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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.