A 20-year-old woman complains of double vision, fainting spells, tingling of the fingers of her left hand and numbness of the fingers of her right hand. Physical examination reveals absence of pulse in her right arm. Laboratory tests show elevated erythrocyte sedimentation rate and thrombocytosis. An aoogram demonstrates narrowing and occlusion of branching aeries, including the right subclan aery. The patient subsequently develops hea failure and dies of massive pulmonary edema. At autopsy, the aoa has a thickened wall and shows vasculitis and fragmentation of elastic fibers. Which of the following is the most likely diagnosis?
Correct Answer: Takayasu aeritis
Description: Takayasu aeritis is an inflammatory disorder of large aeries, classically the aoic arch and its major branches. On gross examination, the aoa is thickened and the intima exhibits focal, raised plaques. The branches of the aoa often display localized stenosis or occlusion, which interferes with blood flow and accounts for the symptoms of "pulseless" disease. Ischemic cerebrovascular episodes in a young woman and a differential between the blood pressure in the left and the right arm suggest the diagnosis of Takayasu disease. More than 90% of patients are women under 30 years of age. The other choices are diseases of aeries that have different clinical and pathologic manifestations.
Category:
Pathology
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