Table 4.
Characteristics of SSc-PAH and SSc-no PAH patients from the validation population.
| SSc-no PAH | SSc-PAH | |||
|---|---|---|---|---|
| N | Value | N | Value | |
| Demographic data | ||||
| Females, n (%) | 18 | 17 (94) | 18 | 17 (94) |
| Age at inclusion (years), mean ± SD | 18 | 70 ± 10 | 18 | 69 ± 9.3 |
| BMI (kg/m2), mean ± SD | 18 | 26 ± 5 | 18 | 25 ± 4.8 |
| Diagnosis of SSc | ||||
| SSc subtype | 18 | 18 | ||
| lcSSc, n (%) | 18 (100) | 18 (100) | ||
| dcSSc, n (%) | 0 (0) | 0 (0) | ||
| Immunological profile | 18 | 18 | ||
| Anti-centromere antibodies, n (%) | 18 (100) | 17 (94) | ||
| Anti-topoisomerase I antibodies, n (%) | 0 (0) | 0 (0) | ||
| Anti-RNA polymerase III antibodies, n (%) | 0 (0) | 0 (0) | ||
| Anti-fibrillarin, n (%) | 0 (0) | 1 (6) | ||
| Overlap with another connective tissue disease | 18 | 6 (33) | 18 | 6 (33) |
| Systemic lupus erythematosus, n (%) | 0 (0) | 0 (0) | ||
| Sjogren’s syndrome, n (%) | 6 (33) | 6 (33) | ||
| Inflammatory myopathy, n (%) | 0 (0) | 0 (0) | ||
| Rheumatoid arthritis, n (%) | 0 (0) | 0 (0) | ||
| Lymphoma (current or previous history), n (%) | 18 | 0 (0) | 18 | 0 (0) |
| Disease duration at inclusion | ||||
| Since diagnosis (years), mean ± SD | 18 | 14 ± 9 | 18 | 17 ± 9.8 |
| Since first non-Raynaud symptom (years), mean ± SD | 16 | 16 ± 7 | 17 | 18 ± 8.1 |
| Since Raynaud phenomenon onset (years), mean ± SD | 18 | 23 ± 11 | 18 | 29 ± 15 |
| History of organ involvements | ||||
| Interstitial lung disease | 18 | 18 | ||
| No ILD, n (%) | 14 (73) | 14 (73) | ||
| Limited ILD, n (%) | 4 (22) | 4 (22) | ||
| Extensive ILD, n (%) | 0 (0) | 0 (0) | ||
| ILD duration at inclusion (years), mean ± SD | 4 | 7 ± 4 | 4 | 10 ± 11 |
| Pulmonary hypertension, n (%) | 18 | 0 (0) | 18 | 18 (100) |
| Group 1, n (%) | 0 (0) | 18 (100) | ||
| Group 1’, n (%) | 0 (0) | 2 (15) | ||
| Group 2, n (%) | 0 (0) | 0 (0) | ||
| Group 3, n (%) | 0 (0) | 0 (0) | ||
| PH duration at inclusion (years), mean ± SD | 18 | 3 ± 2.3 | ||
| Scleroderma renal crisis, n (%) | 18 | 0 (0) | 18 | 0 (0) |
| History of digital ulcers, n (%) | 18 | 11 (61) | 18 | 12 (67) |
| Clinical evaluation at inclusion | ||||
| Modified Rodnan skin score, mean ± SD | 18 | 4.1 ± 3.8 | 18 | 7.1 ± 6.1 |
| NYHA functional class | 18 | 18 | ||
| Class I, n (%) | 8 (44) | 4 (22) | ||
| Class II, n (%) | 8 (44) | 3 (17) | ||
| Class III, n (%) | 1 (6) | 7 (39) | ||
| Class IV, n (%) | 1 (6) | 4 (22) | ||
| 6-minute walk distance (m), mean ± SD | 14 | 383 ± 86 | 17 | 298 ± 125 |
| 6-minute walk distance (% predicted), mean ± SD | 14 | 78 ± 20 | 17 | 59 ± 21 |
| Active Raynaud attacks at inclusion, n (%) | 18 | 10 (56) | 14 | 4 (29) |
| Active digital ulcers at inclusion, n (%) | 18 | 5 (28) | 18 | 2 (11) |
| Telangiectasias, n (%) | 18 | 16 (89) | 18 | 16 (89) |
| Calcinosis cutis, n (%) | 18 | 4 (22) | 15 | 6 (40) |
| Biological data | ||||
| ESR (mm/h), mean ± SD | 17 | 16 ± 8.6 | 16 | 19 ± 13 |
| CRP (mg/L), mean ± SD | 18 | 3.8 ± 1.8 | 18 | 6.6 ± 5.2 |
| Creatinin (mg/L), mean ± SD | 18 | 7.1 ± 1.6 | 18 | 8.7 ± 1.8 |
| Estimated GFR (mL/min/1,73m2), mean ± SD | 18 | 92 ± 29 | 18 | 73 ± 20 |
| Nt-pro-BNP (pg/mL), mean ± SD | 18 | 150 ± 123 | 18 | 950 ± 1241 |
| Ferritin (ng/mL), mean ± SD | 18 | 81 ± 73 | 18 | 58 ± 44 |
| Uric acid (mg/L), mean ± SD | 18 | 46 ± 11 | 17 | 61 ± 24 |
| Complement activation, n (%) | 18 | 0 (0) | 17 | 0 (0) |
| Transthoracic echocardiography | ||||
| Left ventricular ejection fraction (%), mean ± SD | 16 | 64 ± 8.2 | 16 | 66 ± 6.6 |
