Update in Takayasu’s Arteritis

Takayasu arteritis (TAK) is a rare, chronic granulomatous large-vessel arteritis affecting mainly aorta and its major branches. Vesssel wall inflammation causes segmental stenosis, occlusion, dilatation and/or aneurysms. Although all large arteries can be affected, aorta, subclavian and carotid arteries are the most commonly involved arteries. TAK is mainly seen in young females. Recent advances in the diagnosis, clinical course, disease assessment and treatment in TAK were discussed in this review. In the presence of typical symptoms and physical findings such as loss of pulses and/or decreased arterial blood pressure and elevated acute phase responses, the diagnosis should be confirmed easily by angiographic imaging modalities. MRA is the gold standard modality for both the diagnosis and the longitudinal follow-up patients with TAK. In recent years,PET has became a widely used imaging tool for the diagnosis with high sensitivity. The place of PET during follow-up in TAK is still controversial. Prognosis is possibly getting better with lower mortality, but a substantial damage is present even in early cases. It is critical to differentiate irreversible damage from disease activity and thus avoid potential over-treatment with toxic agents such as corticosteroids. There is a clear need to develop a validated set of outcome measures to be used in clinical trials of TAK.In daily practice, routine imaging follow-up is not recommended in clinically and laboratory silent TAK patients assessed as inactive by physician. The expert opinion is still the main determinant while managing TAK patients during daily practice. Glucorticoids are the mainstay of TAK treatment. While tapering glucocoticoids, non-biologic immunosuppressive agents should be added on the treatment. Leflunomide, methotrexate, azathiopurine, or mycophenolate mofetil could be chosen as the first line immunosuppressive agent. If there is a treatment failure with first line agents, switch to tumor necrosis factor inhibitors or tocilizumab should be thought.