From: Significance of anti-neutrophil cytoplasmic antibodies in systemic sclerosis
 | Anti-MPO negative n = 1289 | Anti-MPO positive n = 13 | p | Anti-PR3 negative n = 1284 | Anti-PR3 positive n = 18 | p |
---|---|---|---|---|---|---|
Female | 1115 (86.5%) | 9 (69.2%) | 0.089# | 1112 (86.6%) | 12 (66.7%) | 0.014 |
Race | ||||||
 Caucasian | 1166 (90.5%) | 12 (92.3%) | 0.622# | 1164 (90.7%) | 14 (77.8%) | 0.024# |
 Asian | 57 (4.4%) | 1 (7.7%) |  | 54 (4.2%) | 4 (22.2%) |  |
 Aboriginal-Islander | 15 (1.2%) | 0 (0%) |  | 15 (1.2%) | 0 (0%) |  |
 Other | 18 (1.4%) | 0 (0%) |  | 18 (1.4%) | 0 (0%) |  |
Type of disease | ||||||
 Limited | 966 (74.9%) | 8 (61.5%) | 0.268 | 963 (75.0%) | 11 (61.1%) | 0.178 |
 Diffuse | 323 (25.1%) | 5 (38.5%) |  | 321 (25.0%) | 7 (38.9%) |  |
 Age of scleroderma onset, years | 46.44 ± 14.27 | 42.23 ± 14.26 | 0.290 | 46.39 ± 14.29 | 46.86 ± 13.24 | 0.888 |
 Age at recruitment, years | 57.76 ± 12.52 | 53.07 ± 14.02 | 0.180 | 57.71 ± 12.56 | 58.02 ± 11.17 | 0.916 |
 Disease duration at recruitment, years | 8.31 (12.31) | 13.07 (17.33) | 0.924^ | 8.29 (13.30) | 11.31 (16.86) | 0.934^ |
 Duration of follow-up, years | 3.09 (4.50) | 3.36 (3.48) | 0.619^ | 3.09 (4.50) | 3.97 (3.83) | 0.640^ |
 Ever smoked | 633 (49.1%) | 6 (46.2%) | 0.832 | 634 (49.4%) | 5 (27.8%) | 0.069 |
Autoantibodies | ||||||
 Anti-Scl-70 | 175 (13.6%) | 5 (38.5%) | 0.006 | 172 (13.4%) | 8 (44.4%) | < 0.001 |
 Anti-centromere | 612 (47.6%) | 1 (7.7%) | 0.004# | 608 (47.4%) | 5 (27.8%) | 0.092 |
 ANA | 1211 (93.9%) | 10 (76.9%) | 0.036# | 1205 (93.8%) | 16 (88.9%) | 0.285# |
 Rheumatoid factor | 335 (26.0%) | 2 (15.4%) | 0.532# | 333 (25.9%) | 4 (22.2%) | 0.793# |
 Anti-Ro | 87 (6.7%) | 0 (0.0%) | > 0.999# | 86 (6.7%) | 1 (5.6%) | > 0.999# |
 Anti-La | 20 (1.6%) | 0 (0.0%) | > 0.999# | 20 (1.6%) | 0 (0%) | > 0.999# |
 Anti-RNP | 26 (2.0%) | 0 (0.0%) | > 0.999# | 26 (2.0%) | 0 (0%) | > 0.999# |
 Anti-dsDNA | 41 (3.2%) | 1 (7.7%) | 0.385# | 40 (3.1%) | 2 (11.1%) | 0.157# |
 Anti-Sm | 8 (0.6%) | 0 (0.0%) | > 0.999# | 8 (0.6%) | 0 (0.0%) | > 0.999# |
 Anti-Jo-1 | 7 (0.5%) | 0 (0.0%) | > 0.999# | 7 (0.5%) | 0 (0.0%) | > 0.999# |
 Anti-PM/Scl | 15 (1.2%) | 0 (0.0%) | > 0.999# | 15 (1.2%) | 0 (0.0%) | > 0.999# |
 Anti phospholipid antibodies | 302 (23.4%) | 2 (15.4%) | > 0.999# | 397 (30.9%) | 7 (38.9%) | 0.105 |
 Anti-cardiolipin | 259 (20.1%) | 2 (15.4%) | > 0.999# | 234 (18.2%) | 7 (38.9%) | 0.600# |
 Anti-beta 2 glycoprotein | 93 (7.2%) | 1 (7.7%) | 0.539# | 93 (7.2%) | 1 (5.6%) | 0.668# |
 Lupus anticoagulant | 34 (2.6%) | 1 (7.7%) | 0.226# | 35 (2.7%) | 0 (0.0%) | > 0.999# |
 Anti-RNA polymerase | 113 (8.8%) | 3 (23.1%) | 0.136# | 116 (9.0%) | 0 (0.0%) | 0.149# |
Clinical characteristics and complications | ||||||
 Pulmonary arterial hypertension | 121 (9.4%) | 1 (7.7%) | > 0.999# | 118 (9.2%) | 4 (22.2%) | 0.080# |
