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 |