Table 1 Demographics and clinical characteristics of patients from systemic Scleroderma Toulouse cohort at baseline
| Â | Complete cohort n=375 | Diffuse cutaneous SSc n=63 | Limited cutaneous SSc n=279 | Sine scleroderma n=33 |
|---|---|---|---|---|
Demographics | ||||
 Female sex | 292 (77.9) | 38 (60) | 226 (81) | 28 (85) |
 Ethnic Group | ||||
  European | 256 (89.8) | 40 (80) | 190 (91.3) | 26 (96.3) |
  African | 21 (7.3) | 9 (18) | 11 (5.3) | 1 (3.7) |
  Asiatic | 8 (2.8) | 1 (2) | 7 (3.4) | 0 |
 Smoking | 129 (39.3) | 23 (42.6) | 95 (38.9) | 11 (36.6) |
 BMI (kg/m2) | 23.8 ±4.8 | 23.9 ±4.5 | 23.8 ±4.9 | 23.2 ±3.8 |
 Age at disease onset | 55.3 ±14.2 | 50.8 ± 13.4 | 56.4 ± 14 | 55.1 ± 15.4 |
 Age at first Raynaud’s syndrome | 45.9 ±16.6 | 48 ±13.6 | 45.9 ±16.9 | 43.5 ±17.8 |
 Disease duration (years) | 1.8 ±2.5 | 2.1 ±2.8 | 1.8 ±2.6 | 1.3 ±1.6 |
Raynaud’s syndrome | 362 (96.5) | 60 (95.2) | 268 (96) | 33 (100) |
Skin involvement | ||||
 mRSS | 6.8 ±9.4 | 19.2 ±11.1 | 5.3 ±6.3 | 0 |
 Calcinosis | 46 (12.4) | 10 (15.9) | 33 (12) | 3 (9) |
 Telangiectasias | 141 (38) | 14 (22.2) | 102 (37.1) | 25 (75.7) |
 Active digital ulcers | 57 (15.2) | 7 (11.1) | 48 (17.2) | 3 (9) |
 Previously reported digital ulcers | 53 (14.2) | 11 (17.5) | 38 (13.7) | 4 (12.1) |
Cardiac involvement | ||||
 ECG | ||||
  Bundle branch block | 21 (6.1) | 6 (10.5) | 14 (5.4) | 1 (3.2) |
  Arrhythmia | 29 (8.4) | 7/57 (12.3) | 21/258 (8) | 1 (3.3) |
 TTE |  |  |  |  |
  LVEF (in %) +/- SD | 66 ±9 | 65.6 ±9 | 66 ±9 | 67 ±7 |
  Diastolic dysfunction | 17 (5.6) | 1 (1.8) | 15 (6.7) | 1 (4.2) |
  Pericarditis | 18 (5.8) | 7 (12.5) | 9 (3.9) | 2 (8.3) |
  Valvular disease | 63 (20.4) | 7 (12.5) | 50 (21.8) | 6/ (25) |
PH | ||||
 PAPs ≥ 35 mm Hg (TTE) | 64 (36.9) | 12 (40) | 47 (34.3) | 5 (41.7) |
 PH (Right Catheterisation) | 18 (4.8) | 4 (6.4) | 11 (4.2) | 3 (9.3) |
Lung involvement | ||||
 Interstitial syndrome | 91 (25.4) | 22 (34.5) | 66 (25) | 3 (9.3) |
  FVC < 70% | 36 (12) | 11 (20) | 24 (10.9) | 1 (3.8) |
  DLCO <70% | 140 (46.9) | 36 (65.4) | 96 (43.8) | 8 (33.3) |
Digestive tract involvement | 243 (64.8) | 47 (74.6) | 180 (64.5) | 16 (48.5) |
Kidney involvement | ||||
 Renal crisis | 9 (2.4) | 3 (4.7) | 5 (1.8) | 1 (3) |
Rheumatological involvement | ||||
 Muscular signs | 66 | 19 (30.1) | 43 (15.4) | 4 (12.1) |
 Joint signs | 171 (45.6) | 37 (58.7) | 124 (44.4) | 10 (30.3) |
Neurological involvement | 89 (23.7) | 17 (26.9) | 65 (23.2) | 7 (21.2) |
Autoantibodies | ||||
 Anti-centromere | 185 (50.5) | 7 (11.6) | 152 (55.6) | 26 (78.8) |
 Anti-Scl70 | 87 (23.8) | 30 (48.4) | 53 (19.6) | 4 (12.1) |
 Anti-RNA pol III | 16 (4.3) | 4 (6.4) | 12 (4.4) | 0 |
 Anti-U1RNP | 4 (1.1) | 2 (3.2) | 2 (0.7) | 0 |
 Anti-PMScl | 7 (1.9) | 0 | 6 (2.2) | 1 (3) |
 Anti-TIF1y | 2 (0.5) | 0 | 2 (0.7) | 0 |
 Anti-SSA | 20 (5.5) | 2 (3.2) | 18 (6.6) | 0 |
 Anti-SSB | 12 (3.2) | 1 (1.6) | 11 (4) | 0 |
 ANCA | 5 (2.1) | 1/41 (2.4) | 4/169 (2.4) | 0/21 |
Laboratory parameters | ||||
 Hemoglobin (g/dl), | 13.2 ±1.5 | 13.1 ±1.6 | 13.2 ±1.5 | 13.5 ±1.4 |
 Anemia <12 g/dl | 54 (15.7) | 15 (25) | 35 (13.8) | 4 (13.3) |
 CRP >5 mg/l | 103 (32.8) | 24 (43.6) | 72 (31.2) | 7 (25) |
 Albumin <35 g/l | 34 (12.3) | 10 (20.8) | 23 (11.3) | 1 (4.3) |
Drug exposure | ||||
 Calcium channel blockers | 213 (56.8) | 39 (61.9) | 156 (55.9) | 18 (54.5) |
Immunosuppressants | ||||
 Corticosteroids | 135 (36) | 41 (65.1) | 92 (32.9) | 2 (6.1) |
 Other immunosuppressant | 120 (32) | 38 (60.3) | 78 (27.9) | 4 (12.1) |
  Cyclophosphamide | 44 (36.6) | 18 (47.3) | 25 (32) | 1 (25) |
  Methotrexate | 59 (49.1) | 14 (36.8) | 43 (55.1) | 2 (50) |
  Azathioprine | 25 (20.8) | 7 (18.4) | 17 (21.8) | 1 (25) |
  Mycophenolate | 62 (51.6) | 27 (71) | 34 (43.6) | 1 (25) |
  Rituximab | 20 (16.6) | 4 (10.5) | 13 (16.6) | 0 |