; D006961:Hyperparathyroidism / Q000209:etiology / Q000601:surgery; D006973:Hypertension / Q000150:complications; D008297:Male; D008875:Middle Aged; D008910:Minnesota / Q000453:epidemiology; D009369:Neoplasms / Q000150:complications; D009765:Obesity / Q000150:complications; D016105:Parathyroidectomy; D051436:Renal Insufficiency, Chronic / Q000145:classification / Q000150:complications; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D013885:Thiosulfates / Q000627:therapeutic use; D010959:Tissue Plasminogen Activator / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D013885:Thiosulfates; D010959:Tissue Plasminogen Activator; C017717:sodium thiosulfate