Introduction:

Takayasu arteritis (TA) is a large-vessel vasculitis characterized by granulomatous inflammation of the aorta and its major branches, resulting in stenosis, occlusions, dilations, and/or aneurysms, with a more severe presentation in children.

Case Report:

We present the case of a 16-year-old adolescent diagnosed with aortic valve stenosis at age 3. A diagnosis of type IIb TA was made at age 13 based on CT angiography: narrowing of the aorta at the level of the aortic arch and its branches at their origins, with thickening of the vascular walls. In addition, the patient presented with autoimmune hemolytic anemia (AHA) and pre-renal insufficiency due to decreased blood flow secondary to reduced cardiac output resulting from severe supravalvular aortic stenosis. Treatment included corticosteroids, intravenous immunoglobulin, cyclophosphamide for induction therapy, and rituximab for AHA, with continued maintenance of remission on methotrexate. Cardiac catheterization with stent placement in the ascending aorta was performed, with excellent results and immediate improvement in renal function due to increased blood flow.

Discussion:

In the presence of vascular complications in Takayasu arteritis, due to chronic involvement of the arteries, it is important to consider not only anti-inflammatory treatment but also the ideal vascular therapy for the case (hemodynamic vs. surgical revascularization).

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