BACKGROUND: The role of a hypercoagulable state in the pathogenesis of calciphylaxis has yet to be determined. OBJECTIVE: We sought to find evidence of an association between hypercoagulability and calciphylaxis. METHODS: We reviewed the primary literature for review articles, studies, and case reports that discussed or demonstrated a possible relationship between calciphylaxis and a hypercoagulable state. RESULTS: Review of the primary literature showed that in cases of calciphylaxis with reported levels of protein C and S, 38% of the patients had decreased protein C levels and 43% had decreased levels of protein S. From review of case reports, 3 cases of improvement of skin lesions with low molecular weight heparin treatment, and a fourth case of healing of skin lesions with tissue plasminogen activator treatment, were found. Calciphylaxis was also found in a patient with antiphospholipid antibody syndrome, and a patient with cryofibrinogenemia had clinical and histologic findings consistent with possible calciphylaxis. LIMITATIONS: A limited number of reports were available for review. CONCLUSION: Our review of the literature found sufficient evidence to suggest a possible role of a hypercoagulable state in the pathogenesis of calciphylaxis. A prospective study with serial testing of all relevant clotting factors in patients with calciphylaxis is needed to more definitively establish this role. Copyright Â
BACKGROUND: The role of a hypercoagulable state in the pathogenesis of calciphylaxis has yet to be determined. OBJECTIVE: We sought to find evidence of an association between hypercoagulability and calciphylaxis. METHODS: We reviewed the primary literature for review articles, studies, and case reports that discussed or demonstrated a possible relationship between calciphylaxis and a hypercoagulable state. RESULTS: Review of the primary literature showed that in cases of calciphylaxis with reported levels of protein C and S, 38% of the patients had decreased protein C levels and 43% had decreased levels of protein S. From review of case reports, 3 cases of improvement of skin lesions with low molecular weight heparin treatment, and a fourth case of healing of skin lesions with tissue plasminogen activator treatment, were found. Calciphylaxis was also found in a patient with antiphospholipid antibody syndrome, and a patient with cryofibrinogenemia had clinical and histologic findings consistent with possible calciphylaxis. LIMITATIONS: A limited number of reports were available for review. CONCLUSION: Our review of the literature found sufficient evidence to suggest a possible role of a hypercoagulable state in the pathogenesis of calciphylaxis. A prospective study with serial testing of all relevant clotting factors in patients with calciphylaxis is needed to more definitively establish this role. Copyright Â
Authors: Sagar U Nigwekar; Daniela Kroshinsky; Rosalynn M Nazarian; Jeremy Goverman; Rajeev Malhotra; Vicki Ann Jackson; Mihir M Kamdar; David J R Steele; Ravi I Thadhani Journal: Am J Kidney Dis Date: 2015-05-07 Impact factor: 8.860