The lung is involved in juvenile dermatomyositis
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Published on: October 31, 2025
Guillaume Pouessel1, Antoine Deschildre, Muriel Le Bourgeois
1Unité de Pneumologie et Allergologie, Pôle de Pédiatrie, Centre de Compétence des Maladies Respiratoires Rares de L'enfant, Hôpital Jeanne de Flandre et Université Lille 2, CHRU de Lille, Lille, France; Service de Pédiatrie, Pavillon Médico-Chirurgical de Pédiatrie, Hôpital Victor Provo, 59056 Roubaix Cedex 1, France.
Insights
Respiratory issues are common in children with juvenile dermatomyositis (JDM). Systematic respiratory assessments, including pulmonary function tests (PFTs) and chest HRCT scans, are crucial for early detection and management of lung involvement in JDM patients.
Area of Science:
- Pediatric Rheumatology
- Pulmonology
- Autoimmune Diseases
Background:
- Juvenile dermatomyositis (JDM) is a primary autoimmune inflammatory myopathy in children.
- This study focuses on the respiratory status and treatment of pediatric JDM patients.
Purpose of the Study:
- To describe the respiratory status and treatment in children diagnosed with JDM.
- To highlight the prevalence and types of respiratory involvement in pediatric JDM.
Main Methods:
- A multicenter prospective study analyzing clinical data, pulmonary function tests (PFTs), and chest high-resolution computed tomography (HRCT) scans.
- Evaluated treatments and adverse effects in 21 JDM patients.
Main Results:
- 76% of patients exhibited respiratory involvement, including interstitial lung disease and respiratory muscle weakness.
- Chest HRCT revealed abnormalities in 12 out of 15 children, while PFTs showed ventilatory defects in 7 patients.
- Respiratory muscle tests were abnormal in 6 patients, indicating significant muscle involvement.
Conclusions:
- Systematic respiratory assessment, including PFTs and respiratory muscle strength testing, is recommended for children with JDM.
- Chest HRCT scans are advised for patients with respiratory symptoms or abnormal PFT results.
- Further longitudinal studies are needed to understand long-term outcomes and optimize treatment strategies.
Background:
Juvenile dermatomyositis (JDM) is the main cause of chronic idiopathic inflammatory myopathy of autoimmune origin in children. The aim of this multicenter prospective study was to describe respiratory status and treatment of children followed for JDM.
Methods And Patients:
Clinical manifestations, pulmonary function tests (PFT), chest high-resolution computed tomography (HRCT) scan results, and treatments and their adverse effects were analyzed in children followed for JDM.
Results:
Twenty-one patients (median age: 9.9 years; range: 20 months-18 years) were included. The median of disease duration at the time of the analysis was 3 years (range: 6 months-9 years 4 months). Overall 16 (76%) of 21 children presented with a respiratory involvement related to JDM including interstitial lung disease (n = 3) and/or respiratory muscle involvement (n = 7). Seven patients presented with other nonspecific manifestations. Three children had aspiration pneumonia. A chest HRCT was performed in 15 children, and abnormalities were observed in 12. PFT were performed in 20 of 21 patients. Seven showed functional abnormalities: restrictive ventilatory defect (n = 3) or obstructive ventilatory defect (n = 4). Six patients had abnormal respiratory muscle tests, including three with a restrictive ventilatory defect and one with an obstructive ventilatory defect. One other child with an acute aspiration pneumonia had a clearly muscle respiratory involvement but was too young to perform respiratory muscle tests and confirm this diagnosis. Treatment comprised systemic corticosteroid for all patients and adjuvant immunosuppressive therapy for 11. Adverse effects linked to treatment were reported in eight patients.
Conclusion:
The frequency of lung involvement in children with JDM justifies systematic respiratory assessment with PFT including measures of respiratory muscle strength. We suggest that a chest HRCT scan is indicated in cases of respiratory symptoms and/or PFT abnormalities. Longitudinal studies are needed to assess pediatric characteristics, long-term outcomes, and responses to treatment taking into account the risk-benefit ratio.
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