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Table 1 Clinical characteristics of 26 JDM patients with anti-NXP2 autoantibody positive

From: Clinical characteristics and poor predictors of anti-NXP2 antibody-associated Chinese JDM children

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 /
  1. 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.