Diagnostic approach and proposed criteria for the clinical diagnosis of Takayasu's arteriopathy.

The criteria proposed for the clinical diagnosis of Takayasu's disease (chronic inflammatory arteriopathy of unknown origin) were based on clinical and angiographic data from 108 Japanese patients: 96 with Takayasu's disease and 12 with another disease of the aorta. The criteria consist of one obligatory criterion (age less than or equal to 40 years), two major criteria (left and right mid subclavian artery lesions) and nine minor criteria (high erythrocyte sedimentation rate, common carotid artery tenderness, hypertension, aortic regurgitation or annuloaortic ectasia and lesions of the pulmonary artery, left mid common carotid artery, distal brachiocephalic trunk, thoracic aorta and abdominal aorta). In addition to the obligatory criterion, the presence of two major criteria or of one major plus two or more minor criteria, or of four or more minor criteria suggests a high probability of the presence of Takayasu's disease. The criteria had an 84% sensitivity in 96 patients with this disease: 52 (96%) of 54 patients in the active young group, 8 (80%) of 10 in the active older group, 14 (67%) of 21 in the inactive young group and 7 (64%) of 11 in the inactive older group fulfilled the criteria; however, none of the 12 patients with other aortic diseases did so. Use of these criteria has shortened the delay of an accurate diagnosis in patients with Takayasu's disease.