[Follow-up study of Takayasu arteritis with aortic regurgitation].

The clinical courses of patients with Takayasu arteritis vary especially when aortic regurgitation is involved. The clinical features and outcomes of Takayasu arteritis were studied in 78 patients to clarify the influence of aortic regurgitation on the natural history, especially the earlier stages of aortic regurgitation after onset of Takayasu arteritis. During the average 12.7-year follow-up period, 7% (3/43) of patients without aortic regurgitation died, but 17% (6/35) of patients with aortic regurgitation died. Mortality was low (6%; 1/16) in patients with mild (grade II or less) regurgitation, but high (26%; 5/19) in patients with severe (greater than grade III) regurgitation. Predictors indicating patients likely to die of severe aortic regurgitation were age at onset (mean age of 30.0 years), C reactive protein and erythrocyte sedimentation rate, and presence or absence of involvement of major branches of the aortic arch. Mortality was 33% (2/6) in patients without involvement of the major branches, which was significantly higher than that of patients with such involvement (17%; 2/12). From the initial consultation, most patients with mild regurgitation remained stable, but younger patients with severe regurgitation due to acute pathological processes of the ascending aorta from the early stage and elevated erythrocyte sedimentation rate showed deterioration in their clinical courses. Younger patients with elevated erythrocyte sedimentation rate, intact major branches of the aortic arch, no signs of classical pulseless disease, and severe aortic regurgitation due to Takayasu arteritis have a poor prognosis.