Long-term outcomes and prognostic predictors of patients with Takayasu's arteritis along with pulmonary artery involvement.

Objectives: This study aimed to investigate the clinical characteristics, results, and prognostic predictors of patients with Takayasu's arteritis (TAK) along with pulmonary artery involvement (PAI).

Methods: A total of 806 patients with TAK admitted to the Fuwai Hospital were screened. Clinical symptoms, imaging features, and prognosis were analysed, and patients were categorised into those with and those without pulmonary hypertension (PH). Additionally, risk factors associated with cardiac death and repeated hospitalisation were explored.

Results: Among 806 patients with TAK, 142 patients with PAI were included, 90.8% (n=129) of whom had PH diagnosed by right heart catheterisation and 9.2% (n=13) of whom did not. The median follow-up time was 54 (range, 29-83) months. Sixteen patients died from right heart failure caused by PH. Patients with PH were significantly more likely to have worse outcomes than patients without PH (p=0.027). The multivariate Cox proportional regression hazard model showed that the 6-min walk distance (6MWD) and PH-targeted therapy were independent prognostic predictors of cardiac death and hospital readmissions.

Conclusions: This study found that that a significant proportion of patients with TAK along with PAI had PH. Patients with PH had worse prognosis than those without. Further 6MWD and PH-targeted therapy were independent prognostic predictors of cardiac death or repeated hospitalization. In the future, multi-centre clinical studies are needed to further prospectively clarify this issue.