APPENDIX 1.

Comparison of selected clinical and serological features of ssSSc subjects in the Canadian Scleroderma Research Group cohort compared to those in other large cohorts. Adapted from Marangoni, et al 5. Used with permission. All data are represented as n (%) unless specified otherwise.

Canada, n = 27Pittsburgh 20002, n = 48Germany 20083, n = 22Spain 20114, n = 69Brazil 20135, n = 79
Total n of cohort14175551483916947
  With ssSSc27 (2)48 (9)22 (1.5)69 (7.5)79 (8.3)
  Age at disease onset, mean (SD)44.4 (14.6)51 (range 17–78)44.9 (18.2)46.04 (13.1)
Female24/27 (88.9)41/48 (85)20/22 (90.9)62/69 (89.8)76/79 (96.2)
Digital ulcers5/27 (18.5)16/48 (33)7/22 (31.8)10/69 (14.5)19/79 (24.1)
Articular disease3/27 (11.5)21/48 (44)7/22 (31.8)9/69 (13)35/79 (44.3)
Muscular disease1/27 (3.7)2/48 (4)3/22 (13.6)2/69 (2.9)10/79 (12.7)
Esophageal dysmotility14/25 (56.0)37/48 (77)16/22 (72.7)31/69 (44.9)64/77 (83.1)
Interstitial lung disease7/27 (25.9)32/47 (68)16/22 (72.7)44/69 (63.7)37/65 (56.9)
Pulmonary hypertension3/26 (11.5)11/48 (23)3/22 (13.6)17/69 (24.0)18/79 (22.8)
Anticentromere antibodies9/18 (50.0)15/45 (33)8/22 (36.4)27/69 (24.6)33/79 (41.8)
Mortality rate4/20 (20)19/48 (40)6/69 (8.7)6/79 (7.6)
  • ssSSc: systemic sclerosis sine scleroderma.