Abstract
BackgroundChildhood Takayasu’s arteritis (c-TA) is scarcely reported but is characterized by devastating morbidity and mortality. This study aims to investigate the clinical course of c-TA and prognostic factors associated with rehospitalization and events including vascular complications, flares, and death.MethodsAn ambispective study of 101 c-TA patients satisfying the American College of Rheumatology (ACR) criteria and/or the European League against Rheumatism (EULAR)/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS) criteria was conducted from January 2002 to December 2017. Data on demographic, clinical, laboratory, imaging, and therapeutic features were collected. Event-free survival, complication-free survival, flare-free survival, rehospitalization-free survival, and associated prognostic factors were assessed by Kaplan-Meier survival curve and propensity score analysis.ResultsThe median age at c-TA onset was 14 (interquartile range (IQR) 12–16) years and 76.2% were female. Hypertension (70.3%), blood pressure discrepancy (55.4%), bruits (51.5%), and pulse deficits (37.6%) were core presentations. Major vascular involvement included the renal artery (62.4%), abdominal aorta (42.6%), subclavian artery (43.6%), and carotid artery (42.6%). Glucocorticoids (78.2%), antihypertensive drugs (72.3%), antiplatelet agents (72.3%), and revascularization (57.4%) were made up the majority administered. At a median 2.4 (IQR 0.7–6.1) years of follow-up, events, rehospitalization, vascular complications, flares and death were observed in 44.6%, 37.6%, 44.6%, 26.7%, and 3%, respectively. The 5-year event-free survival, rehospitalization-free survival, vascular complication-free survival, and flare-free survival were 42.8%, 55.8%, 45.9%, and 62.3%, respectively. Body mass index (BMI) (hazard ratio (HR) = 0.49, 95% confidence interval (CI) 0.30–0.81, p = 0.005), stroke (HR = 7.37, 95% CI 2.35–23.1, p = 0.001), and revascularization (HR = 0.51, 95% CI 0.27–0.94, p = 0.032) were independent prognostic predictors of events. Predictors for rehospitalization include age at admission (HR = 0.81, 95% CI 0.69–0.94, p = 0.006), renal artery involvement (HR = 0.49, 95% CI 0.25–0.96, p = 0.037), and elevated C-reactive protein (CRP; HR = 2.50, 95% CI 1.24–5.00, p = 0.01). BMI level (p = 0.024) and renal artery involvement (p = 0.015) were also associated with vascular complications, while revascularization (p = 0.002) independently correlated with re-flares.ConclusionsThis large ambispective study of c-TA revealed an early 3% mortality at the first year and around 50% morbidity within 5 years after diagnosis. Hypertension, renal artery involvement, and revascularization based on anti-inflammation, antihypertension, and antiplatelet medications dominated c-TA with indications for optimistic prognosis. Patients with initial lower BMI level, a younger age at admission, stroke, and elevated CRP have a high risk of poor outcomes, requiring close c-TA monitoring and more aggressive management.Trial registrationNCT03199183, unique protocol ID: 2016-ZX43. June 26, 2017
Highlights
Childhood Takayasu’s arteritis (c-TA) is scarcely reported but is characterized by devastating morbidity and mortality
Patients and inclusion criteria We conducted an ambispective cohort study of 101 c-TA patients in Fuwai Hospital, Chinese Academy of Medical Sciences, with 96 patients retrospectively recruited from January 2002 to December 2016 and five patients prospectively enrolled from January 2017 to December 2017
Three patients not satisfying the American College of Rheumatology (ACR) criteria or the European League against Rheumatism (EULAR)/ Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS) criteria were included with a clinical diagnosis of c-TA
Summary
Childhood Takayasu’s arteritis (c-TA) is scarcely reported but is characterized by devastating morbidity and mortality. Childhood TA (c-TA), the most common large vessel vasculitis in the pediatric population, has been studied in 17 cohorts of 445 c-TA cases (including 24 Chinese patients) to date, with an uncertain clinical profile but devastating mortality reaching 27% (Additional file 1: Table S1) [4,5,6,7,8,9]. Comprehensive data on management and outcome prediction of c-TA are extremely lacking To address these issues, the present study aims to: 1) describe the clinical phenotypes, angiographic findings, and diagnostic algorithm of c-TA; 2) summarize the therapeutic strategies and prognosis for c-TA; and 3) identify the predictors of c-TA outcomes, including vascular complications, flares, all-cause death, and rehospitalization
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