PURPOSE: Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma, PHAROS, is a multi-center, prospective, observational study of scleroderma patients who are at high risk for PAH or who have definite PH by right heart catheterization. Purpose: To describe the relationship and predictive value of scleroderma specific auto-antibodies in this population.
METHODS: Data is collected bi-annually including clinical and objective PFT and echocardiogram data. Right-heart catheterization (RHC) is performed in patients suspected to have PH. Scleroderma autoantibodies (Ab) include anticentromere antibody (ACA), anti-Scl 70 (Scl70), a Nucleolar pattern on ANA testing (NUC), anti U1-RNP (U1RNP), RNA polymerase III (POLIII) and others.
RESULTS: Autoantibody data is available on 269 of the 350 patients entered into PHAROS. More only a small number had POLIII (p<0.0001). Patients with a NUC ab were more frequently entered as definite PH as opposed to Pre-PAH (p<0.01). ACA, U1RNP and NUC patients were significantly more likely to have PAH compared to the Scl 70 patients (p<0.0001) who more frequently had PH with an increased wedge pressure or associated with ILD. Consistent with this, the Scl 70 patients with PH, had a lower FVC 63% compared to the ACA (84%) and the NUC (75%) (p<0.05) and the FVC%/DLCO% ratio was not as high as in the other groups (1.67 vs 2.15 and 2.27) . The mean PAP of the Scl 70 group was lower, 30.7mmHg, compared to 35.1mmHg in ACA and 36.2 in NUC patients and the RVSP was higher 15mmHg compared to 12mmHg in the other patient groups.
CONCLUSION: Scleroderma patients with anti Scl 70 antibody are less likely to have PAH, and are more likely to have either PH associated with ILD or PVH. POL III patients rarely have PH.
CLINICAL IMPLICATIONS: Scleroderma autoantibodies are helpful in identifying patients who are more likely to have PAH.
DISCLOSURE: Virginia Steen, Grant monies (from sources other than industry) Sibley Foundation; Grant monies (from industry related sources) Gilead pharmaceutical; Consultant fee, speaker bureau, advisory committee, etc. Actelion, Gilead, United Therapeutics, Pfizer; No Product/Research Disclosure Information