Current Clinical Features of New Patients With Takayasu Arteritis Observed From Cross-Country Research in Japan: Age and Sex Specificity.

BACKGROUND The clinical features of newly diagnosed Japanese patients with Takayasu arteritis and its age or sex specificities are unknown. METHODS AND RESULTS We analyzed information from nationwide registration forms submitted by patients with Takayasu arteritis between April 2001 and March 2011 as part of a research program by the Japanese Ministry of Health, Labor and Welfare. Among the 7779 patients who submitted their forms, 1372 newly registered patients with Takayasu arteritis were enrolled; 83.8% were female. The median age at onset was 35 years, which was significantly higher in male patients (median, 43.5 years) than in female patients (median, 34 years; P<0.001). Local symptoms and findings were most commonly observed in the cervicobrachial area, with more complaints in the head or neck than in the upper limbs. Approximately 85% of the patients had vascular involvements in the aortic arch or its major branches; many young female patients had localized lesions. Although male patients had extensive aortic lesions or aneurysms with more complications, localized abdominal lesions were relatively more frequent in male patients with age at onset >40 years than in other age-sex groups. Disease statuses were severe in patients who registered at ≥1 year after onset. CONCLUSIONS The proportions of male patients and patients with elderly onset increased in newly diagnosed patients with Takayasu arteritis. Their clinical and angiographic features differed according to onset age and sex.