title: The lung is involved in juvenile dermatomyositis.
abstract: 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
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Unité 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.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Pouessel
authors.forename: Antoine
authors.initials: A
authors.lastname: Deschildre
authors.forename: Muriel
authors.initials: M
authors.lastname: Le Bourgeois
authors.forename: Jean-Marie
authors.initials: JM
authors.lastname: Cuisset
authors.forename: Benoit
authors.initials: B
authors.lastname: Catteau
authors.forename: Chantal
authors.initials: C
authors.lastname: Karila
authors.forename: Véronique
authors.initials: V
authors.lastname: Nève
authors.forename: Caroline
authors.initials: C
authors.lastname: Thumerelle
authors.forename: Pierre
authors.initials: P
authors.lastname: Quartier
authors.forename: Isabelle
authors.initials: I
authors.lastname: Tillie-Leblond
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003882:Dermatomyositis / Q000188:drug therapy / Q000503:physiopathology*; D005260:Female; D005602:France; D006801:Humans; D007223:Infant; D008168:Lung / Q000503:physiopathology*; D008297:Male; D011446:Prospective Studies; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed
keywords: child; diaphragm; interstitial lung disease; juvenile dermatomyositis; lung