Abstract
Childhood‐onset Takayasu arteritis is rare, and data on outcomes beyond two or 3 decades are exceptionally limited. We report one of the longest documented follow‐up observations of childhood‐onset Takayasu arteritis, spanning more than 4 decades. The disease began at 9 years of age, and the patient was diagnosed with morphologically confirmed Type III Takayasu arteritis at the age of 16 years. She underwent renal vascular angioplasty and prosthetic reconstruction of the abdominal aorta for left renal artery occlusion associated with severe renovascular hypertension. In 2010, progression of supraaortic vascular disease required aortocarotid bypass surgery, followed six months later by left nephrectomy for persistent severe hypertension. Long‐term medical management included individualized intermittent treatment with low‐dose prednisolone and methotrexate. Despite extensive vascular involvement, the clinical course was characterized by gradual stabilization, progressively longer periods of clinical stability, and preservation of functional independence. Recent laboratory findings demonstrated only mild elevations in inflammatory markers, while vascular imaging showed chronic extensive arterial lesions with well‐developed collateral circulation, preserved cardiac function, and preserved function of the solitary right kidney. The favorable long‐term course was likely multifactorial, reflecting staged vascular reconstruction, individualized medical management, and adaptive collateral circulation. This case provides rare insight into the long‐term evolution and management of severe childhood‐onset Takayasu arteritis.