Calciphylaxis: Emerging Concepts in Prevention, Diagnosis, and Treatment

Authors

  • William A Wilmer,

    1. * Division of Nephrology, Department of Internal Medicine and Division of Dermatopathology, Department of Pathology, Ohio State University College of Medicine and Public Health, Columbus, Ohio
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  • Cynthia M Magro

    1. * Division of Nephrology, Department of Internal Medicine and Division of Dermatopathology, Department of Pathology, Ohio State University College of Medicine and Public Health, Columbus, Ohio
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Address correspondence to: William A. Wilmer, MD, N210 Means Hall, 1654 Upham Dr., Columbus, OH 43210, or e-mail: wilmer.1@osu.edu.

Abstract

Calciphylaxis is a small vessel vasculopathy involving mural calcification with intimal proliferation, fibrosis, and thrombosis. This syndrome occurs predominantly in individuals with renal failure and results in ischemia and necrosis of skin, subcutaneous fat, visceral organs, and skeletal muscle. The syndrome causes significant morbidity in the form of infection, organ failure, and pain. Mortality rates are high. In individuals with renal failure, risk factors for the development of calciphylaxis include female sex, Caucasian race, obesity, and diabetes mellitus. Many cases occur within the first year of dialysis treatment. Several recent reports demonstrate that prolonged hyperphosphatemia and/or elevated calcium × phosphorus products are associated with the syndrome. Protein malnutrition increases the likelihood of calciphylaxis, as does warfarin use and hypercoagulable states, such as protein C and/or protein S deficiency. Recent advances in diagnostic tools and therapeutic strategies have helped in the management of patients with calciphylaxis.

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