| Left ventricular diastolic dysfunction, n (%) | 16 | 4 (25) | 17 | 7 (41) |
| Peak TRV (m/s), mean ± SD | 14 | 2.46 ± 0.37 | 16 | 3.63 ± 0.65 |
| RA area (cm2), mean ± SD | 17 | 14 ± 4.8 | 18 | 21 ± 7.7 |
| Pulmonary function tests | ||||
| TLC (% predicted), mean ± SD | 17 | 104 ± 12 | 16 | 92 ± 9.3 |
| FVC (% predicted), mean ± SD | 18 | 117 ± 16 | 18 | 102 ± 17 |
| DLCO (% predicted), mean ± SD | 18 | 70 ± 13 | 18 | 38 ± 11 |
| KCO (% predicted), mean ± SD | 18 | 76 ± 13 | 18 | 45 ± 11 |
| FVC (% predicted)/DLCO (% predicted) ratio, mean ± SD | 18 | 1.73 ± 0.35 | 18 | 2.86 ± 0.76 |
| Right heart catheterization | ||||
| At PAH diagnosis | ||||
| Time between RHC and inclusion (months), mean ± SD | 18 | 34 ± 26 | ||
| RAP (mmHg), mean ± SD | 18 | 5.7 ± 3.2 | ||
| sPAP (mmHg), mean ± SD | 18 | 60 ± 16 | ||
| dPAP (mmHg), mean ± SD | 18 | 21 ± 6.5 | ||
| mPAP (mmHg), mean ± SD | 18 | 36 ± 10 | ||
| PAWP (mmHg), mean ± SD | 18 | 7.9 ± 2.3 | ||
| Cardiac output (L/min), mean ± SD | 18 | 4.7 ± 1.0 | ||
| Cardiac index (L/min/m²), mean ± SD | 18 | 2.8 ± 0.6 | ||
| SvO2 (%), mean ± SD | 16 | 69 ± 6 | ||
| PVR (Wood units), mean ± SD | 18 | 6.13 ± 2.38 | ||
| Latest available RHC data | ||||
| Time between RHC and inclusion (months), mean ± SD | 18 | 12 ± 14 | ||
| RAP (mmHg), mean ± SD | 17 | 5.5 ± 3.4 | ||
| sPAP (mmHg), mean ± SD | 17 | 57 ± 16 | ||
| dPAP (mmHg), mean ± SD | 17 | 26 ± 24 | ||
| mPAP (mmHg), mean ± SD | 18 | 35 ± 9.3 | ||
| PAWP (mmHg), mean ± SD | 18 | 9.1 ± 3.2 | ||
| Cardiac output (L/min), mean ± SD | 18 | 5.1 ± 1.3 | ||
| Cardiac index (L/min/m²), mean ± SD | 18 | 3.0 ± 0.6 | ||
| SvO2 (%), mean ± SD | 16 | 66 ± 9.6 | ||
| PVR (Wood units), mean ± SD | 18 | 5.6 ± 2.9 | ||
| Composite scores | ||||
| EScSG-AI score, mean ± SD | 18 | 1.17 ± 1.32 | 18 | 1.44 ± 1.21 |
| Medsger severity score, mean ± SD | 18 | 0.78 ± 1.48 | 16 | 1.26 ± 1.93 |
| sHAQ score, mean ± SD | 13 | 0.87 ± 0.67 | 15 | 1.01 ± 0.54 |
| Treatments at inclusion | ||||
| Corticosteroids, n (%) | 18 | 0 (0) | 18 | 0 (0) |
| Immunosuppressants, n (%) | 18 | 0 (0) | 18 | 0 (0) |
| Hydroxychloroquine, n (%) | 18 | 0 (0) | 18 | 0 (0) |
| PAH specific drugs*, n (%) | 18 | 1 (6) | 18 | 12 (67) |
| Phosphodiesterase 5 inhibitors, n (%) | 1 (6) | 12 (67) | ||
| Endothelin receptor antagonists, n (%) | 0 (0) | 8 (44) | ||
| Prostacyclin analogues, n (%) | 0 (0) | 1 (6) | ||
| Oxygen, n (%) | 18 | 0 (0) | 18 | 4 (22) |
ANA, antinuclear antibodies; BMI, body mass index; CRP, C-reactive protein; dc, diffuse cutaneous; DLCO, diffusing capacity of the lung for carbon monoxide; dPAP, diastolic pulmonary arterial pressure; EScSG-AI, European Scleroderma Study Group Activity Index; ESR, erythrocyte sedimentation rate; FVC, forced vital capacity; GFR, glomerular filtration rate; sHAQ, scleroderma Health Assessment Questionnaire; ILD, interstitial lung disease; KCO, diffusing coefficient for carbon monoxide; lc, limited cutaneous; mPAP, mean systolic pulmonary arterial pressure; Nt-pro-BNP, N-terminal prohormone of brain natriuretic peptide; NYHA, New York Heart Association; PAH, pulmonary arterial hypertension; PAWP, pulmonary arterial wedge pressure; PVR, pulmonary vascular resistance; RA, right atrium; RAP, right atrial pressure; sPAP, systolic pulmonary arterial pressure; SSc, systemic sclerosis; SvO2, venous saturation in oxygen; TLC, total lung capacity; TRV, tricuspid regurgitation velocity.
*In SSc-no PAH patients, these drugs were prescribed for digital ulcers and/or refractory Raynaud phenomenon.