Takayasu arteritis (TA) is a form of idiopathic large vessel vasculitis that predominantly affects women of reproductive age. Although TA is a rare disease, the interpretation of longitudinal data from several countries provides new insights into the clinical course and outcomes in TA across different racial and ethnic groups. Contemporary studies belie prior perceptions of TA as a disease with a self-limited, benign course. We now recognize this disease as one that often relapses, leaves the patient chronically dependent on glucocorticoids for disease control, and frequently leads to disability. Limited data suggest that the targeted inhibition of tumor necrosis factor (TNF) might be an effective therapy for TA.