Takayasu arteritis

Takayasu arteritis (also known as Takayasu disease, pulseless disease, or aortic arch syndrome) is a chronic, progressive inflammatory disease affecting the large blood vessels, primarily the aorta and its major branches, as well as the pulmonary arteries. It belongs to the group of large-vessel vasculitides. The inflammation causes thickening, narrowing (stenosis), occlusion, and sometimes dilation or aneurysm formation of the affected arteries. This leads to reduced blood flow to vital organs and extremities. The disease predominantly affects young women, typically presenting between the ages of 10 and 40 years, and is more common in Asian populations, though it occurs worldwide. Key symptoms include diminished or absent peripheral pulses (hence the name 'pulseless disease'), limb claudication, blood pressure discrepancies between arms, bruits over affected arteries, dizziness, visual disturbances, headaches, fatigue, fever, weight loss, and general malaise. In the early inflammatory phase, patients may experience systemic symptoms such as fever, night sweats, and joint pain. As the disease progresses, vascular complications can lead to hypertension (particularly renovascular), stroke, aortic regurgitation, heart failure, and ischemia of various organs. Diagnosis is based on clinical findings, elevated inflammatory markers, and characteristic imaging findings on CT angiography, MR angiography, or conventional angiography showing arterial wall thickening, stenosis, or aneurysms. Treatment involves immunosuppressive therapy, with glucocorticoids as the first-line treatment. Steroid-sparing agents such as methotrexate, azathioprine, and mycophenolate mofetil are commonly used. Biologic therapies, particularly tocilizumab (an IL-6 receptor inhibitor) and TNF-alpha inhibitors, have shown benefit in refractory cases. Vascular interventions, including angioplasty and surgical bypass, may be necessary for critical stenoses or aneurysms, ideally performed during periods of disease quiescence. Despite treatment, relapses are common, and long-term monitoring is essential.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Takayasu arteritis