Table 1.
Clinical characteristics of 26 JDM patients with anti-NXP2 autoantibody positive
| ID | gender | Age* | Duration | BMI (kg/m2) |
First symptom | CMAS | Dysphagia/ Hoarseness/ Lower Voice |
Ulcer/ Edema |
max CK (U/L) | SF (μg/ml) | ANA/ Anti-Ro-52 |
CD4/CD8 ratio | ILD | Gastro-intestinal | Treatment | Follow -up(m) |
Clinical partial response at 3rd month | CMAS at the end of the follow-up | ||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Rash | Weakness | both | ||||||||||||||||||
| 1 | M | 8 | 1 | 18.1 | Y | 23 | N | N/N | 1794 | 67 | N/N | 2.01 | N | N | 1 + 2 + 0 + 0 + 0 | 41 | N | 52 | ||
| 2 | M | 1 | 42 | 15.6 | Y | 16 | N | N/N | Nd | 48 | Y/N | 1.54 | N | N | 1 + 2 + 0 + 0 + 0 | 49 | N | 45 | ||
| 3 | M | 8 | 1 | 19.5 | Y | 23 | Y | N/N | 3873 | 202 | Y/N | 0.83 | N | N | 1 + 1 + 0 + 0 + 0 | 47 | Y | 52 | ||
| 4 | F | 11 | 7 | 15.2 | Y | 25 | N | N/N | Nd | 97 | N/N | 2.25 | N | N | 1 + 4 + 0 + 0 + 0 | 60 | Y | 52 | ||
| 5 | F | 9 | 3 | 21.9 | Y | 47 | Y | N/N | 8138 | 120 | N/N | 1.68 | N | N | 1* + 2 + I + 0 + 1 | 16 | Y | 47 | ||
| 6 | F | 4 | 6 | 24.6 | Y | 22 | Y | N/N | 535 | 132 | N/N | 2.03 | N | N | 1* + 3 + I + 0 + 0 | 15 | Y | 52 | ||
| 7 | F | 4 | 6 | 15.7 | Y | 47 | N | N/N | 516 | 50 | N/N | 2.23 | N | N | 1* + 1 + I + 2 + 1 | 14 | N | 47 | ||
| 8 | M | 3 | 12 | 15.7 | Y | 22 | N | Y/N | Nd | 141 | N/N | 1.52 | N | N | 1 + 0 + 0 + 0 + 0? | Nd | Nd | Nd | ||
| 9 | F | 6 | Nd | Nd | Y | 23 | N | N/N | 757 | Nd | N/N | 2.18 | N | N | 1 + 2 + 0 + 0 + 0 | 14 | Y | 52 | ||
| 10 | M | 1 | 2 | 17.9 | Y | 4 | Y | N/N | 621 | 474 | Y/N | 2.07 | N | N | 1* + 2 + I + 0 + 0 | 16 | N | 47 | ||
| 11 | F | 7 | 1 | 22.6 | Y | 28 | N | N/N | 922 | 14 | N/N | 1.7 | N | N | 1 + 3 + 0 + 0 + 0 | 51 | N | 52 | ||
| 12 | F | 2 | 1 | 14.1 | Y | 8 | N | N/N | 582 | 193 | N/N | 2.08 | Y | N | 1* + 1 + I + 0 + 0 | 13 | Y | 52 | ||
| 13 | F | 6 | 1 | 18.2 | Y | 0 | Y | N/N | 3327 | 178 | N/N | 2.3 | Y | N | 1* + 3 + I + 0 + 0 | 18 | Y | 52 | ||
| 14 | F | 5 | 1 | 14.9 | Y | 8 | N | N/N | 3392 | 279 | Y/Y | 1.28 | N | N | 1* + 2 + I + 0 + 0 | 22 | Y | 49 | ||
| 15 | F | 4 | 3 | 16.4 | Y | 0 | N | N/N | 1010 | 227 | N/Y | 1.7 | N | N | 1* + 3 + I + 0 + 0 | 34 | Y | 47 | ||
| 16 | F | 6 | 1 | 16.2 | Y | 0 | Y | N/Y | 15,140 | 783 | N/Y | 1.93 | N | N | 1* + 2 + I + 0 + 1 | 16 | Y | 40 | ||
| 17 | F | 13 | 1 | 29.1 | Y | 6 | Y | Y/N | 1156 | 239 | Y/N | 1.25 | Y | N | 1* + 3 + I + 1 + 1 | 31 | Y | 47 | ||
| 18 | F | 1 | 4 | 16.6 | Y | 25 | N | N/N | 221 | 362 | N/N | 2.33 | N | N | 1* + 3 + I + 1 + 0 | 29 | N | 45 | ||
| 19 | M | 1 | 3 | 17.5 | Y | 0 | Y | Y/Y | 1096 | 347 | N/Y | 1.16 | Y | N | 1* + 3 + I + 0 + 1 | 28 | N | 18 | ||
| 20 | M | 1 | 3 | 15.3 | Y | 0 | Y | Y/Y | 2000 | 185 | Y/Y | 0.81 | N | N | 1* + 4 + I + 1 + 1 | 12 | N | 33 | ||
| 21 | F | 1 | 3 | 13.9 | Y | 2 | Y | Y/Y | 1250 | 323 | Y/N | ND | N | Y | 1* + 5 + I + 1 + 1 + ASCT | 12 | N | 27 | ||
| 22 | F | 5 | 1 | 13.3 | Y | 0 | Y | Y/Y | 10,064 | 584 | Y/N | 0.75 | Nd | Y | 1* + 3 + I + 0 + 0 | 0.1 | Y | / | ||
| 23 | F | 3 | 2 | 13.6 | Y | 5 | Y | Y/Y | Nd | Nd | Y/N | Nd | N | Y | 1* + 3 + I + 0 + 0 | 43 | N | / | ||
| 24 | M | 4 | 1 | 16.1 | Y | 5 | Y | Y/Y | 118 | 132 | Y/N | 1.14 | Y | Y | 1* + 5 + I + 1 + 0 | 12 | N | / | ||
| 25 | F | 6 | 1 | 11.3 | Y | 2 | Y | Y/Y | 6457 | 416 | Y/Y | 0.68 | Y | Y | 1* + 3 + I + 0 + 0 | 4 | N | / | ||
| 26 | F | 9 | 1.5 | 12.8 | Y | 2 | Y | Y/Y | 3178 | 1566 | Y/Y | 1.74 | Y | Y | 1* + 1 + I + 1 + 0 | 1 | N | / | ||
Y yes, N no, Nd no data, Age age at onset, Duration time from onset to diagnosis, BMI body mass index, CMAS childhood myositis assessment score, CK creatine kinase, SF serum ferritin, ANA antinuclear antibody, ILD interstitial lung disease, Treatment MP + immunosuppressive agent(s) + IVIG + biological agents (not including JAK inhibitors) + JAK inhibitor, 1* MP + high dose MP pulse, I IVIG, ASCT autologous stem cell transplantation; Immunosuppressive agents: methotrexate (MTX), cyclophosphamide (CTX), cyclosporine (CsA), azathioprine, tacrolimus, mycophenolate mofetil (MMF), hydroxychloroquine, thalidomide, etc.