PULMONARY HYPERTENSION ASSESSMENT AND RECOGNITION OF OUTCOMES IN SCLERODERMA (PHAROS): PATIENTS WITH PULMONARY HYPERTENSION

Abstract

Purpose: Pulmonary hypertension (PH) is a frequent complication of patients with systemic sclerosis (SSc) and remains a leading cause of death. Pulmonary arterial hypertension (PAH, World Health Organization Group I) is the most common form of PH in SSc, but PH related to left heart disease (Group II) and interstitial lung disease (ILD, Group III) can also occur. The purpose of this analysis was to characterize patients with incident PH from the PHAROS Registry. Methods: PHAROS is a multicenter prospective, observational study to determine risk factors and outcomes of patients who are at high risk or have PH. Patients with definite PH, defined as mean pulmonary artery pressure (MPAP) >=25 mmHg at rest or >=30 mmHg with exercise on right heart catheterization within 6 months of enrollment or newly developed during follow-up, were included in the analysis. A pulmonary capillary wedge pressure <60% predicted were included in Group III. ANOVA analyses were used to compare baseline features among the groups. Results: Of the 103 patients with PH, 79 were included in Group I (10 with exercise-induced PAH), and 12 each in Groups II and III. Overall, 85% of the patients were female with a mean(SD) age of 57.8(10.8) years and disease duration from first non-Raynaud's symptom of 9.1(7.8) years. Group III had more African Americans (42% vs. 13% (Group I) and 17% (Group II), p=0.007) and a higher proportion with the SCL-70 antibody (25% vs. 5% (Group I) and 8% (Group II), p=0.02) and a lower proportion with the centromere antibody (8% vs. 28% (Group I) and 25% (Group II), p=0.03), but the groups did not differ by SSc subtype (overall 63% limited). MPAP was similar among the groups with an overall mean of 33.9(11.3) mmHg. Pulmonary vascular resistance was highest in Group I (404(245) dyn*s*cm-5 vs. 210(182) (Group II) and 305(215) (Group III), p=0.03). The groups did not differ with respect to New York Heart Association functional class or percentage using home oxygen. In Group I, initial treatment was with prostacyclins, endothelin receptor antagonists, sildenafil, and combination PH therapies in 13%, 22%, 30%, and 8%, respectively. At last follow-up, the proportion of patients taking these therapies increased to 15%, 38%, 42%, and 19%, respectively. There were 7 deaths in Group I, none in Group II, and 1 in Group III over a mean follow-up time of 22.8(12.2) months. 1- and 2-year survival rates in Group I were 93% and 91%, respectively. Conclusion: Patients with SSc-associated PH have improved short-term outcomes compared with historical controls. Early diagnosis and treatment of all forms of SSc-associated PH may result in improved short-term outcomes.