Case report highlights diagnostic challenges and successful treatment with immunosuppressive therapy in Takayasu arteritis.
Takayasu arteritis (TA) is a rare, chronic, large-vessel vasculitis affecting the aorta and its major branches, typically seen in young females. Childhood-onset Takayasu arteritis (cTAK) poses significant diagnostic challenges due to its heterogeneous presentation and nonspecific systemic symptoms. We report the case of a 14-year-old girl who presented with isolated retrosternal chest pain of one-month duration, unrelieved by empirical therapy. Clinical examination revealed absent upper limb pulses with non-recordable blood pressure, while lower limb pressures were normal. CT aortogram demonstrated diffuse mural thickening involving the aortic arch and its major branches, with occlusion of the right subclavian and vertebral arteries, consistent with type V Takayasu arteritis in the active stage. The patient was treated with high-dose corticosteroids and intravenous tocilizumab (8 mg/kg monthly), resulting in rapid resolution of symptoms and normalization of inflammatory markers. This case highlights the diagnostic importance of a high index of suspicion in adolescents presenting with atypical chest pain. Early vascular imaging and prompt initiation of immunosuppressive therapy, including biologics, are crucial in preventing disease progression and achieving sustained remission. Recognition of such atypical presentations can improve outcomes in pediatric large-vessel vasculitis.
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Vijayan et al. (2025) studied this question.
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