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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.