Treatment of Takayasu arteritis

Abstract

Despite advances in the diagnosis and treatment, the mortality rate of Takayasu arteritis (TAK) is still elevated even today. The diagnosis is often made after along time delay and the course of the disease is characterized by progressive structural vascular lesions. Recently, new recommendations for the management of large vessel vasculitis were published by the European League Against Rheumatism (EULAR). For induction of remission oral glucocorticoids (GC) are administered in an initial daily dose of 40-60 mg. As experience has shown that the cumulative GC demand in TAK is high, GC-sparing treatment with moderately potent immunosuppressants, such as methotrexate, azathioprine and mycophenolate mofetil is recommended from the time of initial diagnosis. In cases of arelapsing course, tocilizumab or tumor necrosis factor (TNF)-alpha inhibitors can be used as an additive off-label treatment. If vascular stenoses persist despite supposedly sufficient inflammation control and if these stenoses are symptomatic, vascular surgery or interventional treatment procedures can be indicated. Such revascularization or even surgical procedures for the treatment of aneurysms should be performed during phases of sufficient drug control of the vasculitis. In quite afew patients progressive vascular lesions continue to develop despite clinical and laboratory analytical remission. Due to the poor correlation of clinical symptoms and acute phase markers with the progression of vascular lesions, the distinction between active and inactive diseases is often achallenge in the clinical practice. Imaging studies can then support therapeutic decisions but are not yet formally and comprehensively validated in the long-term course of TAK.