Characterization of Connective Tissue Disease-Associated Pulmonary Arterial Hypertension From REVEAL Identifying Systemic Sclerosis as a Unique Phenotype

Background REVEAL (the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) is the largest US cohort of patients with pulmonary arterial hypertension (PAH) confirmed by right-sided heart catheterization (RHC), providing a more comprehensive subgroup characterization than previously possible We used REVEAL to analyze the clinical features of patients with connective tissue disease-associated PAH (CTD-APAH) Methods All newly and previously diagnosed patients with World Health Organization (WHO) group 1 PAH meeting RHC criteria at 54 US centers were consecutively enrolled Cross-sectional and 1-year mortality and hospitalization analyses from time of enrollment compared CTD-APAH to idiopathic disease and systemic sclerosis (SSc) to systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), and rheumatoid arthritis (RA) Results Compared with patients with idiopathic disease (n = 1,251), patients with CTD-APAH (n = 641) had better hemodynamics and favorable right ventricular echocardiographic findings but a higher prevalence of pericardial effusions, lower 6-min walk distance (300 5 +/- 118 0 vs 329 4 +/- 134 7 m, P = 01), higher B-type natriuretic peptide (BNP) levels (432 8 +/- 789 1 vs 245 6 +/- 427 2 pg/mL,P < 0001), and lower diffusing capacity of carbon monoxide (DLCO) (44 9% +/- 18 0% vs 63 6% +/- 22 1% predicted, P < 0001) One-year survival and freedom from hospitalization were lower in the CTD-APAH group (86% vs 93%, P < 0001, 67% vs 73%, P = 03) Compared with patients with SSc-APAH (n = 399), those with other CTDs (SLE, n = 110, MCTD, n = 52, RA, n = 28) had similar hemodynamics, however, patients with SSc-APAH had the highest BNP levels (552 2 +/- 977 8 pg/mL), lowest DLCO (41 2% +/- 16 3% predicted), and poorest 1-year survival (82% vs 94% in SLE-APAH, 88% in MCTD-APAH, and 96% in RA-APAH) Conclusions Patients with SSc-APAH demonstrate a unique phenotype with the highest BNP levels, lowest DLCO, and poorest survival of all CTD-APAH subgroups Trial registry ClinicalTrials gov, No NCT00370214, URL clinicaltrials org CHEST 2010, 138(6) 1383-1394