Table 1 Demographics and disease characteristics
Characteristc | Number (%) of subjects | Â | Characteristic | Number (%) with characteristic ever | Number (%) with characteristic at enrollment |
|---|---|---|---|---|---|
Sex | Â | Â | Elevated muscle enzymes | 86/89 (97%) | 6/89 (7%) |
Female | 80 (78%) | Â | Arthritis | 34/86 (40%) | 4/86 (5%) |
Male | 22 (22%) | Â | Calcinosis | 10/87 (12%) | 7/87 (8%) |
Ratio Female:male | 3.6:1 | Â | Cardiac involvement | 1/85 (1%) | 1/85 (1%) |
Race/Ethnicity | Â | Â | GI Ulceration | 3/85 (4%) | 1/85 (1%) |
White, Non-Hispanic | 65 (64%) | Â | Dysphagia/Dysphonia | 26/84 (31%) | 1/84 (1%) |
White, Hispanic | 13 (13%) | Â | ILD | 1/84 (1%) | 0/84 (0%) |
African American | 9 (9%) | Â | Muscle weakness: | Â | Â |
American Indian | 1 (1%) | Â | None | - | 62/91 (68%) |
Pacific Islander | 1 (1%) | Â | Mild | - | 23/91 (25%) |
Multi-racial | 10 (10%) | Â | Moderate | - | 4/91 (4%) |
Unknown | 3 (3%) | Â | Severe | - | 2/91 (2%) |
1° Family hx of autoimmunity | 21/92 (23%) |  | Periungual telangiectasia | - | 39/86 (45%) |
Positive ANA | 55/85 (65%) | Â | Contractures | - | 7/91 (8%) |
Chronology | Median, yrs (quartiles) | Range, yrs | V or shawl sign | - | 5/91 (6%) |
Age | 10.6 (7.1, 14.8) | 2.4-20.8 | Lipodystrophy | - | 4/91 (4%) |
Age of onset | 6.0 (3.3, 9.5) | 0.9-15.9 | Skin ulcer | - | 3/91 (3%) |
Disease duration | 3.0 (1.7, 5.7) | 0.2-12.8 | Malar or facial erythema | - | 24/90 (27%) |
Time to rhematologie care | 0.32 (0.13, 0.67) | 0-5.1 | Gottron sign, papules, or heliotrope | - | 44/91 (48%) |