Abstract: Objective To explore the clinical characteristics and risk factors for poor prognosis in patients with late-onset connective tissue disease (CTD) associated pulmonary arterial hypertension (PAH). Methods A retrospective analysis was conducted on patients diagnosed with CTD-PAH at Department of Rheumatology, the First Affiliated Hospital of Nanjing Medical University from January 2019 to December 2023 using right heart catheterization(RHC). Patients were divided into two groups: early-onset group(onset age<60 years) and late-onset group(onset age ≥60 years). Demographic data, cardiovascular risk factors (hypertension, coronary artery disease and diabetes), echocardiographic and RHC parameters, treatment for CTD and PAH, clinical failure-free survival rates were observed and compared between the two groups. And the influecing factors for clinical failve events was analysized. Results Compared with the early-onset group, the late-onset group had a higher prevalence of coronary heart disease and hypertension; Late-onset patients exhibited worse cardiac function, with a higher proportion of cardiac function grading Ⅲ-Ⅳ and significantly shorter 6-minute walk distance (6MWD) (P＜0.05). Two-dimensional echocardiography showed that left atrial diameter (LAD), left ventricular end-diastolic diameter (LVDd), and the E/e′ ratio were significantly increased in the late-onset group than those in the early-onset group(P＜0.05). Compared with the early-onset group, the utilization rate of glucocorticoids and the rate of combination targeted therapy for PAH were significantly lower in the late-onset group(P＜0.05). Within one year of follow-up, the proportion of patients achieving a low-risk status in the late-onset group was significantly lower than that in the early-onset group (25.0% vs 62.1%,P＜0.05). Cox regression analysis indicated that the E/e′ ratio was a risk factor for clinical failure events in patients with late-onset CTD-PAH(HR=1.295,95%CI:1.039-1.615,P=0.022). Conclusions Late-onset CTD-PAH patients have a higher incidence of cardiovascular risk factors, with elevated left atrial pressure and impaired left ventricular diastolic function. They generally have a poorer prognosis, and E/e′ is a risk factor for adverse outcomes in these patients.    

Key words: late-onset, connective tissue disease, pulmonary arterial hypertension, clinical characteristics, prognosis