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. 2018 Aug 6;215(8):2175–2195. doi: 10.1084/jem.20171767

Table 1. Characteristics of iPAH patients.

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.

a

Levels of serum cytokines and IgE in human subjects were evaluated by ELISA. For iPAH patients, n = 25; for healthy subjects, n = 18.

b

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.

c

Gene expression in human CD4+ T cells was determined by quantitative PCR (q-PCR). For iPAH patients, n = 6; for healthy subjects, n = 6.