A 22-year old male patient was admitted to the Emergency room of local clinic because of the stuporous mental status. On physical examination, he apperaed as a chronically ill, slender man there was complete absence of the left arm pulse, and a
prominent thrill and briuts were present over the right subclavicular area. the blood pressure which checked in the other regions except for the left arm was hypertensive (right arm 180/100, right leg 210/120, and left leg 200/130nmHg). An x-ray
film of
the chest and doppler ultrasounds showed characteristic dilatation of aortic arch, ascending aorta and innominate artery. The clinical impression was Takayasu's disease.
This patient's condition deteriorated rapidly in spite of intensive care and died on the seventh hospital day.
At autopsy, the cause of death was massive left brain infarction due to middle cerebral artery embolization. The opened luminal surface of the aorta and its major branches revealed irregulary thick-walled, tree-bark -like appearance. Also in the
left
common carotid artery, the lumen was nearly obliterated by thromi and intimal fibrous plaques. But the thoraco-abdominal aorta was spared. Microscopic examination disclosed granulomatous and sclerosing arteritis. In the former, the inflammatory
process
was confined to the media and adventitia and the cell infiltrate consisted predominantly of lymphocytes, plasma cells and histiocytes, with a varying number of giant cells. The latter was characterized bland intimal fibrous hyperplasia, medial
degeneration with disrupted elastic lamella, and adventitial fibrosis. The pathologic diagnosis was Takayasu's disease of type I.
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