Background Pulmonary arterial hypertension (PAH) is a major cause of mortality in connective tissue disease (CTD).
Objectives The survival rates and mortality-predictive factors of a nationwide registry of Korean patients with CTD-PAH were determined.
Methods Patients with CTD-PAH were enrolled between April 2008 and December 2012. Hemodynamic parameters and clinical data (WHO-functional class [FC], organ involvement, laboratory tests, and treatment agents) were recorded. Survival rates were calculated by using the Kaplan–Meier method. Mortality-associated factors were examined by Cox proportional hazards regression analysis.
Results In total, 174 incident PAH cases (61 with systemic lupus erythematosus, 50 with systemic sclerosis, 10 with mixed CTD, 22 with rheumatoid arthritis [RA], and 31 with other CTDs) were diagnosed by right heart catheterisation or Doppler echocardiography. Of these, 25 (14%) died during the 3.8±2.7 year follow-up period after PAH diagnosis. The 1 and 3 year survival rates were 90.7% and 87.3%, respectively. Compared to the other CTD-PAHs, RA-PAH had the lowest survival rates (56% 3 year survival; p=0.022). Multiple regression analysis revealed that low DLCO, pleural effusion, and diabetes mellitus were poor prognostic factors (p=0.008, 0.04, and 0.009, respectively). Anti-UI-RNP antibody positivity was protective (p=0.022). In patients with WHO-FC III/IV, patients who received vasodilators had lower mortality than those who did not (p=0.038).
Conclusions In Korean patients with CTD-PAH, the 3 year survival rate was 87%. Low DLCO, pleural effusion, and DM were independent poor prognostic factors. Anti-UI-RNP antibody was protective. Prompt PAH-specific vasodilator therapy may improve the survival of patients with severe CTD-PAH.
Disclosure of Interest None declared
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