Takayasu arteritis is a rare chronic vasculitis primarily involving the aorta and its main branches. We report an adolescent girl with Takayasu arteritis who presented with an isolated aortic valve regurgitation as part of a systemic inflammatory process. This patient was initially misdiagnosed as having rheumatic heart disease and the correct diagnosis was made only 1 year later. CONCLUSION: Takayasu arteritis should be considered among the diagnostic possibilities in patients who present with an unexplained systemic inflammatory syndrome and a cardiac murmur.
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