Takayasu Arteritis is a rare, chronic inflammatory disease that primarily affects the large arteries, most notably the aorta and its major branches.

This vascular inflammation leads to damage characterized by narrowing (stenosis), occlusion, or aneurysm formation in the affected blood vessels, which can significantly impair blood flow to various organs and tissues.

Pathophysiology and Causes

The precise cause of Takayasu Arteritis remains unknown, but it is believed to involve an immune-mediated inflammatory process whereby white blood cells invade the arterial walls, causing thickening, scarring, and damage. This injury can reduce or block blood flow, leading to ischemic symptoms.

Environmental factors combined with a genetic predisposition may contribute to disease onset. Some hypotheses suggest an infectious trigger, although this remains unproven.

Clinical Presentation

The disease manifests in two stages:

Early (Systemic) Stage: Non-specific symptoms such as fatigue, fever, night sweats, weight loss, muscle and joint aches, and anemia often appear, making early diagnosis challenging.

Late (Occlusive) Stage: As arterial inflammation progresses, narrowing or blockage leads to symptoms related to reduced blood flow, including limb claudication (pain or weakness with use), dizziness, headaches, chest pain, hypertension (sometimes difficult to detect due to vessel narrowing), absent or weak pulses in limbs (hence the nickname "pulseless disease"), and complications such as heart failure.

Diagnosis

Diagnosis is primarily clinical, supported by vascular imaging techniques such as angiography, MRI, or CT scans which visualize arterial narrowing, occlusion, or aneurysms. Blood tests reveal markers of inflammation but are not specific. Early recognition facilitates better management and complication prevention.

Treatment Strategies

Management centers on controlling inflammation and preventing arterial damage using corticosteroids as first-line treatment. Immunosuppressive agents like methotrexate or azathioprine often supplement steroids to enable dosage reduction and minimize side effects.

Biologic therapies targeting specific immune pathways are reserved for refractory cases. Interventions such as angioplasty or vascular surgery may be necessary to address critical arterial stenosis.

Dr. Gary S. Hoffman, MD, MS, a world-renowned authority on vasculitis, emphasized "Takayasu's arteritis is a great masquerader. Its early phase involves vague symptoms like fever, fatigue, and weight loss, often leading to delayed diagnosis. The disease typically becomes apparent only later, when arterial damage causes clear signs like claudication, absent pulses, or hypertension."

Takayasu Arteritis is a rare but serious inflammatory disease of large arteries that primarily affects young women. It produces a spectrum of symptoms ranging from systemic inflammation to severe vascular insufficiency and ischemia. While the exact cause remains unknown, immune-mediated arterial inflammation and genetic-environmental interplay appear central to its pathogenesis.

Diagnosis depends on clinical suspicion reinforced by imaging studies, and treatment focuses on immunosuppression to halt disease progression. Expert consensus highlights the importance of early recognition to mitigate vascular complications and improve patient prognosis.