Table 2 Characteristics of ANCA-positive and ANCA-negative patients (n = 1303)
From: Significance of anti-neutrophil cytoplasmic antibodies in systemic sclerosis
|  | ANCA negative n = 1187 | ANCA positive n = 116 | p |
|---|---|---|---|
Female | 1028 (86.6%) | 97 (83.6%) | 0.372 |
Race | |||
 Caucasian | 1082 (91.2%) | 96 (82.8%) |  |
 Asian | 44 (3.7%) | 15 (12.9%) | 0.001# |
 Aboriginal-Islander | 14 (1.2%) | 1 (0.9%) |  |
 Other | 17 (1.4%) | 1 (0.9%) |  |
Type of disease | |||
 Limited | 890 (75.0%) | 84 (72.4%) |  |
 Diffuse | 297 (25.0%) | 32 (27.6%) | 0.544 |
 Age at scleroderma onset, years | 46.43 ± 14.16 | 45.95 ± 15.74 | 0.744 |
 Age at recruitment, years | 57.73 ± 12.31 | 57.54 ± 14.72 | 0.880 |
 Disease duration at recruitment, years | 8.29 (13.27) | 9.06 (13.84) | 0.891^ |
 Duration of follow-up, years | 3.07 (4.54) | 3.48 (3.48) | 0.174^ |
 Ever smoked | 597 (50.3%) | 42 (36.2%) | 0.004 |
Autoantibodies | |||
 Anti-Scl 70 | 152 (12.8%) | 29 (25.0%) | < 0.001 |
 Anti-centromere | 579 (48.8%) | 34 (29.3%) | < 0.001 |
 ANA | 1115 (93.9%) | 107 (92.2%) | 0.360 |
 Rheumatoid factor | 301 (26.7%) | 36 (31.0%) | 0.313 |
 Anti-Ro | 74 (6.2%) | 13 (11.2%) | 0.040 |
 Anti-La | 17 (1.4%) | 3 (2.6%) | 0.413# |
 Anti-RNP | 24 (2.0%) | 2 (1.7%) | > 0.999# |
 Anti-dsDNA | 34 (2.9%) | 8 (6.9%) | 0.062 |
 Anti-Sm | 7 (0.6%) | 1 (0.9%) | 0.527# |
 Anti-Jo-1 | 6 (0.5%) | 1 (0.9%) | 0.480# |
 Anti-Scl/PM | 14 (1.2%) | 1 (0.9%) | > 0.999# |
 Anti-cardiolipin | 231 (19.5%) | 30 (25.9%) | 0.801# |
 Anti-Beta 2 glycoprotein | 83 (7.0%) | 11 (9.5%) | 0.658 |
 Lupus anticoagulant | 31 (2.6%) | 4 (3.4%) | 0.778# |
 Anti-RNA polymerase | 105 (8.8%) | 11 (9.5%) | 0.409 |
Clinical characteristics and complications | |||
 Pulmonary arterial hypertension | 107 (9.0%) | 15 (12.9%) | 0.167 |
 Interstitial lung disease | 259 (21.8%) | 52 (44.8%) | < 0.001 |
 Renal crisis | 32 (2.7%) | 3 (2.6%) | > 0.999# |
 Digital ulcers | 587 (49.5%) | 59 (50.9%) | 0.772 |
 SIBO | 35 (2.9%) | 2 (1.7%) | 0.767# |
 GAVE | 107 (9.0%) | 5 (4.3%) | 0.085 |
 Oesophageal dysmotility | 124 (10.4%) | 8 (6.9%) | 0.226 |
 Oesphageal stricture | 137 (11.5%) | 9 (7.8%) | 0.218 |
 Synovitis | 454 (38.2%) | 63 (54.3%) | 0.001 |
 Malignancy | 221 (18.6%) | 31 (26.7%) | 0.035 |
 Hospitalised | 433 (36.5%) | 57 (49.1%) | 0.007 |
 Deep vein thrombosis (DVT) | 83 (7.0%) | 8 (6.9%) | 0.969 |
 Pulmonary embolus (PE) | 36 (3.0%) | 10 (8.6%) | 0.002 |
 Overlap features with another connective tissue disease | 62 (5.2%) | 14 (12.1%) | 0.003 |
 Overlap syndrome with rheumatoid arthritis | 22 (1.9%) | 5 (4.3%) | 0.076 |
 Overlap syndrome with polymyositis | 12 (1.0%) | 1 (0.9%) | > 0.999# |
 Overlap syndrome with Sjogren’s | 19 (1.6%) | 5 (4.3%) | 0.038 |
 Overlap syndrome with SLE | 9 (0.8%) | 3 (2.6%) | 0.084# |
 Overlap syndrome with dermatomyositis | 0 (0.0%) | 1 (0.9%) | 0.089# |
Treatments | |||
 Prednisolone | 520 (43.8%) | 60 (51.7%) | 0.052 |
 Azathioprine | 89 (7.5%) | 16 (13.8%) | 0.017 |
 Mycophenolate | 89 (7.5%) | 14 (12.1%) | 0.082 |
 Cyclophosphamide | 104 (8.8%) | 14 (12.1%) | 0.236 |
 Ca channel antagonist | 760 (64.0%) | 87 (75.0%) | 0.018 |
 Topical vasodilator | 93 (7.8%) | 12 (10.3%) | 0.343 |
 Iloprost | 150 (12.6%) | 11 (9.5%) | 0.324 |
 Penicillamine | 95 (8.0%) | 13 (11.2%) | 0.232 |
 Rituximab | 9 (0.8%) | 2 (1.7%) | 0.256# |
 Abatacept | 1 (0.1%) | 0 (0%) | > 0.999# |
 TNF alpha inhibitor | 9 (0.8%) | 2 (1.7%) | 0.256# |
 Tocilizumab | 4 (0.3%) | 1 (0.9%) | 0.373 |
 Biologics | 21 (1.8%) | 5 (4.3%) | 0.062 |