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Table 2 Demographic and clinical data of 26 patients with anti-NXP2 autoantibody positive JDM

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

Baseline characteristics

n (%) or Median (range)

Diagnosis

 JDM

26(100%)

Gender

 Male

9(34.6%)

 Female

17(65.4%)

Age at onset (y)

4.5(1–13)

Duration from onset to diagnosis (m)

2.0(1.0–42.0)

First symptom

 Rash

8(30.8%)

 Muscle weakness

5(19.2%)

 Rash & muscle weakness

13(50%)

Proximal muscle strength

  < = grade 3

18(69.2%)

  > grade 3

8(30.8%)

CMAS

7(0–47)

dysphagia/hoarseness/soft voice

15(57.7%)

Other signs

 Cutaneous ulceration

10(38.5%)

 Periorbital edema

5(19.2%)

 Calcification

3(11.5%)

Other organ systems

 Arthritis

8(30.8%)

 Interstitial lung disease

7(26.9%)

 Gastrointestinal involvement

6(23.1%)

Laboratory testing

 CK (ng/ml)

1203(118–15,140)

 Serum ferritin (ng/ml)

198(14–1566)

 ANA (+) only

8(30.8%)

 Anti-Ro52 antibody (+) only

3(11.5%)

 ANA (+) & Anti-Ro52 antibody (+)

4(15.4%)

Other examination

 MRI: myositis

26

 EMG: myogenic damage

18(81.8%)

 HRCT: interstitial lung disease

7(28%)

Treatmenta

 GC + IA≤2 ± (IVIG)

8(30.8%)

 GC + IA>2 ± (IVIG)

8(30.8%)

 GC + IA≤2 + IVIG+BA

4(15.4%)

 GC + IA> 2 + IVIG+BA

6(23.1%)

Follow-up(m)

27.5(1.0–106.0)

Refractory JDM

11(42.3%)

Death

5(19.2%)

  1. JDM juvenile dermatomyositis, JPM juvenile polymyositis, CMAS childhood myositis assessment score, CK creatine kinase, normal range: 50–220 U/L; serum ferritin normal range: 15–200 ng/ml, ANA antinuclear antibody, MRI magnetic resonance imaging, EMG electromyography, HRCT high-resolution computed tomography
  2. aGC: glucocorticoids including methylprednisolone and prednisolone; IA: immunosuppressive agents; IA≤2: two or less than 2 types of immunosuppressive agents; IA>2: more than 2 types of immunosuppressive agents; IVIG: intravenous immunoglobulin; BA: biological agents, including monoclonal antibodies and JAK inhibitors like tofacitinib. m: months for follow-up.