Table 1

Patient demographics and clinical characteristics

Demographics/characteristicsTotal (n=2205)Type 0 (n=81)Type 1 (n=1070)Type 2 (n=197)Type 3 (n=857)p Value
Demographics
Sex
 Male374 (17.0)6 (7.4)140 (13.1)33 (16.8)195 (22.8)<0.001
 Female1831 (83.0)75 (92.6)930 (86.9)164 (83.3)662 (77.3)
Age at onset* (years)46.2±13.648.7±15.746.1±13.946.6±13.846.1±13.10.4
SSc duration* (years)5.6±7.26.1±6.97.4±8.26.0±7.43.4±4.7<0.0001
Follow-up time* (range, years)4.1±4.5 (0–26.3)1.8±2.6 (0–10.8)4.4±4.6 (0–26.3)4.3±4.5 (0–17.0)3.9±4.4 (0–25.6)<0.0001
Race
 White1652 (74.9)62 (76.5)875 (81.8)154 (78.2)561 (65.5)<0.001
 Black367 (16.6)12 (14.8)126 (11.8)24 (12.2)205 (23.9)<0.001
 Asian48 (2.2)3 (3.7)21 (2.0)1 (0.5)23 (2.7)0.2
 Other138 (6.3)4 (4.9)48 (4.5)18 (9.1)68 (7.9)0.005
Smoking status
 Never1102 (50.0)37 (45.7)523 (48.9)88 (44.7)454 (53.0)0.09
 Former822 (37.3)31 (38.3)410 (38.3)80 (40.6)301 (35.1)0.4
 Current251 (11.4)11 (13.6)121 (11.3)26 (13.2)93 (10.9)0.7
Known status1488 (67.5)46 (56.8)723 (67.6)127 (64.5)592 (69.1)0.1
 Deceased550 (37.0)24 (52.2)236 (32.6)48 (37.8)242 (40.9)0.003
Clinical characteristics
mRSS* (range 0–51)12.2±11.90.0±0.04.7±3.810.2±5.223.2±11.7<0.0001
Calcinosis585 (26.7)7 (8.8)316 (29.6)72 (36.7)190 (22.3)<0.001
Telangiectasias1916 (87.3)62 (77.5)991 (92.9)170 (86.7)693 (81.4)<0.001
Sicca1225 (55.9)35 (43.8)643 (60.3)123 (63.1)424 (49.8)<0.001
Raynaud's2146 (98.1)76 (98.7)1064 (99.7)188 (97.9)818 (96.1)<0.001
 RP score* (0–4)1.8±0.91.3±0.81.8±1.02.0±1.01.9±0.9<0.0001
 Score ≥3565 (25.8)8 (10.4)265 (24.8)64 (33.3)228 (26.8)0.001
GI disease1993 (90.9)67 (83.8)963 (90.4)180 (91.8)783 (91.9)0.09
 GI score* (0–4)1.4±0.81.2±0.91.4±0.81.5±0.91.5±0.90.0001
 Score ≥3214 (9.8)7 (8.8)82 (7.7)19 (9.7)106 (12.4)0.007
Heart disease500 (25.5)13 (22.8)255 (26.3)51 (30.7)181 (23.5)0.2
 Heart score* (0–4)0.8±1.40.6±1.30.8±1.50.9±1.60.7±1.40.2
Renal disease352 (17.8)8 (14.0)165 (17.0)29 (16.7)150 (19.3)0.5
 Renal score* (0–4)0.3±0.80.2±0.50.3±0.70.3±0.80.4±0.90.04
Muscle disease303 (13.9)3 (3.8)94 (8.8)18 (9.3)188 (22.2)<0.001
Joint disease1527 (69.8)30 (38.5)686 (64.3)136 (69.7)675 (79.6)<0.001
 Arthralgias1461 (66.7)29 (37.2)672 (63.0)129 (66.2)631 (74.3)<0.001
 Synovitis332 (15.2)6 (7.7)146 (13.7)38 (19.5)142 (16.8)0.02
 TFR371 (17.0)1 (1.3)26 (2.4)14 (7.2)330 (39.2)<0.001
ECHO-PH528 (29.4)16 (29.6)298 (33.0)46 (29.9)168 (24.6)0.005
RLD1474 (66.9)42 (51.9)640 (59.8)135 (68.5)657 (76.7)<0.001
FVC† (% predicted)70.2±20.878.9±21.773.5±20.871.0±20.565.0±19.7<0.0001
TLCO† (% predicted)61.3±25.168.6±28.963.6±25.461.1±23.657.9±24.2<0.0001
Autoantibody status
 ANA1397 (96.2)44 (95.7)704 (96.4)125 (97.7)524 (95.6)0.7
 ACA393 (27.5)16 (35.6)316 (44.1)37 (28.9)24 (4.4)<0.001
 Anti-RNP106 (8.5)4 (9.3)60 (9.8)13 (11.9)29 (6.0)0.08
 Anti-Scl-70304 (23.0)5 (12.8)108 (17.0)39 (32.8)152 (28.7)<0.001
  • All values are given as number (%) unless otherwise specified. Only patients with non-missing data were analysed for each variable. Online supplementary table S1 reports all the missing values for the sociodemographic and clinical parameters included in the study.

  • RP, GI, renal and heart severity scores are reported as previously defined by Medsger et al.24

  • *Mean±SD; †minimum recorded value for the percentage of predicted±SD.

  • ACA, anticentromere antibody; ANA, antinuclear antibody; ECHO-PH, pulmonary hypertension; FVC, forced vital capacity; GI, gastrointestinal; mRSS, modified Rodnan skin thickness score; RLD, restrictive lung disease; RNP, ribonucleoprotein; RP, Raynaud's phenomenon; Scl-70, topoisomerase I; SSc, systemic sclerosis; TFR, tendon friction rubs; TLCO, carbon monoxide transfer factor.