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BACKGROUND To investigate the clinical features, management, and outcomes of childhood Takayasu arteritis (c-TA) initially presenting with hypertension. METHODS This study retrospectively reviewed medical charts of 96 inpatient c-TA cases from January 2002 to December 2016, with 5 additional patients being prospectively recruited from January 2017 to December 2017. Data were compared between c-TA groups initially presenting with and without hypertension. Blood pressure (BP) control, event-free survival, and associated risk factors were assessed by logistic regression, Kaplan–Meier survival curve, and COX regression models. RESULTS The hypertensive cohort (N = 71, 28.2% males) as compared with non-hypertensive cohort had significantly fewer active diseases; fewer episodes of claudication, syncope, blurred vision, and myocardial ischemia; and fewer systemic symptoms (P &lt; 0.05). The hypertensive group presented with more localized abdominal lesions (OR = 14.4, P = 0.001) and limited supradiaphragmatic arterial involvement. Renovascular disease (P = 0.001) and revascularization (P = 0.006) were associated with hypertension. At the median 3-year follow-up, 53% of hypertensive patients achieved BP control and 39% experienced events including vascular complications, flares, or death. The 1-, 3-, 5-, and 10-year event-free survival were 78.7% (95% CI: 65.7%–87.2%), 63.0% (95% CI: 48.1%–74.7%), 48.9% (95% CI: 32.0%–63.8%), and 31.6% (95% CI: 13.8%–51.2%), higher than in non-hypertensive group (P = 0.014). Heart failure, stroke, and body mass index &lt;18.5 kg/m2 were prognostic factors for events. Intervention and baseline systolic BP were independent factors for BP control (P &lt; 0.05). CONCLUSIONS Majority of c-TA has hypertension, presenting with a more quiescent disease without typical systemic and/or ischemia symptoms, more localized abdominal lesions, higher proportion of revascularizations and better event-free survival. Three-year BP control is more than 50%. Intervention particularly on renal artery is beneficial for BP control and decreased events. CLINICAL TRIAL REGISTRATION Trial Number: NCT03199183

Clinical Course, Management, and Outcomes of Pediatric Takayasu Arteritis Initially Presenting With Hypertension: A 16-year overview