Analysis of single-center clinical data of juvenile dermatomyositis complicated with interstitial lung disease
10.3760/cma.j.issn.1673-4912.2025.10.010
- VernacularTitle:幼年皮肌炎合并间质性肺病单中心临床资料分析
- Author:
Lingling GENG
1
;
Yue PENG
1
;
Li WANG
1
;
Xiuhong XUE
1
;
Xinran WEN
1
;
Duomei SHI
1
;
Xiaoqing LI
1
Author Information
1. 西安交通大学附属儿童医院风湿免疫科 710003
- Publication Type:Journal Article
- Keywords:
Juvenile dermatomyositis;
Interstitial lung disease;
Myositis-specific antibodies;
Anti-MDA5 antibodies;
Biologics
- From:
Chinese Pediatric Emergency Medicine
2025;32(10):764-768
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To summarize the clinical data of single-center juvenile dermatomyositis（JDM）complicated with interstitial lung disease（ILD），and provide experience for pediatricians.Methods:Data of 61 children with JDM who were admitted to Children's Hospital affiliated to Xi'an Jiaotong University from January 2016 to May 2023 were collected. General data，clinical symptoms，chest high-resolution CT，laboratory examination and myositis antibody spectrum of the children were recorded.Results:Among the 61 children with JDM，there were 30 cases（13 males and 17 females）without ILD. The age of onset was 5.96（3.50，8.92）years and the course of disease was（11.79±20.00）months. There were 31 cases with ILD（14 males and 17 females），the age of onset was 7.42（4.50，10.08）years，and the duration of ILD was（5.47±8.09）months. There was statistical difference in the course of disease between the two groups（ P<0.05），but no statistical difference in gender and age between the two groups（ P>0.05）. Among 61 children with JDM，there were statistical differences in fever between the two groups（ P<0.05），but no statistical differences in heliotrope discoloration，gottron’s papules，calcinosis and myasthenia between the two groups（ P>0.05）. AST and FER showed statistical difference between the two groups（ P<0.05），while CK，LDH，CK-MB，ESR，C3 and C4 showed no statistical difference（ P>0.05）. All 61 cases of children were tested for myositis antibody spectrum，and there was statistical difference in anti-MDA5 antibody between the two groups（ P<0.05），but no statistical difference in the rest（ P>0.05）. There were statistical differences between the two groups in the treatment of methotrexate，hydroxychloroquine and cyclophosphamide（ P<0.05）. A total of 11 cases（36.67%）in the without ILD group were treated with biologics（8 adalimumab，2 infliximab and 1 tofacitinib），and 23 cases（74.19%）in the ILD group were treated with biologics（11 adalimumab，9 tofaciib，2 infliximab and 1 tocilizumab）. All 61 cases with JDM were followed up. Among the 30 children without ILD，1 case was lost to follow-up 2 months after treatment，and the rest were treated effectively without death. Among the 31 children with ILD，3 cases died of severe pulmonary infection with multidrug-resistant bacteria during treatment，of which 1 case was positive for anti-MDA5 antibody and 2 cases were negative for myositis specific antibody. Conclusion:JDM is more likely to be complicated with ILD，fever is more likely to occur in ILD group，and children with positive anti-MDA5 antibody are more likely to occur ILD. Biologic agents such as adalimumab and tofacitinib are effective in combination therapy. In the course of treatment，multi-drug resistant bacteria infection should be guarded against to reduce mortality.
