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Table 1 Demographics and clinical characteristics of 625 patients with SSc at baseline

From: Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and meta-analysis of the literature

  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
  1. Results are expressed as n (%) for qualitative variables and mean ± SD or median [IQR] for quantitative variables
  2. N number of patients with available data, lcSSc limited cutaneous systemic sclerosis, mRSS modified Rodnan score, GFR glomerular filtration rate, AV block atrioventricular block, BB block bundle branch block, LVEF left ventricular ejection fraction, PH pulmonary hypertension, PAH pulmonary arterial hypertension, echo echocardiography, RHC right heart catheterization, 6MWD 6-min walking distance, sPAP systolic pulmonary arterial pressure, TLC total lung capacity, FVC forced vital capacity, DLCO diffusing capacity of the lungs for carbon monoxide, CRP C reactive protein, BMI body mass index, ACA anti-centromere antibodies, APL antiphospholipid antibodies