Buerger's disease and protein S deficiency: successful treatment with prostacyclin.
In this report we describe a female patient who presented with peripheral circulatory impairment. She had previously been thought to have a systemic vasculitis, but had failed to respond to corticosteroid and immunosuppressive therapy. Serologic investigations were negative. The patient was a very heavy smoker, and it became clear that her condition had many of the clinical features of Buerger's disease (thrombangiitis obliterans). The diagnosis was confirmed by angiography, and a coagulation 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 detail the possible pathologic mechanisms which might explain the association of protein S deficiency and thrombangiitis obliterans.
Athanassiou
P
P
Department of Medicine, Hammersmith Hospital, London, UK.
McHale
J
J
Dikeou
S
S
Laffan
M
M
Dantis
P
P
Davies
K A
KA
D000328:Adult; D011464:Epoprostenol / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D018455:Protein S Deficiency / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D013919:Thromboangiitis Obliterans / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy
D010975:Platelet Aggregation Inhibitors; D011464:Epoprostenol