Table 4.
Treatment strategies for anti–MDA5 DM
| Drug/treatment | Type of study | n | Outcome |
|---|---|---|---|
| Dual therapy–CS + CYC/CNI | |||
| Gono et al. [102] | R | 8 | Overall survival, 75% |
| Muro et al. [113] | R | 11 | Overall survival, 72% |
| Upfront triple combination–CS + i.v. CYC + CNI | |||
| Gono et al. [102] | R | 12 | Overall survival, 41% |
| Kameda et al. [114] | P | 10 | 1 year survival, 50% |
| Matsuda et al. [88] | R | 8 | 1 year survival, 87.5% |
| Tsuji et al. [89] | P | 29 (15 from historical cohort) | 6 month survival, 89 vs 33% |
| Rituximaba | |||
| Ho so et al. [115] | R | 4 | 2 year survival,100% |
| Tokunaga et al. [116] | R | 2 | Overall survival, 0% |
| Basiliximabb | |||
| Zou et al. [117] | R | 4 | Overall survival, 75% |
| Plasma exchange (PE)a | |||
| Shirakshi et al. [94] | R | 8 of 13 received PE | 1 year survival, 60% vs 0 |
| Saito et al. [118] | R | 6 | 6 month survival, 77% |
| Abe et al. [93] | R | 6 of 10 received PE | 1 year survival, 100% vs 25% |
| Polymyxin B hemoperfusiona | |||
| Okabayashi et al. [119] | R | 14 | 3 month survival, 35.7% |
| Takada et al. [120] | R | 2 | 1 year survival, 50% |
| Case reports [121, 122] | 2 | Overall survival, 100% | |
| Tofacitinib | |||
| Kurasawa et al. [123] | R | 5 | Overall survival, 60% 6–month survival, 100% vs 78% |
| Chen et al. [43] | P | 18 (historical controls) | |
| Pirfenidone add–on | |||
| Li et al. [98] | P | 27 | 1–year survival, 73% vs 50%, subgroup analysis– no impact on survival in the acute ILD group |
| ECMO | |||
| Vuillard et al. [124] | R | 6 | Overall Survival, 0 |
| Hunag et al. | R | 3 | Survival, 100% (followed by lung transplant) |
CS corticosteroids, CYC cyclophosphamide, CNI calcineurin inhibitors, PE plasma exchange, R retrospective, P prospective, ECMO extra corporeal Membrane oxygenation
aRefractory to CS, CYC, CNI
bRefractory to CS, CNI