| Objective:Investigating the clinical Feature,treatment and prognostic factors in children with JDM, In order to guide the clinical work.Methods:79 children are retrospectively analyized by reviewing clinical data,including course, erythra, muscle strength, dry cough, arthralgia, rales, dysphagia, fever, edema, hypertension etc. Examination including electromyography, muscle biopsy, antibodies, erythrocyte ESR, CRP, muscle enzymes etc.And treatment during the period of hospitalization, including hormones, immunosuppressive therapy, and long-term follow-up after discharge. Statistical analysis was based on statistical software SPSS21.0, the packet count data adopted χ2 test, measurement data adopted F test, the relationship among variables adopted correlation analysis, multivariate analysis adopted Logistic regression analysis to define the independent risk factors, P<0.05 had statistical significance.Results:79 patients with JDM was statistical analysised, the results are as follows:1. The age of onset 1-14 years old,average age 7.6+3.6 years old, mainly occurred in school-age children, sex ratio:35/44.65 cases DM(82.3%), 12 cases CADM(15.2%),2 cases PM(2.5%). Muscle damage occurred in 47 cases (59.5%),32 cases (40.5%) appeared muscle pain expression, Gottron’s syndrome occurred in 45 cases (57%),1 cases (1.3%) occurred calcinosis cutis,29 cases (36.7%) showed different degrees of edema. Lung involved in 29 cases (36.7%),12 cases(15.2%) concurrented ILD. Examination:77 cases (97.3%) showed muscle enzyme increased in different degrees, Jo-1 antibody was positive in 6 patients (7.6%).54 cases of EMG,34 cases (63%) anomaly,28 cases muscle biopsy,14 cases (50%) anomaly. MRI was performed in 3 cases,2 cases (66.7%) showed length T2 shadow of the lesion muscle.2. DM, CADM often latent onset, or chronic or subacute onset, PM often showed acute onset, PM group showed more muscle symptoms, including Myalgia (χ2=11.968, P=0.003), decreased muscle strength (F=5.200, P=0.008), dysphagia (χ2=12.525, P=0.002) and LDH (F=5.070, P=0.009), electromyogram (χ2= 16.575, P=0.000), muscle biopsy (χ2=60.540, P=0.000) anomaly. Gottron’s syndrome (χ2=6.371, P=0.041) was common in DM group, fever (χ2=8.204, P=0.017) was more common in group CADM. Increased CK was negatively correlated with muscle strength (r=-0.293, P=0.009), CK was often associated with myogenic damage (χ2=20.871, P=0.007). Purple spots appear eyelid accompanied by decreased muscle strength (t=2.665, P＝0.009), Purple spots appear eyelid often accompanied with decreased muscle strength (t=2.665, P=0.009) and erythrocyte sedimentation rate increasing (t=2.017, P=0.047). The occurrence of skin edema often accompanied with serum IgG increasing(t=2.112, P=0.038).3. Multivariate Logistic regression analysis showed that cough (p=2.158, P=0.045, OR=14.9) and joint pain(β=1.913, P=0.024, OR=9.5) was positively associated with whether concurrent ILD.4. Treatment was based on glucocorticoid. Treatment combined with methylprednisolone (2=7.761, P=0.005) or hormone therapy (2=4.270, P=0.039) in children with severe situation, high risk or poor efficacy can significantly improve the curative effect, overall mortality was less than 3%.Conclusion:JDM has a better prognosis than adult, CK can be used as an indicator to assess skeletal muscle involvement and severity. The emergence of eyelid purple erythema was usually hint of disease activity, MRI examination can be used as a specific index for the assessment of disease activity. The prognosis of children with respiratory muscle involvement is poor, dry cough, joint pain, hypertension were dangerous factors of juvenile dermatomyositis complaint with interstitial lung disease.the evaluation of organ involvement is important for the diagnosis and the judgement of the disease. Early diagnosis and appropriate hormone or combined with immunosuppressive therapy will improve the prognosis. |
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