Characteristic | Totala | n(%) or mean ± SD |
---|---|---|
Sex | 451 | |
Female | 397 (88.0%) | |
Male | 54 (12.0%) | |
Race | 435 | |
Caucasian | 413 (94.3%) | |
Asian | 17 (3.9%) | |
Aboriginal-Islander | 8 (1.8%) | |
Age (years) | ||
At disease onsetb | 441 | 46.5 ± 13.7 |
At recruitment | 448 | 58.1 ± 12.4 |
Disease duration (years) | ||
At recruitment | 441 | 11.6 ± 10.0 |
At time of the studyc | 441 | 13.6 ± 10.3 |
Recruited within 2 years of SSc onsetb | 451 | 84 (18.6%) |
Recruited within 5 years of SSc onsetb | 451 | 151 (33.5%) |
Duration of follow-upc,d (years) | 315 | 1.9 ± 0.8 |
Disease subtypee | 451 | |
Limited | 319 (70.7%) | |
Diffuse | 132 (29.3%) | |
Disease manifestationsf | ||
Raynaud's phenomenon | 436 | 436 (100%) |
Digital ulcers | 451 | 194 (43.0%) |
Tendon friction rub | 451 | 32 (7.1%) |
Joint contractures | 451 | 166 (36.8%) |
Highest mRSS during follow-upg | 434 | 11.7 ± 9.6 |
Synovitis | 451 | 98 (21.7%) |
Myositis | 451 | 4 (0.9%) |
Renal crisish | 451 | 24 (5.4%) |
Systemic hypertensioni | 444 | 190 (42.8%) |
Gastrointestinal involvementj | 451 | 451 (100%) |
Pulmonary arterial hypertensionk | 451 | 49 (10.9%) |
Interstitial lung diseasel | 451 | 139 (30.8%) |
Cardiac involvementm | 451 | 31 (6.9%) |
Serologic profilen | ||
Antinuclear antibodies | 451 | 433 (96.0%) |
Anti-centromere pattern | 450 | 197 (43.8%) |
Anti-RNA polymerase III antibody | 451 | 69 (15.3%) |
Anti-topoisomerase antibodies | 446 | 79 (17.7%) |
Anti-histidyl-tRNA synthetase antibodies | 446 | 0 (0%) |
Anti-ribonucleoprotein antibodies | 445 | 4 (0.9%) |
Anti-Ro antibodies | 446 | 27 (6.1%) |
Anti-La antibodies | 446 | 6 (1.4%) |
Anti-Smith antibodies | 445 | 1 (0.2%) |
Anti-double-stranded DNA antibodies | 298 | 6 (2.0%) |
Anti-polymyositis-scleroderma antibodies | 447 | 4 (0.9%) |
Anti-neutrophil cytoplasmic antibodies | 437 | 73 (16.7%) |
Myeloperoxidase specificity | 436 | 12 (2.8%) |
Proteinase-3 specificity | 436 | 15 (3.4%) |
Rheumatoid factor | 423 | 126 (29.8%) |
Anti-phospholipid antibodies | 436 | 138 (31.7%) |
Lupus anticoagulant | 325 | 7 (2.2%) |
Treatmentf | 451 | |
Corticosteroids | 198 (43.9%) | |
Antimalarialso | 74 (16.4%) | |
Immunosuppressivesp | 143 (31.7%) | |
Biological therapiesq | 5 (1.1%) | |
Smoking ever | 436 | 183 (42.0%) |
Malignancyr | 451 | 64 (14.2%) |
Solid organs | 35 (7.8%) | |
Hematopoietict | 7 (1.6%) | |
Skin (nonmelanoma)u | 13 (2.9%) | |
Melanoma | 6 (1.3%) | |
Otherv | 3 (0.7%) | |
Time interval between SSc onsetw and malignancy diagnosis (years) | 9.3 ± 8.1 |