Natural history of aortoarteritis (Takayasu's disease).

Abstract

The natural history of aortoarteritis was studied in 88 patients (54 women and 34 men). The average age was 24.0 .+-. 8.8 years at onset of symptoms and 28.3 .+-. 9.9 years at diagnosis. The folow-up period was 83.6 .+-. 74.4 months from onset and 33.2 .+-. 37.0 months from diagnosis. Ten patients (11.4%) died during follow-up (0.016 deaths/patient year), and 22 patients (25%) suffered major nonfatal events (0.042 events/patient year). The cumulative survival and 5 and 10 years after the onset was 91.0 .+-. 3.3% and 84.0 .+-. 5.6% (mean .+-. SEM), respectively. The event-free survival rates at the same intervals after onset were 74.9 .+-. 5.0% and 64.0 .+-. 7.4%, respectively. The overall survival and event-free survival at 10 years after diagnosis was 80.3 .+-. 6.5% and 61.6 .+-. 7.5%, respectively. Patients with no complications or a mild single complication at diagnosis had a higher event-free survival rate than those with severe single complication or multiple complications at 5 years-97.0 .+-. 2.9% and 59.7 .+-. 7.3%, respectively (p < 0.001). Severe hypertension (p < 0.01), severe functional disability (p < 0.01), and evidence of cardiac involvement (p < 0.05) were good predictors of either death or major event on follow-up. These data are useful in making an objective assessment of the prognosis and in planning elective interventions.