Clinical features and prognosis of seven cases with juvenile dermatomyositis associated interstitial lung disease
10.3760/cma.j.issn.1673-4912.2025.08.008
- VernacularTitle:幼年皮肌炎并肺间质病变七例临床特点及预后分析
- Author:
Xuan ZHANG
1
;
Tao XU
;
Chengcheng LIN
;
Xiangrong LIU
;
Yibing WANG
;
Guangmei CUI
;
Lili SUN
;
Qing SUN
Author Information
1. 青岛大学附属妇女儿童医院肾脏免疫科 266000
- Publication Type:Journal Article
- Keywords:
Interstitial lung disease;
Children;
Dermatomyositis;
High resolution computed tomography;
Anti- melanoma differentiation-associated gene 5 antibody
- From:
Chinese Pediatric Emergency Medicine
2025;32(8):601-605
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical features，diagnosis，treatment and prognosis of children with juvenile dermatomyositis(JDM) complicated with interstitial lung disease(ILD).Methods:The clinical manifestations，laboratory examination，treatment and prognosis of 7 children with JDM-ILD who were hospitalized in the Department of Nephrology and Immunology，Women and Children's Hospital Affiliated to Qingdao University from December 2019 to December 2023 were retrospectively analyzed.Results:Among the 7 cases，4 were male and 3 were female.The age of onset was 1.8-10.0 years（mean age 5.6 years），the occurrence time of pulmonary involvement was 0.6-4.0 months（mean time 2.0 months），and the follow-up time was 1.8-4.0 years.All the 7 cases had typical rash and different degrees of myasthenia.Four cases were accompanied by skin mucosal ulceration and 4 cases had fever during the course of the disease.Of the 7 cases，2 were accompanied by macrophage activation syndrome，and 1 of them had nervous system involvement，including convulsion and coma.All the children had increased creatase of varying degrees，and only 1 case had increased creatine kinase.Five cases had positive anti- melanoma differentiation-associated gene 5（MDA5）antibody and 4 cases had positive anti- Ro-52 antibody.Interleukin-6 was increased in 5 cases，interferon-γ was increased in 3 cases，and tumor necrosis factor-α was increased in 2 cases.Electromyography showed myogenic injury，MRI showed different degrees of myositis.Chest high-resolution CT showed ground glass shadow，rope shadow，consolidation shadow，pleural thickening，mesh shadow，etc.Four cases had limited lung function or mixed ventilation function restriction.All 7 cases received methylprednisolone pulse treatment combined with immunosuppressant treatment，and 5 cases received immunoglobulin treatment.Pulmonary lesions improved in 5 cases and partially improved in 1 case.One case died due to macrophage activation and multiple organ failure.Conclusion:The respiratory symptoms of JDM-ILD are obscure，and the incidence of ILD is high in children with anti-MDA5 antibody positive.High-resolution CT contributes to early diagnosis.Reasonable early application of glucocorticoid and immunosuppressants could improve the survival rate and quality of life.
