Longitudinal study of 16 patients with Takayasu's arteritis: clinical features and therapeutic management.

摘要

Takayasu's arteritis (TA) is a rare vasculitis that affects the aorta and its primary branches. Heterogeneous clinical manifestations have been described in different geographical areas. We aimed to evaluate demographic, clinical, and angiographic features and the outcome in 16 TA patients who were followed in a single center in Serbia. TA was diagnosed according to the 1990 American College of Rheumatology criteria with median delay of 2 years. The majority of patients (81%) experienced one or more disease relapses and long-term remission was achieved in 94%. Five-year survival rate was 94%. Common systemic manifestations at disease onset included fever (69%), arthralgia (25%), weight loss (19%) and vascular symptoms, arm claudication (100%), headaches (69%), dizziness (56%), and hypertension (37%). All patients had multiple arterial bruits with diminished/absent left arm pulse in 88%. The raised erythrocyte sedimentation rate and C-reactive protein levels were found in 63% and 80%, respectively. Angiography revealed stenotic or occlusive sites of multiple arterial involvement. Stenosis of the left subclavian artery was the most frequent lesion (88%). The angiographic classification was as follows, 50% type I, 19% type IIa, and 31% type V arteritis, suggesting relatively favorable vascular lesions and infrequent serious complications. Glucocorticoids were the main initial therapy for active disease; however, treatment with additional immunosuppressive agents was required in two-thirds of the patients. Seven patients (44%) underwent one or multiple surgical interventions. The main indications for surgery were cerebral hypoperfusion, severe limb claudication, and gangrenous infection. The demographic and angiographic disease patterns in our cohort were similar to American, Italian, Japanese, and Korean cohorts in terms of frequent involvement of the branches of aortic arch and differed from cohorts from other Asian countries in which abdominal aorta and renal artery involvement prevailed.