Anti-RNAP+ (n= 69) | Anti-RNAP- (n= 382) | ||||
---|---|---|---|---|---|
Characteristic | Totala | n(%) or mean ± SD | Totala | n(%) or mean ± SD | P value |
Sex | 69 | 382 | |||
Female | 58 (84.1%) | 339 (88.7%) | 0.27 | ||
Male | 11 (15.9%) | 42 (11.1%) | |||
Race | 65 | 373 | |||
Caucasian | 61 (93.9%) | 352 (94.4%) | 0.97 | ||
Asian | 3 (4.6%) | 14 (3.8%) | |||
Aboriginal-Islander | 1 (1.5%) | 7 (1.9%) | |||
Age (years) | |||||
At disease onsetb | 68 | 47.0 ± 11.7 | 373 | 46.4 ± 14.0 | 0.70 |
At recruitment | 68 | 56.0 ± 12.1 | 380 | 58.4 ± 12.4 | 0.14 |
Disease duration (years) | |||||
At recruitment | 68 | 9.0 ± 7.9 | 373 | 12.1 ± 10.3 | 0.02 |
At the time of the studyc | 68 | 11.4 ± 8.1 | 373 | 14.1 ± 10.7 | 0.06 |
Duration of follow-upc (years) | 59 | 1.9 ± 0.9 | 256 | 1.9 ± 0.8 | 0.97 |
Disease subtyped | 69 | 382 | |||
Limited | 17 (24.6%) | 302 (79.1%) | < 0.0001 | ||
Diffuse | 52 (75.4%) | 80 (20.9%) | |||
Disease manifestationse | |||||
Raynaud's phenomenon | 66 | 66 (100%) | 370 | 370 (100%) | 1.0 |
Digital ulcers | 69 | 35 (50.7%) | 382 | 159 (41.6%) | 0.16 |
Tendon friction rubs | 69 | 8 (11.6%) | 382 | 24 (6.3%) | 0.12 |
Joint contractures | 69 | 51 (73.9%) | 382 | 115 (30.1%) | < 0.0001 |
Highest mRSS during follow-upf | 68 | 20.6 ± 12.4 | 366 | 10.1 ± 7.9 | < 0.0001 |
Synovitis | 69 | 22 (31.9%) | 382 | 76 (19.9%) | 0.03 |
Myositis | 69 | 2 (2.9%) | 382 | 2 (0.5%) | 0.05 |
Renal crisisg | 69 | 17 (24.6%) | 382 | 7 (1.8%) | < 0.0001 |
Systemic hypertensionh | 69 | 41 (59.4%) | 375 | 149 (39.7%) | 1.0 |
Gastrointestinal involvementi | 69 | 69 (100%) | 382 | 379 (100%) | 0.75 |
Pulmonary arterial hypertensionj | 69 | 7 (10.1%) | 382 | 42 (11.0%) | 0.52 |
Interstitial lung diseasek | 69 | 19 (27.5%) | 382 | 120 (31.4%) | 0.80 |
Cardiac involvementl | 69 | 7 (10.1%) | 382 | 24 (6.3%) | 0.24 |
Serologic profilem | 69 | ||||
Antinuclear antibodies | 67 (97.1%) | 382 | 366 (95.8%) | 0.61 | |
Anti-centromere pattern | 4 (5.8%) | 381 | 193 (50.7%) | < 0.0001 | |
Anti-topoisomerase antibodies | 1 (1.5%) | 377 | 78 (20.7%) | 0.0001 | |
Anti-histidyl-tRNA synthetase antibodies | 0 (0%) | 377 | 0 (0%) | 1.0 | |
Anti-ribonucleoprotein antibodies | 1 (1.5%) | 376 | 3 (0.8%) | 0.6 | |
Anti-Ro antibodies | 3 (4.4%) | 377 | 24 (6.4%) | 0.52 | |
Anti-La antibodies | 0 (0%) | 377 | 6 (1.6%) | 0.29 | |
Anti-Smith antibodies | 44 | 0 (0%) | 376 | 1 (0.3%) | 0.67 |
Anti-double stranded DNA antibodies | 69 | 0 (0%) | 254 | 6 (2.4%) | 0.30 |
Anti-polymyositis-scleroderma antibodies | 64 | 0 (0%) | 378 | 4 (1.1%) | 0.39 |
Anti-neutrophil cytoplasmic antibodies | 64 | 10 (15.6%) | 373 | 63 (16.9%) | 0.80 |
Myeloperoxidase specificity | 64 | 4 (16.3%) | 372 | 8 (2.2%) | 0.06 |
Proteinase-3 specificity | 64 | 2 (13.1%) | 372 | 13 (3.5%) | 0.88 |
Rheumatoid factor | 65 | 8 (12.5%) | 359 | 118 (32.9%) | 0.001 |
Anti-phospholipid antibodies | 43 | 9 (13.9%) | 371 | 129 (34.8%) | 0.0008 |
Lupus anticoagulant | 69 | 0 (0%) | 282 | 7 (12.5%) | 0.30 |
Treatmente | 69 | 382 | |||
Corticosteroids | 40 (58.0%) | 158 (41.4%) | 0.01 | ||
Antimalarialsn | 10 (14.5%) | 64 (16.8%) | 0.64 | ||
Immunosuppressiveso | 40 (58.0%) | 103 (27.0%) | < 0.0001 | ||
Biological therapiesp | 1 (1.5%) | 4 (1.1%) | 0.77 | ||
Smoking ever | 65 | 30 (46.2%) | 371 | 153 (41.2%) | 0.44 |
Malignancye | 69 | 14 (13.0%) | 382 | 50 (13.2%) | 0.37 |
Solid organq | 9 (13.0%) | 26 (6.8%) | |||
Hematopoieticr | 0 (0%) | 7 (1.8%) | |||
Skin (nonmelanoma)s | 4 (5.8%) | 9 (2.4%) | |||
Melanoma | 1 (1.5%) | 5 (1.3%) | |||
Othert | 0 (0%) | 3 (0.8%) | |||
Malignancy diagnosed within 5 years of onset of SScu,v | 45 | 6 (13.3%) | 254 | 10 (3.9%) | 0.01 |
Time interval between SSc onset and malignancy diagnosis (years)u | 9 | 4.1 ± 3.1 | 37 | 11.7 ± 9.1 | 0.0002 |
Malignancy in inception cohorte,w | 31 | 120 | 0.42 | ||
Solid organq | 4 | 6 | |||
Hematopoieticr | 0 | 1 | |||
Skin (nonmelanoma)s | 1 | 3 | |||
Melanoma | 0 | 0 | |||
Othert | 0 | 0 |