Objective To investigate the impact of regular follow-up with treatment to target within one year on the prognosis of patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH).

Methods A retrospective analysis was conducted on the clinical data of 65 patients with CTD-PAH diagnosed by right heart catheterization in the Department of Rheumatology and Immunology of the First Affiliated Hospital of Nanjing Medical University from January 2017 to December 2021. Treatment to target was determined according to the following three criteria: World Health Organization (WHO) functional class Ⅰto Ⅱ, 6-minute walk distance (6MWD)>440 m, and N-terminal pro-B-type natriuretic peptide (NT-proBNP) < 300 ng/L. The primary study endpoint was 3-year all-cause mortality, and the secondary endpoint was the first clinical worsening event within 3 years. The Kaplan-Meier method was used to calculate cumulative survival rate and event-free survival rate.

Results Among the 65 CTD-PAH patients, 38 underwent standardized follow-up within one year after PAH diagnosis by right heart catheterization, while 27 received non-standardized follow-up. There were no significant differences in proportion of gender (female), age, disease types of CTD, and PAH severity between the regular follow-up group and the irregular follow-up group (P>0.05). The 3-year survival rate of CTD-PAH patients with regular follow-up within one year of diagnosis was significantly higher than that of patients with irregular follow-up (P=0.004). Among the 38 patients with regular follow-up, one patient died, with 1-year and 3-year cumulative survival rates of 100.0% and 95.7%, respectively; among the 27 patients with irregular follow-up, 8 patients died, with 1-year and 3-year cumulative survival rates of 81.5% and 68.0%, respectively. Further analysis of factors influencing follow-up compliance in CTD-PAH patients revealed that age (OR=1.045, 95%CI, 1.006 to 1.085, P=0.023) and non-medical insurance payment (OR=5.333, 95%CI, 1.260 to 22.567, P=0.023) were the main factors influencing follow-up compliance for CTD-PAH. Among the 38 CTD-PAH patients with regular follow-up, 14 achieved PAH treatment targets within one year, with no clinical failure events occurring, and 1-year and 3-year event-free survival rates of 100.0%; 24 patients did not achieve PAH treatment targets within one year, with 7 experiencing clinical worsening events, and 1-year and 3-year event-free survival rates of 87.5% and 69.3%, respectively.

Conclusion Regular follow-up within one year of diagnosis may improve the long-term prognosis of patients with CTD-PAH.