Baseline clinical, radiological, lung function and RHC data
| Total (N=206) | Normal (N=35) | Borderline (N=28) | Resting pH (N=143) | |||||
|---|---|---|---|---|---|---|---|---|
| Measure (N=number of patients; normal/borderline/resting PH) | Mean (SD) | Median | Mean (SD) | Median | Mean (SD) | Median | Mean (SD) | Median |
| NYHA classification, N (%) (N=27/26/125) | ||||||||
| 1=Dyspnoea with extreme activity | 40 (22.4) | 9 (33.3) | 8 (30.8) | 23 (18.4) | ||||
| 2=Dyspnoea with moderate activity | 79 (44.4) | 15 (55.6) | 12 (46.2) | 52 (41.6) | ||||
| 3=Dyspnoea with minimal activity | 59 (33.1) | 3 (11.1) | 6 (23.1) | 50 (40.0) | ||||
| Modified Rodnan skin score | 9.1 (8.5) | 6.0 | 8.8 (8.7) | 5.0 | 7.7 (6.0) | 5.5 | 9.5 (8.8) | 6.0 |
| Autoantibodies, N (%) (N=34/23/128) | ||||||||
| Anticentromere antibody | 43 (15.7) | 4 (11.8) | 4 (17.4) | 35 (27.3) | ||||
| Anti-Scl 70 antibody | 29 (15.7) | 8 (23.5) | 4 (17.4) | 17 (13.3) | ||||
| Anti-U1RNP antibody | 14 (7.6) | 5 (14.7) | 2 (8.7) | 7 (5.5) | ||||
| Anti-RNA polymerase III antibody | 5 (2.7) | 1 (2.9) | 0 (0) | 4 (3.1) | ||||
| ANA isolated nucleolar pattern | 49 (26.5) | 9 (26.5) | 5 (21.7) | 35 (27.3) | ||||
| ANA mixed or other staining pattern | 32 (17.3) | 4 (11.8) | 4 (17.4) | 24 (18.8) | ||||
| ANA negative | 13 (7.0) | 3 (8.8) | 4 (17.4) | 6 (4.7) | ||||
| Pulmonary function test (N=30/24/128) | ||||||||
| FVC% predicted | 74.7 (20.1) | 75.8 | 81.9 (18.9) | 82.8 | 69.3 (16.1)* | 66.0 | 74.1 (20.7) | 76.3 |
| FVC <65% predicted, N (%) | 59 (32.4) | 7 (23.3) | 10 (41.7) | 42 (32.8) | ||||
| FVC <70% predicted, N (%) | 74 (40.7) | 9 (30.0) | 16 (66.7)* | 49 (38.3) | ||||
| DLCO% predicted | 40.6 (16.1) | 37.7 | 45.7 (10.9) | 47.5 | 40.5 (18.1) | 38.2 | 39.4 (16.6) | 36.6 |
| FVC/DLCO | 2.1 (0.9) | 1.9 | 1.8 (0.3) | 1.9 | 2.1 (1.4) | 1.9 | 2.1 (0.9) | 2.0 |
| HRCT fibrosis, N (%) (N=24/20/88) | ||||||||
| None | 48 (36.4) | 9 (37.5) | 4 (20.0) | 35 (39.8) | ||||
| Mild | 36 (27.2) | 9 (37.5) | 8 (40.0) | 19 (21.6) | ||||
| Moderate | 33 (25.0) | 6 (25.0) | 6 (30.0) | 21 (23.9) | ||||
| Severe | 15 (11.4) | 0 (0) | 2 (10.0) | 13 (14.8) | ||||
| 6-Min walk test, m (N=25/23/109) | 353.7 (129.9) | 384.0 | 454.3 (82.6) | 457.2 | 393.3 (121.5) | 405.8 | 321.6 (127.0) | 341.9 |
| Echo RVSP, mm Hg (N=31/22/129)¶ | 53.0 (21.0) | 50.0 | 36.2 (8.7) | 35.0 | 46.3 (13.6)† | 44.0 | 58.1 (21.8) | 54.0 |
| RHC | ||||||||
| mPAP, mm Hg | 31 (12) | 29 | 17 (3) | 17 | 24 (5)† | 23 | 36 (11) | 31 |
| PCWP, mm Hg | 11 (5) | 10 | 8 (3) | 8 | 10 (3)† | 10 | 12 (5) | 11 |
| Cardiac output, l/min | 5.2 (1.5) | 5.1 | 5.7 (1.2) | 5.8 | 5.4 (1.3) | 5.3 | 5.1 (1.6) | 5.0 |
| PVR, dyn/s/cm−5 | 345 (251) | 258 | 137 (74) | 126 | 210 (110)† | 177 | 422 (260) | 348 |
| TPG, mm Hg | 20 (12) | 16 | 9 (3) | 9 | 13 (6)† | 13 | 24 (12) | 21 |
| Exercise RHC (N=18/16/25) | ||||||||
| mPAP, mm Hg | 37 (10) | 37 | 31 (7) | 31 | 39 (7)* | 40 | 41 (11) | 39 |
| PCWP, mm Hg | 14 (7) | 14 | 13 (6) | 11 | 17 (9) | 15 | 13 (6) | 14 |
| Cardiac output, l/min | 7.5 (2.5) | 7.0 | 8.7 (2.6) | 7.9 | 7.7 (1.9) | 7.2 | 6.7 (2.5) | 6.4 |
| PVR, dyn/s/cm−5 | 295 (215) | 215 | 175 (74) | 192 | 255 (215) | 212 | 391 (235) | 358 |
↵¶ Echo right ventricular systolic pressure on echocardiogram is the same as echo estimate systolic pulmonary arterial pressure in case of the absence of pulmonary valvular stenosis.
↵* p<0.05.
↵† p<0.002 between normal and borderline mPAP.
ANA, antinuclear antibody; DLCO, carbon monoxide diffusing capacity; FVC, forced vital capacity; HRCT, high-resolution CT; mPAP, mean pulmonary arterial pressure; NYHA, New York Heart Association; PCWP, pulmonary capillary wedge pressure; PVR, pulmonary vascular resistance; RHC, right heart catheterisation; RVSP, right ventricular systolic pressure on echocardiogram; TPG, transpulmonary gradient.