Table 1 Demographics and clinical characteristics of 625 patients with SSc at baseline
| Â | N (N for dcSSc) | Total | dcSSc | lcSSc | p |
|---|---|---|---|---|---|
Demographics | |||||
 Female sex | 625/179 | 493 (79) | 124 (69) | 369 (83) | < 0.001 |
 Age at first RP (years) | 554/155 | 45.4 ± 15.7 | 45.8 ± 15.7 | 45.3 ± 15.8 | 0.736 |
 Age at first non-RP symptom (years) | 502/160 | 50.6 ± 14.5 | 48.5 ± 14.4 | 51.5 ± 14.4 | 0.031 |
 Age at diagnosis (years) | 625/179 | 52.7 ± 14.9 | 49.5 ± 14.5 | 53.9 ± 14.9 | < 0.001 |
 Disease duration from first non-RP symptom to diagnosis (years) | 499/160 | 0.8 [2.2] | 0.7 [1.4] | 0.9 [2.7] | 0.042 |
 Follow-up time from inclusion to death or last visit (years) | 625/179 | 4.4 [5.3] | 4.0 [5.2] | 4.8 [5.3] | 0.023 |
 Genetic background | |||||
  European | 503/147 | 453 (90) | 118 (80) | 335 (94) | < 0.001 |
  African | 503/147 | 50 (10) | 29 (20) | 21 (6) | < 0.001 |
Skin involvement | |||||
 lcSSc | 625/179 | 446 (71) | – | – |  |
 mRSS | 342/123 | 9.2 ± 10.2 | 19.6 ± 10.1 | 3.5 ± 3.6 | < 0.001 |
 Telangiectasia | 572/160 | 264 (46) | 65 (41) | 199 (48) | 0.112 |
 Calcinosis | 549/152 | 64 (12) | 7 (5) | 57 (14) | < 0.001 |
 Digital ulcers (past or active) | 538/145 | 161 (30) | 66 (46) | 95 (24) | < 0.001 |
Pulmonary involvement | |||||
 NYHA |  |  |  |  | 0.702 |
  Classes I–II | 515/150 | 425 (83) | 122 (81) | 303 (83) |  |
  Classes III–IV | 515/150 | 90 (17) | 28 (19) | 62 (17) |  |
 6MWD (meters) | 274/61 | 427 ± 127 | 432 ± 135 | 425 ± 125 | 0.705 |
 TLC < 70% predicted | 472/145 | 64 (14) | 33 (23) | 31 (9) | < 0.001 |
 FVC < 70% predicted | 475/148 | 82 (17) | 44 (30) | 38 (12) | < 0.001 |
 DLCO < 70% predicted | 471/141 | 249 (53) | 102 (72) | 147 (45) | < 0.001 |
 Interstitial lung disease | 582/166 | 262 (45) | 115 (69) | 147 (35) | < 0.001 |
PH (echo. and/or RHC) | 547/157 | 67 (12) | 18 (11) | 49 (13) | 0.775 |
 sPAP (echo.) |  |  |  |  | 0.004 |
  < 35 mmHg | 397/118 | 307 (77) | 89 (75) | 218 (78) |  |
  35–46 mmHg | 397/118 | 43 (11) | 21 (18) | 22 (8) |  |
  > 46 mmHg | 397/118 | 47 (12) | 8 (7) | 39 (14) |  |
 PAH (RHC) | 490/116 | 40 (8) | 4 (3) | 36 (10) | 0.033 |
Heart involvement | |||||
 Arrhythmia | 519/150 | 17 (3) | 5 (3) | 12 (3) | 1.000 |
 AV block | 512/146 | 7 (1) | 4 (3) | 3 (1) | 0.106 |
 BB block | 479/128 | 16 (3) | 6 (5) | 10 (3) | 0.388 |
 LVEF (%) | 402/102 | 64.9 ± 7.1 | 65.5 ± 8.6 | 64.7 ± 6.6 | 0.251 |
 Diastolic dysfunction | 423/110 | 20 (5) | 6 (5) | 14 (4) | 0.613 |
 Pericarditis | 478/136 | 32 (7) | 14 (10) | 18 (5) | 0.066 |
 Valvular disease | 430/111 | 29 (7) | 5 (5) | 24 (8) | 0.380 |
Renal involvement | |||||
 GFR < 80 ml/min | 459/136 | 179 (39) | 38 (28) | 141 (44) | 0.002 |
 Scleroderma renal crisis | 428/139 | 44 (10) | 31 (22) | 13 (5) | < 0.001 |
Gastrointestinal involvement | 611/172 | 429 (70) | 135 (78) | 294 (67) | 0.006 |
 BMI (kg/m2) | 514/159 | 24.4 ± 5.0 | 23.6 ± 4.0 | 24.7 ± 5.3 | 0.016 |
 Albuminemia < 35 g/l | 331/108 | 52 (16) | 28 (26) | 24 (11) | < 0.001 |
Muscular involvement | 604/172 | 137 (23) | 71 (41) | 66 (15) | < 0.001 |
 CPK > 200 IU/l | 250/82 | 66 (26) | 33 (40) | 33 (20) | < 0.001 |
Joint involvement | 598/172 | 291 (49) | 127 (74) | 164 (39) | < 0.001 |
Cancer | 625/179 | 49 (8) | 17 (10) | 32 (7) | 0.327 |
Hemoglobin | 559/163 | 13.0 ± 1.6 | 12.5 ± 1.6 | 13.1 ± 1.5 | < 0.001 |
Anemia | 559/163 | 127 (23) | 52 (32) | 75 (19) | 0.001 |
CRP > 8 mg/l | 470/136 | 118 (25) | 57 (42) | 61 (18) | < 0.001 |
Serologic features | |||||
 ACA | 557/151 | 221 (40) | 6 (4) | 215 (53) | < 0.001 |
 Anti-Scl70 antibodies | 504/149 | 177 (35) | 90 (60) | 87 (25) | < 0.001 |
 Anti-U1RNP antibodies | 342/63 | 15 (4) | 4 (6) | 11 (4) | 0.492 |
 Anti-RNAP3 antibodies | 345/72 | 18 (5) | 13 (18) | 5 (2) | < 0.001 |
 Anti-PMScl antibodies | 343/62 | 16 (5) | 3 (5) | 13 (5) | 1.000 |
 Anti-SSa antibodies | 387/79 | 60 (16) | 20 (25) | 40 (13) | 0.014 |
 Anti-SSb antibodies | 338/62 | 11 (3) | 3 (5) | 8 (3) | 0.431 |
 APL antibodies | 441/129 | 31 (7) | 16 (12) | 15 (5) | 0.007 |
 Low complement | 482/130 | 18 (4) | 6 (5) | 12 (3) | 0.589 |
Smoking | 572/158 | 215 (38) | 69 (44) | 146 (35) | 0.067 |