Table 1.
Baseline clinical characteristics of study cohort.
| Variable | CTD (n = 117) | CTD-aPAH | P value′ | P value′′ | ||
|---|---|---|---|---|---|---|
| Total (n = 53) | Mild to moderate (n = 22) | Severe (n = 31) | ||||
| Gender | ||||||
| Female (%) | 81.2% (95) | 79.2% (42) | 77.3% (17) | 80.6% (25) | 0.766 | 0.765 |
| Age (years) | 61.0 ± 9.6 | 63.9 ± 8.5 | 64.0 ± 9.9 | 63.8 ± 7.5 | 0.058 | 0.921 |
| BMI (kg/m2) | 26.8 ± 2.6 | 26.1 ± 2.9 | 26.0 ± 3.2 | 26.2 ± 2.6 | 0.155 | 0.738 |
| FEV1% | 78.5 ± 10.4 | 71.3 ± 8.7 | 74.1 ± 6.3 | 69.2 ± 9.7 | <0.01 | 0.030 |
| DLCO% | 72.2 ± 8.5 | 68.3 ± 7.8 | 71.0 ± 8.4 | 66.5 ± 6.9 | <0.01 | 0.037 |
| mPAP (mmHg) | 21.0 ± 4.0 | 52.0 ± 8.5 | 48.9 ± 9.9 | 54.3 ± 6.6 | <0.01 | 0.032 |
| sPAP (mmHg) | 33.7 ± 5.0 | 71.0 ± 9.2 | 67.7 ± 7.0 | 73.4 ± 7.7 | <0.01 | 0.009 |
| PCWP (mmHg) | — | 12.2 ± 1.3 | 12.0 ± 1.3 | 12.3 ± 1.3 | — | 0.385 |
| CI (L/min/m2) | 2.9 ± 3.2 | 2.6 ± 3.3 | 2.8 ± 0.3 | 2.5 ± 0.3 | <0.01 | 0.005 |
| 6MWD (m) | 380.3 ± 75.1 | 294.3 ± 40.7 | 307.8 ± 42.0 | 284.7 ± 37.4 | <0.01 | 0.041 |
| WHO functional class | ||||||
| I (%) | 76.1% (89) | 35.8% (19) | 54.5% (12) | 22.6% (7) | <0.01 | 0.017 |
| II-III (%) | 23.9% (28) | 64.2% (34) | 45.5% (10) | 77.4% (24) | <0.01 | 0.017 |
| Raynaud's phenomenon (%) | 41.0% (48) | 58.5% (31) | 40.9% (9) | 71.0% (22) | 0.034 | 0.029 |
| Cause of CTD | ||||||
| SS | 29 | 31 | 12 | 19 | ||
| SLE | 42 | 6 | 2 | 4 | ||
| MCTD | 22 | 13 | 4 | 9 | ||
| RA | 21 | 1 | 0 | 1 | ||
| pSS | 3 | 2 | 1 | 1 | ||
P value′: CTD versus total CTD-aPAH; P value′′: mild to moderate CTD-aPAH versus severe CTD-aPAH. Data were presented as mean ± SD, n (%).
CTD: connective tissue diseases; CTD-aPAH: connective tissue diseases-associated pulmonary arterial hypertension; BMI: body mass index; FEV1%: forced expiratory lung volume in 1s (% predicted); DLCO%: carbon monoxide diffusion capacity (% predicted); mPAP: mean pulmonary artery pressure; sPAP: systolic pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; CI: cardiac index; 6MWD: 6-minute walk test distance; SS: systemic sclerosis; SLE: systemic lupus erythematosus; MCTD: mixed connective tissue disease; RA: rheumatoid arthritis; pSS: primary Sjogren's syndrome.