Characteristics | France/USA10 | Germany6 | Spain7 | Belgium8 | Brazil9 | Japan37 | China42 | Malaysia43 |
---|---|---|---|---|---|---|---|---|
No. study subjects | 127/247 | 1483 | 916 | 438 | 947 | 405 | 419 | 31 |
Female | 105 (83) /183 (81) | 1236 (83.4) | 796 (86.9) | 352 (80.4) | – | 376 (92.8) | – | 28 (90) |
Age, yrs | 48.4 ± 15.6/47.2 ± 13.7 | 55.7 ± 13.7 | 53.5 ± 14.2 | 55.4 ± 13.2 | – | – | – | 51.3 ± 12.8 |
Onset age, yrs | – | – | 45.9 ± 15.6 | 42.6 ± 14.1 | 47 ± 0.7 | – | 42.6 ± 13.4 | |
dcSSc | 24 (19)/116 (47) | 484 (32.7) | 243 (26.5) | 86 (19.6) | 235 (24.8) | 132 (32.6) | – | 9 (29) |
lcSSc | 69 (54)/104 (42) | 674 (45.5) | 568 (61.8) | 279 (63.7) | 712 (75.2) | 273 (67.4) | – | 22 (71) |
mRSS, mean ± SD or median (range) | – | 9.2 ± 9.2 | – | 4 (0–40) | – | – | – | – |
GERD | – | – | 526 (57.4) | 153 (35) | 897 (94.7) | 187 (46.2) | – | 23 (74.2) |
ILD | – | – | 418 (45.6) | 101 (23) | 538 (56.8) | 204 (50.4) | – | – |
Pulmonary fibrosis | 40 (36)/75 (34) | 512 (34.5) | – | – | – | – | 18 (58.1) | |
PAH | – | – | 161 (15.6) | 35 (8) | 221 (23.3) | 65 (16) | – | 18 (58.1) |
Isolated PAH | 9 (10)/18 (19) | 234 (15.8) | 43 (4.7) | – | – | – | – | – |
Renal crisis | 2 (2)/23 (9) | – | 24 (2.7) | 16 (3.7) | 25 (2.6) | 13 (3.2) | – | – |
ANA | 125 (98.4)/245 (99.1) | 1341 (90.4) | 840 (92.2) | 409 (95.6) | 839 (88.6) | – | – | – |
Anti-Scl-70 antibody | 45 (35)/54 (22) | 409 (27.6) | 173 (19.0) | 102 (23.9) | 152 (16.1) | 82 (23.3) | 251 (59.9) | 10 (32.2) |
ACA | 18 (23)/52 (21) | 539 (36.4) | 356 (39.1) | 177 (41.3) | 209 (22.1) | 127 (36.1) | 56 (13.4) | 3 (9.7) |
ARA | 4 (5)/61 (25) | – | – | 26 (6.1) | – | – | 4 (1.3) | 2 (6.5) |
SSc: systemic sclerosis; dcSSc: diffuse cutaneous SSc; lcSSc: limited cutaneous SSc; mRSS: modified Rodnan skin score; GERD: gastroesophageal reflux disease; ILD: interstitial lung disease; PAH: pulmonary arterial hypertension; ANA: antinuclear antibody; ACA: anticentromere antibody; ARA: anti-RNA polymerase III.