Table 2.
Clinical features of anti-MDA5 DM described across various cohorts
| Cohort/country | Skin rash (%) | Skin ulcers (%) | ILD (%) | RP ILD (%) | CADM (%) | Arthritis (%) | Cancer (%) | Survival |
|---|---|---|---|---|---|---|---|---|
| Japan [8] | 50–75 | – | – | 50 | 100 | 50 | 0 | – |
| Japan [44] | 50–75 | 60 | 94 | 71 | 75 | – | 0 | 1 year, 60% |
| Japan [102] | – | – | – | 74 | 80 | – | 4 | 1 year, 68% (50% for those with RP ILD) |
| China [9] | 100 | 11 | 100 | 38 | 35 | – | – | 1 year, 40% |
| Korea [58] | – | – | 67 | 44 | 0 | – | 0 | – |
| Italy [47] | 40–80 | 20 | 60 | 20 | 100 | 0 | 0 | – |
| Spain [50] | – | 21 | 64 | 57 | 57 | – | 28 | 1 year, 70% |
| France [51] | 70 | 41 | 76 | 32 | 70 | 70 | 7 | Overall 70% (20% at 1 year with RP ILD) |
| USA (Stanford) [52] | 50–70 | 80 | 67 | 22 | 50 | 31 | – | – |
| USA (Pittsburgh) [49, 103] | 50–70 | 20 | 50 | 43.7 | 50 | 12 | – | 1 year, 70% |
| USA (Hopkins) [46] | 80–100 | 80 | 72 | 0 | 45 | 80 | – | Overall survival, 100% |
| India (adults) [104] | 80 | 20 | 60 | 40 | 0 | 60 | 0 | 1 year, 40% |
| India (children) [104] | 100 | 0 | 33 | 0 | 0 | 33 | 0 | 1 year, 100% |
| Canada [75] (70% Asians) | 100 | 42 | 100 | 38 | 57 | 53 | – | Overall survival, 80% (40% in those with RP ILD) |
| UK [105] | 70–80 | 50 | 10–19 | 0 | 12 | 86 | – | Overall survival, 100% |
| USA, Canada [106] | 60–97 | 30 | 25 | 5 | 0 | 90 | – | Overall Survival 97% |
| France [40] | 77 | 70 | 46 | 7 | – | 100 | – | Overall survival, 93% |
MDA5 melanoma differentiation-associated gene 5, DM dermatomyositis, USA United Sates of America, UK United Kingdom, ILD interstitial lung disease, RP rapidly progressive, – data unavailable