title: Calciphylaxis: A Disease of Pannicular Thrombosis.
abstract: OBJECTIVE
  To identify coagulation risk factors in patients with calciphylaxis and the relationship between anticoagulation use and overall survival.
  
  
  METHODS
  Study subjects were 101 patients with calciphylaxis seen at Mayo Clinic from 1999 to September 2014. Data including thrombophilia profiles were extracted from the medical records of each patient. Survival status was determined using patient registration data and the Social Security Death Index. Survival was estimated using the Kaplan-Meier method, and associations were evaluated using Cox proportional hazards models.
  
  
  RESULTS
  Sixty-four of the 101 patients underwent thrombophilia testing. Of these, a complete test panel was performed in 55 and a partial panel in 9. Severe thrombophilias observed in 60% (33 of 55) of the patients included antiphospholipid antibody syndrome protein C, protein S, or antithrombin deficiencies or combined thrombophilias. Of the 55 patients, severe thrombophilia (85%, 23 of 27) was noted in patients who were not on warfarin at the time of testing (27). Nonsevere thrombophilias included heterozygous factor V Leiden (n=2) and plasminogen deficiency (n=1). For the comparison of survival, patients were divided into 3 treatment categories: Warfarin (n=63), other anticoagulants (n=20), and no anticoagulants (n=18). There was no statistically significant survival difference between treatment groups.
  
  
  CONCLUSIONS
  Laboratory testing reveals a strikingly high prevalence of severe thrombophilias in patients with calciphylaxis, underscoring the importance of congenital and acquired thrombotic propensity potentially contributing to the pathogenesis of this disease. These findings may have therapeutic implications; however, to date, survival differences did not vary by therapeutic choice.
authors.affiliation: Department of Dermatology, Mayo Clinic, Rochester, MN. Electronic address: el-Azhary.rokea2@mayo.edu.
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 Medicine, Division of Cardiovascular Disease, Mayo Clinic, Rochester, MN.
authors.forename: Robert D
authors.initials: RD
authors.lastname: McBane
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 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
authors.affiliation: Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN.
authors.forename: James T
authors.initials: JT
authors.lastname: McCarthy
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000150:complications; D002115:Calciphylaxis / Q000150:complications* / Q000401:mortality; D005165:Factor V / Q000235:genetics; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D005340:Fibrinogen / Q000032:analysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation; D010958:Plasminogen / Q000172:deficiency; D019851:Thrombophilia / Q000150:complications*; D014859:Warfarin / Q000627:therapeutic use
chemical_list: D000925:Anticoagulants; D005338:Fibrin Fibrinogen Degradation Products; C036309:fibrin fragment D; D014859:Warfarin; D005165:Factor V; D005340:Fibrinogen; D010958:Plasminogen