Etiologic aspects of coagulopathy in Takayasu's aortitis

Sixteen patients with Takayasu's aortitis were studied to evaluate the significance of coagulopathy in the etiology. In the initial stage of the disease (three cases) characterized by severe inflammatory symptoms with aortic lesions localized in the thoracic portion, marked hyperfibrinogenemia, and hypofibrinolytic activity of the plasma were observed. In the intermediate stage (five cases) characterized by moderate inflammation and aortic lesions expanded into thoracic and abdominal portions, hyperfibrinogenemia and hypofibrinolytic activity were also seen; in three of these cases, hypercoagulability was evident because of the presence of fibrinogen derivatives having gamma-gamma-diads in the plasma. When the inflammation subsided after the establishment of aortic lesions, the end-stage (eight cases), hyperfibrinolytic activity with normal plasma fibrinogen was observed. It is suggested that hypercoagulability may be in response to the arterial disease and may possibly play a part in the development of this disorder.