Calciphylaxis: A Disease of Pannicular Thrombosis

Rokea A. el-Azhary, Michelle T. Patzelt, Robert D. McBane, Amy L. Weaver, Robert C. Albright, Alina D. Bridges, Paul L. Claus, Mark D P Davis, John J. Dillon, Ziad M El-Zoghby, LaTonya Hickson, Rajiv Kumar, Kathleen A M McCarthy-Fruin, Marian T. McEvoy, Mark R. Pittelkow, David A. Wetter, Amy W. Williams, James T. McCarthy

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Objective To identify coagulation risk factors in patients with calciphylaxis and the relationship between anticoagulation use and overall survival. Patients and 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. Conclusion 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.

Original languageEnglish (US)
Pages (from-to)1395-1402
Number of pages8
JournalMayo Clinic Proceedings
Volume91
Issue number10
DOIs
StatePublished - Oct 1 2016

Fingerprint

Calciphylaxis
Thrombosis
Thrombophilia
Survival
Warfarin
Anticoagulants
Computer Security
Blood Coagulation Factors
Antiphospholipid Syndrome
Antithrombins
Social Security
Plasminogen
Protein S
Therapeutics
Protein C
Proportional Hazards Models
Medical Records

ASJC Scopus subject areas

  • Medicine(all)

Cite this

el-Azhary, R. A., Patzelt, M. T., McBane, R. D., Weaver, A. L., Albright, R. C., Bridges, A. D., ... McCarthy, J. T. (2016). Calciphylaxis: A Disease of Pannicular Thrombosis. Mayo Clinic Proceedings, 91(10), 1395-1402. https://doi.org/10.1016/j.mayocp.2016.06.026

Calciphylaxis : A Disease of Pannicular Thrombosis. / el-Azhary, Rokea A.; Patzelt, Michelle T.; McBane, Robert D.; Weaver, Amy L.; Albright, Robert C.; Bridges, Alina D.; Claus, Paul L.; Davis, Mark D P; Dillon, John J.; El-Zoghby, Ziad M; Hickson, LaTonya; Kumar, Rajiv; McCarthy-Fruin, Kathleen A M; McEvoy, Marian T.; Pittelkow, Mark R.; Wetter, David A.; Williams, Amy W.; McCarthy, James T.

In: Mayo Clinic Proceedings, Vol. 91, No. 10, 01.10.2016, p. 1395-1402.

Research output: Contribution to journalArticle

el-Azhary, RA, Patzelt, MT, McBane, RD, Weaver, AL, Albright, RC, Bridges, AD, Claus, PL, Davis, MDP, Dillon, JJ, El-Zoghby, ZM, Hickson, L, Kumar, R, McCarthy-Fruin, KAM, McEvoy, MT, Pittelkow, MR, Wetter, DA, Williams, AW & McCarthy, JT 2016, 'Calciphylaxis: A Disease of Pannicular Thrombosis', Mayo Clinic Proceedings, vol. 91, no. 10, pp. 1395-1402. https://doi.org/10.1016/j.mayocp.2016.06.026
el-Azhary RA, Patzelt MT, McBane RD, Weaver AL, Albright RC, Bridges AD et al. Calciphylaxis: A Disease of Pannicular Thrombosis. Mayo Clinic Proceedings. 2016 Oct 1;91(10):1395-1402. https://doi.org/10.1016/j.mayocp.2016.06.026
el-Azhary, Rokea A. ; Patzelt, Michelle T. ; McBane, Robert D. ; Weaver, Amy L. ; Albright, Robert C. ; Bridges, Alina D. ; Claus, Paul L. ; Davis, Mark D P ; Dillon, John J. ; El-Zoghby, Ziad M ; Hickson, LaTonya ; Kumar, Rajiv ; McCarthy-Fruin, Kathleen A M ; McEvoy, Marian T. ; Pittelkow, Mark R. ; Wetter, David A. ; Williams, Amy W. ; McCarthy, James T. / Calciphylaxis : A Disease of Pannicular Thrombosis. In: Mayo Clinic Proceedings. 2016 ; Vol. 91, No. 10. pp. 1395-1402.
@article{65ff10b65f954fe8ad5cff48c0b335b3,
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. Patients and 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. Conclusion 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.",
author = "el-Azhary, {Rokea A.} and Patzelt, {Michelle T.} and McBane, {Robert D.} and Weaver, {Amy L.} and Albright, {Robert C.} and Bridges, {Alina D.} and Claus, {Paul L.} and Davis, {Mark D P} and Dillon, {John J.} and El-Zoghby, {Ziad M} and LaTonya Hickson and Rajiv Kumar and McCarthy-Fruin, {Kathleen A M} and McEvoy, {Marian T.} and Pittelkow, {Mark R.} and Wetter, {David A.} and Williams, {Amy W.} and McCarthy, {James T.}",
year = "2016",
month = "10",
day = "1",
doi = "10.1016/j.mayocp.2016.06.026",
language = "English (US)",
volume = "91",
pages = "1395--1402",
journal = "Mayo Clinic Proceedings",
issn = "0025-6196",
publisher = "Elsevier Science",
number = "10",

}

TY - JOUR

T1 - Calciphylaxis

T2 - A Disease of Pannicular Thrombosis

AU - el-Azhary, Rokea A.

AU - Patzelt, Michelle T.

AU - McBane, Robert D.

AU - Weaver, Amy L.

AU - Albright, Robert C.

AU - Bridges, Alina D.

AU - Claus, Paul L.

AU - Davis, Mark D P

AU - Dillon, John J.

AU - El-Zoghby, Ziad M

AU - Hickson, LaTonya

AU - Kumar, Rajiv

AU - McCarthy-Fruin, Kathleen A M

AU - McEvoy, Marian T.

AU - Pittelkow, Mark R.

AU - Wetter, David A.

AU - Williams, Amy W.

AU - McCarthy, James T.

PY - 2016/10/1

Y1 - 2016/10/1

N2 - Objective To identify coagulation risk factors in patients with calciphylaxis and the relationship between anticoagulation use and overall survival. Patients and 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. Conclusion 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.

AB - Objective To identify coagulation risk factors in patients with calciphylaxis and the relationship between anticoagulation use and overall survival. Patients and 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. Conclusion 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.

UR - http://www.scopus.com/inward/record.url?scp=84994217975&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84994217975&partnerID=8YFLogxK

U2 - 10.1016/j.mayocp.2016.06.026

DO - 10.1016/j.mayocp.2016.06.026

M3 - Article

C2 - 27712638

AN - SCOPUS:84994217975

VL - 91

SP - 1395

EP - 1402

JO - Mayo Clinic Proceedings

JF - Mayo Clinic Proceedings

SN - 0025-6196

IS - 10

ER -