Table 2.
Characteristics and treatment outcome of anti-MDA5-DM-associated ILD patients treated with JAK inhibitors in the previous literatures
| JAK inhibitors (number of patients) | Tofacitinib (78), Peficitinib (1) |
| Gender, male (%) | 32 (40.5) |
| Age, years, mean (SD) | 52.9 (11.5) |
| Diagnosis (number of patients) | DM (22), CADM (49), NA (8) |
| CK, U/L, mean (SD), available number of patients | 212 (507), 40 |
| LD, U/L, mean (SD), available number of patients | 366 (178), 61 |
| CRP, mg/dL, mean (SD), available number of patients | 1.72 (2.24), 40 |
| Ferritin, ng/mL, mean (SD), available number of patients | 1304 (1438), 48 |
| KL-6, U/mL, median (IQR), available number of patients | 867 (501), 37 |
| Accompanied autoimmune diseases (number of patients) |
Psoriatic arthritis (1) Rheumatoid arthritis (1) |
| Positioning (number of patients) | Initial (29), Additional (22), Re-induction (2), NA (26) |
| Concomitant treatment (number of patients) | GC (78), CNI (32), CY (27), PE (20), RTX (11), IVIG (7), Pirfenidone (4), MMF (2), Unspecified (11) |
| Complications |
Viral infections (38) CMV (23), herpes zoster (7), others (8) Bacterial infections (12) Respiratory (6), sepsis (4), others (2) Fungal infections (14) Other events Cytopenia (15), Pneumomediastinum (3), Thrombotic microangiopathy (2), Renal dysfunction (1), Venous thrombosis (1), Alveolar proteinosis (1), Liver dysfunction (1), Hepatic failure (1), Shock of unknown cause (1), Intramuscular bleeding (1), Hip fracture (1) |
| Number of discontinuation cases due to complications | 11 |
| Number of survivors (%) | 60 (75.9) |
CADM clinically amyopathic dermatomyositis; CK creatinine kinase; CMV cytomegalovirus; CNI calcineurin inhibitors; CRP C-reactive protein; CY cyclophosphamide; DM dermatomyositis; GC glucocorticoid; IQR interquartile range; IVIG intravenous immunoglobulin; JAK Janus kinase; KL-6 Krebs von den Lungen-6; LD lactate dehydrogenase; MMF mycophenolate mofetil; NA not assessed; PE plasma exchange; RTX rituximab; SD standard deviation