Takayasuarteritis

Takayasu arteritis is a rare granulomatous vasculitis that preferentially involves the aorta and its major branches. It typically presents in young adults, especially women of Asian descent, though cases occur worldwide. The disease causes inflammation of large arteries, leading to stenosis, occlusion, or aneurysm formation and resulting organ ischemia.

Pathophysiology involves chronic granulomatous inflammation of the vessel wall with intimal hyperplasia and adventitial scarring, producing vessel narrowing. Inflammation can affect the aorta, subclavian, carotid, renal, mesenteric, and iliac arteries. Genetic and immune factors, including HLA associations, contribute to susceptibility.

Clinical features include systemic symptoms such as fever and weight loss, as well as vascular signs like claudication of limbs, diminished or unequal pulses, blood pressure differences between arms, and bruits over affected arteries. Hypertension and neurologic or organ-specific symptoms reflect involvement of renal, carotid, or mesenteric arteries.

Diagnosis relies on clinical suspicion supported by imaging and laboratory tests. The American College of Rheumatology criteria require at least three of six items: age at onset under 40, claudication of extremities, decreased pulses, blood pressure difference, bruits, and arteriographic abnormality. Imaging (CTA, MRA, or conventional angiography) shows arterial abnormalities.

Treatment centers on reducing vessel inflammation and preventing progression. First-line therapy is glucocorticoids, followed by immunosuppressive agents such as methotrexate or azathioprine. Biologic therapy (eg, tocilizumab) may be used for refractory disease. Revascularization or endovascular procedures can relieve critical stenosis but carries restenosis risk.

Prognosis varies with extent and speed of diagnosis. Relapses are common and long-term monitoring is needed. Outcomes have improved with modern immunosuppression, but vascular complications, including occlusion or aneurysm, remain a concern. The disease is rare, with higher incidence in young women from East Asia.