TREATMENT
Active phase TA is initially treated with high-dose glucocorticoid therapy (prednisone or methylprednisolone). Typical prednisone doses are 0.5 to 1 mg/kg/d.5 Clinical improvement is seen in almost all patients with glucocorticoid therapy,6,10,23 but relapse is common when prednisone is tapered to less than 20 mg/d.5 The corticosteroid dose is gradually tapered depending on patient response. Common side effects of corticosteroids may include weight gain, elevations in blood glucose, insomnia, increased infection risk, osteoporosis, and slowing of wound healing.
Because nearly half of all patients treated with glucocorticoids alone demonstrate chronic active disease, immunosuppressive therapies are almost always used concomitantly.27 Immune-suppressing drugs that may be used include methotrexate (15 to 25 mg/wk), azathioprine (2 mg/kg/d), and cyclophosphamide (1 to 2 mg/kg/d orally).5,28 Tumor necrosis factor (TNF)–blocking agents used to treat TA include etanercept, infliximab, or adalimumab.28,29 Adverse effects associated with immunosuppressive therapies and TNF-blocking agents include an increased risk for infection(s) and malignancy, bone marrow suppression, and hepatitis B reactivation. Although data are limited on anti-TNF agents, this class of drug has shown promise when used in conjunction with corticosteroids.28
In one open-label study by Hoffman and colleagues, remission rates with methotrexate plus steroids were 81%. Relapse occurred in 44% of study participants when the steroid dose was tapered or decreased to near discontinuation.27 More recently, in an uncontrolled study series involving 15 TA patients from India who were treated with azathioprine plus steroids, remission was achieved following 12 weeks of therapy. Angiographically, there was no progression of arterial disease after one year.30
Surgical and endovascular procedures used to return blood flow in stenotic or occluded vessels include synthetic or autologous vessel bypass, endarterectomy, and percutaneous transluminal angioplasty.5 When aortic insufficiency is present, aortic root replacement or repair is undertaken.5 These procedures are performed by vascular or cardiovascular surgeons and interventional radiologists. Rheumatologists are the medical specialists most involved in the direct care and management of TA patients. Cardiologists are sometimes consulted as well.
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