 Interstitial lung disease | 305 (23.7%) | 5 (38.5%) | 0.213 | 301 (23.4%) | 9 (50.0%) | 0.009 |
 Renal crisis | 35 (2.7%) | 0 (0.0%) | > 0.999# | 35 (2.7%) | 0 (0.0%) | > 0.999# |
 Digital ulcers | 639 (49.6%) | 6 (46.2%) | 0.806 | 635 (49.5%) | 10 (55.6%) | 0.607 |
 SIBO | 37 (2.9%) | 0 (0.0%) | > 0.999# | 37 (2.9%) | 0 (0.0%) | > 0.999# |
 GAVE | 112 (8.7%) | 0 (0.0%) | 0.616# | 112 (8.7%) | 0 (0.0%) | 0.393# |
 Oesophageal dysmotility | 130 (10.1%) | 1 (7.7%) | > 0.999# | 130 (10.1%) | 1 (5.6%) | > 0.999# |
 Oesphageal stricture | 143 (11.1%) | 3 (23.1%) | 0.171# | 145 (11.3%) | 1 (5.6%) | 0.711# |
 Synovitis | 509 (39.5%) | 7 (53.8%) | 0.292 | 505 (39.3%) | 11 (61.1%) | 0.061 |
 Malignancy | 250 (19.6%) | 2 (15.4%) | > 0.999# | 249 (19.4%) | 3 (16.7%) | > 0.999# |
 Hospitalised | 484 (37.5%) | 6 (46.2%) | 0.524 | 481 (37.5%) | 9 (50.0%) | 0.276 |
 Deep vein thrombosis | 89 (6.9%) | 1 (7.7%) | 0.608# | 89 (6.9%) | 1 (5.6%) | > 0.999# |
 Pulmonary embolus | 44 (3.4%) | 1 (7.7%) | 0.368# | 42 (3.3%) | 3 (16.7%) | 0.022# |
 Overlap features with another connective tissue disease | 74 (5.7%) | 2 (15.4%) | 0.173# | 74 (5.8%) | 2 (11.1%) | 0.283# |
 Overlap syndrome with rheumatoid arthritis | 25 (1.9%) | 2 (15.4%) | 0.028# | 25 (1.9%) | 2 (11.1%) | 0.052# |
 Overlap syndrome with polymyositis | 13 (1.0%) | 0 (0.0%) | > 0.999# | 13 (1.0%) | 0 (0.0%) | > 0.999# |
 Overlap syndrome with Sjogren’s | 24 (1.9%) | 0 (0.0%) | > 0.999# | 24 (1.9%) | 0 (0.0%) | > 0.999# |
 Overlap syndrome with SLE | 12 (0.9%) | 0 (0.0%) | > 0.999# | 12 (0.9%) | 0 (0.0%) | > 0.999# |
 Overlap syndrome with dermatomyositis | 1 (0.1%) | 0 (0.0%) | > 0.999# | 1 (0.1%) | 0 (0.0%) | > 0.999# |
Treatments | ||||||
 Prednisolone | 570 (44.2%) | 9 (69.2%) | 0.093# | 568 (44.2%) | 11 (61.1%) | 0.153 |
 Azathioprine | 101 (7.8%) | 3 (23.1%) | 0.079# | 101 (7.9%) | 3 (16.7%) | 0.169# |
 Mycophenolate | 100 (7.8%) | 2 (15.4%) | 0.271# | 98 (7.6%) | 4 (22.2%) | 0.046# |
 Cyclophosphamide | 113 (8.8%) | 4 (30.8%) | 0.023# | 113 (8.8%) | 4 (22.2%) | 0.071# |
 Calcium channel antagonist | 836 (64.9%) | 10 (76.9%) | 0.364# | 833 (64.9%) | 13 (72.2%) | 0.516 |
 Topical vasodilator | 103 (8.0%) | 2 (15.4%) | 0.283# | 103 (8.0%) | 2 (11.1%) | 0.651# |
 Iloprost | 160 (12.4%) | 1 (7.7%) | > 0.999# | 159 (12.4%) | 2 (11.1%) | > 0.999# |
 Operation for peripheral vascular disease | 14 (1.1%) | 0 (0.0%) | > 0.999# | 14 (1.1%) | 0 (0%) | > 0.999# |
 Penicillamine | 105 (8.1%) | 3 (23.1%) | 0.086# | 105 (8.2%) | 3 (16.7%) | 0.183# |
 Rituximab | 10 (0.8%) | 1 (7.7%) | 0.105# | 10 (0.8%) | 1 (5.6%) | 0.142# |
 Abatacept | 1 (0.1%) | 0 (0.0%) | > 0.999# | 1 (0.1%) | 0 (0%) | > 0.999# |
 TNF alpha inhibitor | 11 (0.9%) | 0 (0.0%) | > 0.999# | 11 (0.9%) | 0 (0%) | > 0.999# |
 Tocilizumab | 5 (0.4%) | 0 (0.0%) | > 0.999 | 5 (0.4%) | 0 (0%) | > 0.999 |
 Biologics ever | 25 (1.9%) | 1 (7.7%) | 0.232# | 25 (1.5%) | 1 (5.6%) | 0.306# |