Abstract
Forty-five patients with Takayasu's disease were studied between 1972 and the end of 1979. Operative treatment was less frequently needed for aortic arch disease than for descending aorta disease. Correct evaluation may be difficult in the latter till the time of operative exploration. Children with significant stenosis need early operation and a "plasty' type of repair is favoured over bypass grafts. Contrary to some descriptions, the disease process can affect the region of the aortoiliac bifurcation, needing surgical management based on established principles. Correct evaluation can make operative treatment safe and rewarding for those patients who need intervention as the recurrence rate of the disease process has been low in surgically treated patients. Immunologically the patients show defective T-lymphocyte function, increase in ther serum level of IgG, and a reduction in serum complement constituents C3 and C4, indicating the possibility of formation of a complement-binding immune complex. Histochemical studies show deposition of IgG and PAS-positive material in the intima and probable deposition of IgM and IgA in the junctional area of media and intima, with total destruction of the elastic lamina. A hypothesis for the pathogenesis of the disease is presented.
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