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Table 1 Cohort characteristics (n = 1303)

From: Significance of anti-neutrophil cytoplasmic antibodies in systemic sclerosis

Characteristic

Values

Female

1125 (86.3%)

Race

 Caucasian

1178 (90.4%)

 Asian

59 (4.5%)

 Aboriginal-Islander

15 (1.2%)

 Other

18 (1.4%)

Disease subtype

 Limited

974 (74.8%)

 Diffuse

329 (25.2%)

 Age at scleroderma onset, years

46.40 ± 14.26

 Age at recruitment, years

57.71 ± 12.54

 Disease duration at recruitment, years

11.26 ± 10.31

 Duration of follow-up, years

3.46 ± 2.69

 Ever smoked

639 (49.0%)

Autoantibodies

 ANCA

116 (8.9%)

 Anti-Scl-70

181 (13.9%)

 Anti-centromere

613 (47.0%)

 Antinuclear antibody

1222 (93.8%)

 Rheumatoid factor

337 (25.9%)

 Anti-Ro

87 (6.7%)

 Anti-La

20 (1.5%)

 Anti-RNP

26 (2.0%)

 Anti-dsDNA

42 (3.2%)

 Anti-Sm

8 (0.6%)

 Anti-Jo-1

7 (0.5%)

 Anti-Scl/PM

15 (1.2%)

 Anti-cardiolipin

262 (20.1%)

 Anti-beta 2 glycoprotein

94 (7.2%)

 Lupus anti-coagulant

35 (2.7%)

 Anti-RNA polymerase

116 (8.9%)

Clinical characteristics and complications

 Pulmonary arterial hypertension

163 (12.5%)

 Interstitial lung disease

311 (23.9%)

 Renal crisis

35 (2.7%)

 Digital ulcers

646 (49.6%)

 SIBO

37 (2.8%)

 GAVE

112 (8.6%)

 Oesophageal dysmotility

132 (10.1%)

 Oesphageal stricture

146 (11.2%)

 Synovitis

517 (39.7%)

 Malignancy

252 (19.3%)

 Hospitalisation

490 (37.6%)

 Deep vein thrombosis (DVT)

91 (7.0%)

 Pulmonary embolus (PE)

46 (3.5%)

 Overlap features with another connective tissue disease

76 (5.8%)

 Overlap syndrome with rheumatoid arthritis

27 (2.1%)

 Overlap syndrome with polymyositis

13 (1.0%)

 Overlap syndrome with Sjogren’s

24 (1.8%)

 Overlap syndrome with SLE

12 (0.9%)

 Overlap syndrome with dermatomyositis

1 (0.1%)

Treatments

 Prednisolone

580 (44.5%)

 Azathioprine

105 (8.1%)

 Mycophenolate

103 (7.9%)

 Cyclophosphamide

118 (9.1%)

 Calcium channel antagonist

847 (65.0%)

 Topical vasodilator

105 (8.1%)

 Iloprost

161 (12.4%)

 Penicillamine

108 (8.3%)

 Rituximab

11 (0.8%)

 Abatacept

1 (0.1%)

 TNF alpha inhibitor

11 (0.8%)

 Tocilizumab

5 (0.4%)

 Biologics

26 (2.0%)

  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