Calciphylaxis induced by warfarin therapy in a patient with anti-phospholipid antibody syndrome associated with systemic lupus erythematosus

Abstract

Calciphylaxis is a life-threatening complication of end-stage kidney disease (ESKD) and leads to cutaneous necrosis and gangrene. Various risk factors for calciphylaxis have been reported, and warfarin therapy is a particularly strong trigger. Here we report the case of 50-yearold woman with ESKD and systemic lupus erythematosus who developed calciphylaxis after anti-thrombotic therapy, including warfarin, for ischemic skin ulcers due to arteriosclerosis obliterans and anti-phospholipid antibody syndrome. Although warfarin improved the thrombotic skin ulcers, it might also be a trigger for calciphylaxis. Discontinuation of the warfarin and the addition of lowdensity lipoprotein apheresis and sodium thiosulfate infusion failed to improve the gangrene; eventually, her legs had to be amputated to prevent lethal infection. The histology of the dermal and soft tissue obtained from the amputated legs showed typical findings of calciphylaxis. Warfarin is a vitamin K antagonist with inhibitory effects on the calcification of regulatory proteins, such as matrix Gla protein and fetuin-A. Therefore, the warfarin therapy might have induced calciphylaxis in our patient.

Efficacy and Safety of Sodium Thiosulfate in Japanese Patients with Calciphylaxis

“…On the other hand, relatively few case reports have described the effect of STS for calciphylaxis in Japanese patients [8][9][10][11][12][13] . STS was effective in all of the previously reported Japanese patients who were undergoing dialysis.…”

“…Conversely, Tokashiki et al and Ishikawa et al administered 20 and 5-25 g/day, respectively 9,13) . The treatment was discontinued in the latter due to side effects, such as metabolic acidosis, anorexia, and nausea 12) .…”