Scleroderma-specific antibodies and pulmonary function and echocardiography values in patients with Pre-PAH. Autoantibody data were available for 127/166 Pre-PAH patients. Pre-PAH patients were divided according to the 3 most commonly present autoantibodies to determine the association between scleroderma-specific antibodies and clinical PAH markers. The other patients did not have detectable autoantibodies or had an anti-U1-RNP, anti-RNA polymerase III, other, or no autoantibody information was available. A patient without an SSc-specific antibody who had multiple ANA patterns was characterized as “other.” There were no statistically significant differences between groups.
| Mean (SD) | ACA, n = 28 | Scl-70, n = 23 | Nucleolar, n = 17 |
|---|---|---|---|
| FVC, % predicted | 95 (16) | 79 (15) | 79 (16) |
| DLCO, % predicted | 56 (22) | 46 (16) | 51 (22) |
| FVC/DLCO | 1.78 (0.46) | 1.85 (0.52) | 1.74 (0.60) |
| RVSP, mm Hg | 37 (7) | 41 (10) | 39 (9) |
ACA: anticentromere, Scl-70: antitopoisomerase antibodies; ANA: antinuclear antibodies.