[Clinical characteristics and prognosis of childhood-onset Takayasu arteritis involving pulmonary artery].

Objective

To investigate the clinical characteristics, imaging features, risk factors, and prognosis of childhood-onset Takayasu arteritis (TAK) with pulmonary artery involvement.

Methods

A retrospective cohort study was conducted in 107 pediatric patients who were initially diagnosed with childhood-onset TAK at Department of Rheumatology and Immunology, Capital Center for Children's Health, Capital Medical Universiy, from January 2010 to December 2024. Clinical data, including demographic information, imaging features, treatment regimens, and prognosis were collected. Patients were divided into with and without pulmonary artery involvement groups. Intergroup comparisons were performed. Multivariate logistic regression was used to identify risk factors for pulmonary artery involvement. Kaplan-Meier analysis with Log-Rank testing was used for survival analyze.

Results

Among 107 children with TAK, 26 were male, 81 were female, with a diagnosis age of 88 (5, 137) months. Si cases were in the pulmonary artery involvement group and 91 cases in the non-pulmonary artery involvement group. The pulmonary artery involvement group was predominantly female (14 cases), with a diagnosis age of 39 (4, 104) months. The pulmonary artery involvement group had higher incidence rates of fatigue,pulmonary hypertension, right heart failure,superior mesenteric artery involvement,as well as higher neutrophil counts, C-reactive protein (CRP) levels (all P<0.05). Hemoglobin was lower in the pulmonary artery involvement group (P<0.05). Imaging findings revealed that all 16 children in the pulmonary artery involvement group showed signs of pulmonary arterial wall thickening. Other manifestations included dilation in 2 cases, stenosis in 2 cases, and occlusion in 1 case. Unilateral involvement (12 cases) was more common, and the right pulmonary artery (10 cases) was more frequently affected. Independent risk factors for pulmonary artery involvement in childhood-onset TAK patients included superior mesenteric artery involvement (OR=5.58, 95%CI 1.41-22.10, P=0.014) and elevated CRP levels (OR=1.02, 95%CI 1.00-1.03, P=0.027). During a follow-up of 3.9 (1.4,8.1) years, 2 patients with pulmonary artery involvement (all with pulmonary hypertension), among the survivors in the pulmonary artery involvement group, 2 cases still exhibited persistent pulmonary artery dilation, and one case had pulmonary artery occlusion; and 6 patients (6.6%) without pulmonary artery involvement died. Patients with pulmonary artery involvement had significantly lower survival rates compared to those without involvement (P=0.024).

Conclusions

Childhood-onset TAK with pulmonary artery involvement has an insidious clinical presentation, and can progress to pulmonary hypertension, pulmonary artery occlusion, and a significantly reduced survival rate. Patients with mesenteric artery involvement or elevated CRP have higher risks of pulmonary artery involvement, requiring close pulmonary vascular monitoring and early intervention to improve prognosis.