Characteristics | Anti-RNAP3+ | Anti-RNAP3− | p | OR (95% CI) | Anti-RNAP3− Anti-Topo I+ | p§ | Anti-RNAP3− ACA+ | p§ |
---|---|---|---|---|---|---|---|---|
Age at disease onset, yrs, mean (SD), (n available) | 50.7 (13.9) (144) | 48.5 (13.2) (192) | 0.145 | 48.3 (12.6) (61) | 0.255 | 38.4 (13.0) (30) | 0.400 | |
Male | 34/150 (22.7) | 47/195 (24.1) | 0.755 | 19/65 (29) | 0.307 | 1/30 (3) | 0.011 | |
White | 36/39 (92.3) | 80/84 (95.2) | 0.678 | 44/47 (94) | 1.000 | 19/19 (100) | 0.544 | |
Country of origin | ||||||||
Italy | 43/158 | 54/199 | 0.115 | |||||
Swiss | 22 | 38 | ||||||
Sweden | 21 | 34 | ||||||
Hungary | 21 | 21 | ||||||
France | 27 | 28 | ||||||
Others | 24 | 24 | ||||||
Diffuse cutaneous subtype | 74/121 (61.2) | 114/168 (67.9) | 0.239 | 55/64 (86) | < 0.0001 | 9/29 (31) | 0.006 | |
Peak mRSS (0/51 to 51/51), (n available) | 21.3 (12.0) (95) | 18.6 (10.6) (157) | 0.131 | 22.3 (8.7) (52) | 0.594 | 9.9 (7.0) (21) | <0.0001 | |
Gastroesophageal reflux disease | 100/157 (63.7) | 155/199 (77.9) | 0.003 | 0.50 (0.30–0.82) | 52/65 (80) | 0.018 | 24/30 (80) | 0.095 |
Anorectal incontinence | 4/158 (2.5) | 5/199 (2.5) | 1.000 | 0/65 (0) | 0.325 | 2/30 (7) | 0.245 | |
SIBO requiring therapy | 6/157 (3.8) | 13/199 (6.5) | 0.344 | 2/65 (3) | 1.000 | 3/30 (10) | 0.159 | |
Primary biliary cirrhosis | 3/154 (1.9) | 2/198 (1.0) | 0.657 | 0/65 (0) | 0.557 | 0/30 (0) | 1.000 | |
GAVE | 13/157 (8.3) | 2/197 (1.0) | 0.0009 | 8.80 (1.85–57.4) | 0/65 (0) | 0.012 | 0/30 (0) | 0.133 |
Scleroderma renal crisis | 19/158 (12.0) | 5/199 (2.5) | 0.0005 | 5.30 (1.81–16.6) | 2/65 (3) | 0.043 | 0/30 (0) | 0.047 |
Death | 25/158 (15.8) | 31/198 (15.7) | 0.966 | 13/65 (20) | 0.441 | 1/30 (3) | 0.085 | |
Malignancies | 28/158 (17.7) | 18/199 (9.0) | 0.015 | 2.17 (1.15–4.08) | 4/65 (6) | 0.034 | 2/30 (7) | 0.176 |
Malignancies synchronous, −6/+12 mos | 11/158 (7.0) | 2/199 (1.0) | 0.004 | 7.38 (1.61–33.8) | 0/65 (0) | 0.004 | 0/30 (0) | 0.079 |
Malignancies nonsynchronous, < −6/> +12 mos | 17/158 (10.7) | 16/199 (8.0) | 0.486 | 4/65 (6) | 0.327 | 2/30 (7) | 0.745 | |
Malignancies synchronous, ± 2 yrs* | 14/155 (9.0) | 5/199 (2.5) | 0.007 | 3.85 (1.36–10.9) | 1/61 (2) | 0.073 | 0/30 (0) | 0.131 |
Malignancies nonsynchronous, < −2/> +2 yrs* | 14/155 (9.0) | 13/199 (6.5) | 0.423 | 3/61 (5) | 0.120 | 2/30 (7) | 1.000 | |
Solid tumors | 22/158 (13.9) | 12/199 (6.0) | 0.012 | 2.52 (1.21–5.27) | 4/65 (6) | 0.113 | 1/30 (3) | 0.133 |
Solid tumors synchronous, ± 2 yrs* | 13/155 (8.4) | 4/199 (2.0) | 0.010 | 4.46 (1.32–16.6) | 1/61 (2) | 0.120 | 0/30 (0) | 0.132 |
Breast cancers | 11/158 (7.0) | 4/199 (2.0) | 0.030 | 3.65 (1.14–11.7) | 1/65 (2) | 0.187 | 1/30 (3) | 0.694 |
Breast cancers synchronous, ± 2 yrs* | 7/155 (4.5) | 0/199 (0) | 0.003 | 20.2 (1.41–355) | 0/61 (0) | 0.195 | 0/30 (0) | 0.600 |
Solid tumors other than breast cancer synchronous, ± 2 yrs* | 6/155 (3.9) | 4/199 (2.0) | 0.344 | 1/61 (2) | 0.676 | 0/30 (0) | 0.591 | |
Hematologic malignancies, including leukemia | 1/158 (0.6) | 2/199 (1.0) | 0.702 | 0/65 (0) | 1.000 | 0/30 (0) | 1.000 | |
Nonmelanoma skin cancer | 3/158 (1.9) | 4/199 (2) | 0.940 | 0/65 (0) | 0.558 | 1/30 (3) | 0.504 | |
Melanoma | 2/158 (1.3) | 0/199 (0) | 0.195 | 0/65 (0) | 1.000 | 0/30 (0) | 1.000 |
↵* Patients with < 2 years of followup were excluded.
↵§ Compared with anti-RNAP3+ patients. SSc: systemic sclerosis; mRSS: modified Rodnan skin score; SIBO: small intestinal bacterial overgrowth; GAVE: gastric antral vascular ectasia; anti-RNAP3: anti-RNA polymerase III antibodies; anti-topo I: antitopoisomerase I; ACA: anticentromere antibodies.