| Parameter . | iPAH patients (n = 32) . | Healthy subjects (n = 25) . | P . |
|---|---|---|---|
| General characteristics | |||
| Male/female (number) | 14/18 | 12/13 | 0.75 |
| Age (yr) | 33.3 ± 1.4 | 34.6 ± 2.2 | 0.73 |
| BMI (kg/m2) | 23.0 ± 0.8 | 23.2 ± 0.5 | 0.81 |
| Heart rate (beats per min) | 82.4 ± 2.1 | 79.3 ± 2.4 | 0.34 |
| 6MWD (m) | 439.6 ± 19.1 | — | — |
| mPAP (mm Hg) | 54.9 ± 3.5 | — | — |
| PCWP (mm Hg) | 9.5 ± 0.6 | — | — |
| CO (L/min) | 4.8 ± 0.4 | — | — |
| PVR (WU) | 11.2 ± 1.2 | — | — |
| mRAP (mm Hg) | 9.8 ± 0.9 | — | — |
| Proteinsa | |||
| IL-4 (pg/ml) | 309.1 ± 22.9 | 168.2 ± 16.9 | <0.01 |
| IL-5 (pg/ml) | 213.1 ± 13.7 | 116.6 ± 8.6 | <0.01 |
| IL-13 (pg/ml) | 255.2 ± 17.3 | 103.7 ± 11.9 | <0.01 |
| Periostin (ng/ml) | 54.0 ± 1.2 | 33.6 ± 1.3 | <0.01 |
| IgE (ng/ml) | 205.6 ± 11.1 | 63.2 ± 11.9 | <0.01 |
| Blood cells (%)b | |||
| CD4+ cells | 31.2 ± 1.7 | 18.9 ± 0.9 | <0.01 |
| CD8+ cells | 15.2 ± 1.1 | 13.8 ± 0.7 | 0.26 |
| CD4/CD8 | 2.1 ± 0.2 | 1.4 ± 0.1 | <0.01 |
| Eosinophil | 3.3 ± 0.3 | 2.0 ± 0.2 | <0.01 |
| Basophil | 8.4 ± 1.0 | 6.1 ± 0.6 | 0.08 |
| Mast | 0.2 ± 0.0 | 0.1 ± 0.0 | <0.01 |
| IlC2 | 1.2 ± 0.1 | 1.0 ± 0.1 | 0.12 |
| B cell | 3.5 ± 0.4 | 1.7 ± 0.2 | <0.01 |
| T reg | 2.7 ± 0.4 | 6.6 ± 0.6 | <0.01 |
| Gene expression (q-PCR)c | |||
| Tbx21 | (1.7 ± 0.2) × 10−3 | (1.4 ± 0.3) × 10−3 | 0.48 |
| GATA2 | (2.4 ± 0.2) × 10−3 | (2.0 ± 0.1) × 10−3 | 0.22 |
| GATA3 | (1.6 ± 0.1) × 10−2 | (0.7 ± 0.1) × 10−2 | <0.01 |
| RORC | (2.5 ± 0.3) × 10−3 | (2.3 ± 0.3) × 10−3 | 0.60 |
| Foxp3 | (1.5 ± 0.2) × 10−3 | (2.7 ± 0.4) × 10−3 | <0.01 |
| IL-4 | 0.27 ± 0.09 | 0.02 ± 0.01 | <0.01 |
| IL-5 | 0.21 ± 0.06 | 0.07 ± 0.01 | <0.01 |
| IL-13 | 0.28 ± 0.05 | 0.02 ± 0.01 | <0.01 |
| IFN-γ | 0.22 ± 0.06 | 0.19 ± 0.04 | 0.65 |
| TNF-α | 0.09 ± 0.01 | 0.08 ± 0.01 | 0.73 |
| IL-1β | 0.03 ± 0.01 | 0.03 ± 0.01 | 0.72 |
| Parameter . | iPAH patients (n = 32) . | Healthy subjects (n = 25) . | P . |
|---|---|---|---|
| General characteristics | |||
| Male/female (number) | 14/18 | 12/13 | 0.75 |
| Age (yr) | 33.3 ± 1.4 | 34.6 ± 2.2 | 0.73 |
| BMI (kg/m2) | 23.0 ± 0.8 | 23.2 ± 0.5 | 0.81 |
| Heart rate (beats per min) | 82.4 ± 2.1 | 79.3 ± 2.4 | 0.34 |
| 6MWD (m) | 439.6 ± 19.1 | — | — |
