Pulmonary hypertension in Takayasu arteritis with pulmonary artery involvement: risk factors and impact on mortality

BACKGROUND: Pulmonary artery involvement (PAI) in Takayasu arteritis (TAK) can cause pulmonary hypertension (PH). We aimed to compare clinical and imaging features between TAK patients with and without PH, identify factors associated with PH and describe long-term outcomes. METHODS: A prospective cohort of 147 TAK patients was studied from 2016 to 2024. Eligibility required fulfilment of established TAK criteria or multidisciplinary confirmation of CT-proven PAI after exclusion of other causes. Baseline demographics, clinical symptoms, laboratory and imaging findings, haemodynamic parameters and treatments were collected. The primary endpoint was all-cause mortality. Logistic regression identified factors associated with PH, and Cox proportional hazards regression evaluated predictors of mortality. RESULTS: Among 87 TAK-PAI patients, 40 (46%) had PH. Compared with non-PH patients, those with PH had higher Qanadli scores, longer diagnostic delay (median 20.5 vs 6.0 months, p=0.011), more severe symptoms, worse cardiopulmonary function, and higher N-terminal pro-B-type natriuretic peptide and right ventricular dimension (all p<0.01). During a median follow-up of 32 months, all eight deaths occurred in the PH group, seven without balloon pulmonary angioplasty. Kaplan-Meier survival was significantly worse for PH (p=0.0025). Multivariable analysis identified female sex (OR 4.543; 95% CI 1.103 to 18.717, p=0.036), higher Qanadli score (OR 14.729; 95% CI 2.718 to 79.809, p=0.002), uric acid (OR 1.007; 95% CI 1.002 to 1.013, p=0.010) and diagnostic delay per month (OR 1.036; 95% CI 1.010 to 1.063, p=0.006) as independent risk factors for PH. Worse WHO-functional class predicted mortality (HR 4.832; 95% CI 1.121 to 20.832, p=0.035). CONCLUSIONS: PH occurs in nearly half of TAK-PAI patients and is linked to severe symptoms, functional impairment and increased mortality. Higher Qanadli score, hyperuricaemia and delayed diagnosis are independent risk factors. Early recognition and timely intervention may improve outcomes. TRIAL REGISTRATION NUMBER: NCT07184814.