Table 1.
General information at the first prescription of refractory JDM patients
| Feature | Number (%) or median [IQR] |
|---|---|
| Sex | |
| Male | 39 (44.3%) |
| Female | 49 (55.7%) |
| Ethnicity | |
| Han Chinese | 100% |
| Median age at disease onset | 82.0 ± 4.1 months |
| Manifestation at disease onset | |
| Skin involvement | 86 (97.9%) |
| Medium CAT activity score | 2.7 ± 0.1 |
| Medium CAT damage score | 2.7 ± 0.2 |
| Muscle involvement | 65 (73.8%) |
| Medium CMAS | 34.0 ± 1.2 |
| Pulmonary involvement (ILD) | 50 (56.8%) |
| Joint involvement | 33 (37.5%) |
| Digestive system involvement | 3 (3.4%) |
| Central nervous system involvement | 5 (5.6%) |
| Calcinosis | 17 (19.3%) |
| Myositis specific and associated antibodies | |
| Anti-TIF1γ | 5 (5.6%) |
| Anti-NXP2 | 12 (13.6%) |
| Anti-MDA5 | 19 (21.5%) |
| Anti-Mi2 | 1 (1.1%) |
| Anti-SNP | 1 (1.1%) |
| Anti-PL-7 | 1 (1.1%) |
| Anti-SSA | 5 (5.6%) |
| Anti-Ku | 1 (1.1%) |
| Anti-Jo | 1 (1.1%) |
| Other antibodies (anti-SNP) | 1 (1.1%) |
| Time at JAKi start | 22.0 ± 2.7 months |
| Duration of JAKi therapy | 10.1 ± 0.6 months |