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) |
|
| 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) |
|
|
| 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.