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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
  1. Values are given as number (%), mean ± SD or median (IQR)
  2. Abbreviations: ANA anti nuclear antibodies, ANCA anti-neutrophil cytoplasmic antibodies, anti-phospholipid antibodies: any one or combination of anti-cardiolipin, anti-beta-2-glycoprotein and lupus anticoagulant, anti-dsDNA anti-double stranded deoxyribonucleic acid, anti-RNP ribonucleoprotein, SIBO small intestinal bacterial overgrowth, SLE systemic lupus erythematosus, anti-Sm anti-Smith
  3. ^Rank sum test
  4. #Fisher’s exact test