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%) |