Table 1.
Clinical and laboratory characteristics of 26 JDM patients with anti-NXP2 autoantibodies: p values comparing Group A and B were non-significant for all variables.
| All | Group A | Group B | |
|---|---|---|---|
| Number of patients | 26 (19F/7M) | 11 (10F/1M) | 15 (9F/6M) |
| Ethnicity | 14c, 8as, 3aa, 1h | 5c, 4as, 2aa | 9c, 4as, 1aa, 1h |
| Mean age at disease presentation (y) | 6.5 (SD 3.7) | 5.2 (SD 2.9) | 7.5 (SD 3.8) |
| Age at study inclusion (y) | 11.1 (SD 4.6) | 11.5 (SD 4.2) | 10.8 (SD 4.8) |
| Median diagnosis delay (mo) | 4 (range 0.5–27) | 4 (range 0.5–24) | 3.6 (range 0.5–27) |
| Myositis (%) | 25 (96) | 11 (100) | 14 (93) |
| Gottron's papule (%) | 25 (96) | 11 (100) | 14 (93) |
| Typical rash (%) | 25 (96) | 11 (100) | 14 (93) |
| Lipodystrophy (%) | 5 (19) | 4 (36) | 1 (6) |
| Skin ulcerations (%) | 4 (15) | 3 (27) | 1 (6) |
| Polyarthritis (%) | 3 (11) | 2 (18) | 1 (6) |
| Gut involvement (%) | 3 (11) | 2 (18) | 1 (6) |
| Lung involvement (%) | 3 (11) | 1 (9) | 2 (13) |
| ESR (mean, mm) | 16.9 (SD 12.1) | 13.8 (SD 8.9) | 19.3 (SD 13.8) |
| Platelet count (mean,109/L) | 281 (SD 91.7) | 306.5 (SD 85.8) | 258.4 (SD 94.7) |
| CK (mean, U/L) | 1,700.3 (SD 2,508) | 1,548.6 (SD 2,397.7) | 1,811.6 (SD 2,664.9) |
| AST (mean, U/L) | 107.2 (SD 99.4) | 111.4 (SD 111.3) | 103.8 (SD 93.1) |
| ALT (mean, U/L) | 59.4 (SD 65.2) | 72.2 (SD 87.5) | 50.8 (SD 46.6) |
| LDH (mean, U/L) | 909.7 (SD 863.2) | 1,048.3 (SD 1,037.3) | 808.0 (SD 732.1) |
| IgG (mean, g/L) | 10.8 (SD 3.9) | 11.8 (SD 3.7) | 9.9 (SD 4.1) |
| ANA (number of positive patients) | 21 (80%) | 9 (81%) | 12 (80%) |
| MDA-5 | 1 | 1 | 0 |
| PM-SCL100 | 2 | 1 | 1 |
| Mi-2beta | 1 | 0 | 1 |
| SRP | 1 | 0 | 1 |
Group A—patients with calcinosis, Group B—patients without calcinosis.
F, female; M, male; y, year; mo, month; c, Caucasian; as, Asian; aa, African-American; h, Hispanic.