Refractory Case of Takayasu Arteritis in a Young Woman: A Clinical Challenge

Takayasu arteritis (TA) is an idiopathic chronic inflammatory vasculitis of the aorta and its main branches, which if not treated can lead to severe vascular damage and fatal vascular events. Glucocorticoids (GCs) are the mainstay of the therapy of TA but a significant proportion of patients tend to experience flare-ups when their GCs are tapered. We report a case of a 42-year-old female with TA, diagnosed according to the 1990 American College of Rheumatology Criteria for TA. Cardiovascular assessment showed normal carotid upstrokes with bilateral carotid bruits and soft right and left subclavian bruits with weak peripheral pulses. A computed tomography (CT) aortogram of the chest showed severe stenosis of bilateral subclavian arteries and mild stenosis of right and left common carotid arteries at the origin. A CT aortogram of the abdomen showed an occluded left renal artery, a very small left kidney, and mild narrowing of the abdominal aorta below the level of renal arteries.  She was initially managed with GCs along with immunosuppressive therapy including methotrexate, azathioprine, and cyclophosphamide, but her disease remained active. She was then sequentially treated with inhibitor etanercept (ETN), inhibitor tocilizumab (TCZ) and monoclonal anti-CD20 antibody rituximab (RTX), and in spite of aggressive biologic therapy she continued to have active disease. To the best of our knowledge, this is the first case of refractory TA treated sequentially with three different biologic drugs.