BUERGERS-DISEASE AND PROTEIN-S DEFICIENCY - SUCCESSFUL TREATMENT WITHPROSTACYCLIN

In this report we describe a female patient who presented with periphe ral circulatory impairment. She had previously been thought to have a systemic vasculitis, but had failed to respond to corticosteroid and i mmunosuppressive therapy. Serologic investigations were negative. The patient was a very heavy smoker, and it became clear that her conditio n had many of the clinical features of Buerger's disease (thrombangiit is obliterans). The diagnosis was confirmed by angiography, and a coag ulation screen showed her to have associated protein S deficiency. Her symptoms responded rapidly to cessation of smoking, warfarin therapy and a series of intravenous prostacyclin infusions. We discuss in deta il the possible pathologic mechanisms which might explain the associat ion of protein S deficiency and thrombangiitis obliterans.