Oxidative stress-dependent activation of collagen synthesis is induced in human pulmonary smooth muscle cells by sera from patients with scleroderma-associated pulmonary hypertension

Table 1 Patient demographics and clinical characteristics

Variables	No PAH (N = 17)	PAH (N = 19)	HD (N = 14)	p value^§
Age at serum sampling (years)*	53.3 ± 11.6	64.0 ± 9.4	54.1 ± 10.4	0.009
Female	15 (88)	16 (84)	15 (85)	0.727
Race
White	14 (82)	16 (84)	12 (80)	0.881
Black	3 (18)	3 (16)	3 (20)	0.881
Smoking status
Never	9 (53)	10 (53)	8 (53)	0.280
Past	6 (35)	9 (47)	5 (33)
Current	2 (12)	0	2 (13)
SSc types
Limited	11 (65)	16 (84)		0.177
Diffuse	6 (35)	3 (16)		0.177
mRSS* (range 0–51)	5.5 ± 6.1	7.3 ± 10.3		0.567
SSc duration (RP onset)*, years	14.0 ± 12.6	21.7 ± 9.4		0.008
SSc duration (1st non-RP symptom)*, years	10.5 ± 7.3	18.5 ± 9.5		0.010
RP severity score* (range 0–4)	1.6 ± 0.8	2.0 ± 1.0		0.194
Heart severity score* (range 0–4)	0.2 ± 0.7	1.2 ± 1.7		0.062
Lung severity score* (range 0–4)	1.1 ± 1.3	3.1 ± 1.3		<0.001
Hemodynamics (RHC)
mPAP* (mm Hg)	NA	35.2 ± 8.1		NA
PCWP* (mm Hg)	NA	11.5 ± 4.0		NA
FVC* (% predicted)	81.9 ± 22.9	73.1 ± 9.9		0.149
DLCO* (% predicted)	78.2 ± 23.0	48.7 ± 16.8		<0.001
RLD^†	6 (35)	6 (32)		0.813
eRVSP*	24.0 ± 6.3	65.2 ± 19.9		<0.001
Autoantibody status
ACA	4 (24)	10 (53)		0.07
Anti-Scl-70	7 (41)	1 (5)		0.01
Anti-RNA-polymerase 3	2 (12)	0		0.124
Medication use (current)
Immunosuppressants^‡	5 (29)	5 (26)		0.836
Calcium channel blocker	10 (59)	7 (37)		0.187
Endothelin receptor antagonist	1 (6)	6 (32)		0.052
Phosphodiesterase 5 inhibitor	4 (24)	11 (58)		0.037
Prostanoid	0	0		NA
Statin	6 (35)	5 (26)		0.559
Aspirin	5 (29)	5 (26)		0.836

All values are given as number (%) unless otherwise specified.
*Mean ± SD. ^†The presence of RLD was defined by a FVC < 70% of predicted. ^‡Use of immunosuppressants include cyclophosphamide, mycophenolate, methotrexate, hydroxycholorquine or prednisone. ^§P values were determined by Fisher’s exact test or the Wilcoxon rank-sum test, as appropriate.
ACA, anticentromere antibody; DLCO, diffusion capacity of lung for carbon monoxide; eRVSP, estimated right ventricular systolic pressure by echocardiography; FVC, forced vital capacity; HD, healthy donors; mRSS, modified Rodnan skin score; mPAP, mean pulmonary artery pressure; PAH, pulmonary arterial hypertension, PCWP, pulmonary capillary wedge pressure, RHC, right heart catheterization, RLD, restrictive lung disease; RP, Raynaud’s phenomenon; Scl-70, topoisomerase I; SSc, systemic sclerosis.

ISSN: 1750-1172