| mPAP (mm Hg) | 54.9 ± 3.5 | — | — |
| PCWP (mm Hg) | 9.5 ± 0.6 | — | — |
| CO (L/min) | 4.8 ± 0.4 | — | — |
| PVR (WU) | 11.2 ± 1.2 | — | — |
| mRAP (mm Hg) | 9.8 ± 0.9 | — | — |
| Proteinsa | |||
| IL-4 (pg/ml) | 309.1 ± 22.9 | 168.2 ± 16.9 | <0.01 |
| IL-5 (pg/ml) | 213.1 ± 13.7 | 116.6 ± 8.6 | <0.01 |
| IL-13 (pg/ml) | 255.2 ± 17.3 | 103.7 ± 11.9 | <0.01 |
| Periostin (ng/ml) | 54.0 ± 1.2 | 33.6 ± 1.3 | <0.01 |
| IgE (ng/ml) | 205.6 ± 11.1 | 63.2 ± 11.9 | <0.01 |
| Blood cells (%)b | |||
| CD4+ cells | 31.2 ± 1.7 | 18.9 ± 0.9 | <0.01 |
| CD8+ cells | 15.2 ± 1.1 | 13.8 ± 0.7 | 0.26 |
| CD4/CD8 | 2.1 ± 0.2 | 1.4 ± 0.1 | <0.01 |
| Eosinophil | 3.3 ± 0.3 | 2.0 ± 0.2 | <0.01 |
| Basophil | 8.4 ± 1.0 | 6.1 ± 0.6 | 0.08 |
| Mast | 0.2 ± 0.0 | 0.1 ± 0.0 | <0.01 |
| IlC2 | 1.2 ± 0.1 | 1.0 ± 0.1 | 0.12 |
| B cell | 3.5 ± 0.4 | 1.7 ± 0.2 | <0.01 |
| T reg | 2.7 ± 0.4 | 6.6 ± 0.6 | <0.01 |
| Gene expression (q-PCR)c | |||
| Tbx21 | (1.7 ± 0.2) × 10−3 | (1.4 ± 0.3) × 10−3 | 0.48 |
| GATA2 | (2.4 ± 0.2) × 10−3 | (2.0 ± 0.1) × 10−3 | 0.22 |
| GATA3 | (1.6 ± 0.1) × 10−2 | (0.7 ± 0.1) × 10−2 | <0.01 |
| RORC | (2.5 ± 0.3) × 10−3 | (2.3 ± 0.3) × 10−3 | 0.60 |
| Foxp3 | (1.5 ± 0.2) × 10−3 | (2.7 ± 0.4) × 10−3 | <0.01 |
| IL-4 | 0.27 ± 0.09 | 0.02 ± 0.01 | <0.01 |
| IL-5 | 0.21 ± 0.06 | 0.07 ± 0.01 | <0.01 |
| IL-13 | 0.28 ± 0.05 | 0.02 ± 0.01 | <0.01 |
| IFN-γ | 0.22 ± 0.06 | 0.19 ± 0.04 | 0.65 |
| TNF-α | 0.09 ± 0.01 | 0.08 ± 0.01 | 0.73 |
| IL-1β | 0.03 ± 0.01 | 0.03 ± 0.01 | 0.72 |
Values are presented as means ± SEMs. 6MWD, 6-min walking distance; BMI, body mass index; iPAH, idiopathic PAH; mPAP, mean pulmonary arterial pressure; CO, cardiac output; mRAP, mean right atrial pressure; PCWP, pulmonary capillary wedge pressure; PVR, pulmonary vascular resistance; WU, Woods units.
Levels of serum cytokines and IgE in human subjects were evaluated by ELISA. For iPAH patients, n = 25; for healthy subjects, n = 18.
Inflammatory cells in peripheral blood of human subjects were analyzed by flow cytometry. For iPAH patients, n = 6–7; for healthy subjects, n = 6–8.
Gene expression in human CD4+ T cells was determined by quantitative PCR (q-PCR). For iPAH patients, n = 6; for healthy subjects, n = 6.