title: [Lung is also involved in juvenile dermatomyositis].
abstract: Juvenile dermatomyositis is the leading cause of chronic idiopathic inflammatory myopathy of auto-immune origin in children. Lung involvement in inflammatory myopathies is well described in adults, involving mostly interstitial lung disease, aspiration pneumonia and alveolar hypoventilation. We propose to describe its specificities in children. Pulmonary involvement may be asymptomatic and therefore must be systematically screened for. In case of clinical or functional respiratory abnormality, a chest computed tomographic (CT) scan is necessary. In children, a decrease of respiratory muscle strength seems common and should be systematically and specifically searched for by non-invasive and reproducible tests (sniff test). Interstitial lung disease usually associates restrictive functional defect, impairment of carbon monoxide diffusion and interstitial lung disease on CT scan. As in adults, the first-line treatment of juvenile dermatomyositis is based on corticosteroids. Corticosteroid resistant forms require corticosteroid bolus or adjuvant immunosuppressive drugs (methotrexate or cyclosporine). There is no consensus in pediatrics for the treatment of diffuse interstitial lung disease. Complications of treatment, including prolonged steroid therapy, are frequent and therefore a careful assessment of the treatments risk-benefit ratio is necessary, especially in growing children.
authors.affiliation: Unité de pneumologie et allergologie pédiatriques, pôle enfant, hôpital Jeanne-de-Flandre, CHRU de Lille, avenue Avinée, 59037 Lille, France; Service de pédiatrie, hôpital Victor-Provo, 59100 Roubaix, France.
authors.forename: G
authors.initials: G
authors.lastname: Pouessel
authors.affiliation: Unité de pneumologie et allergologie pédiatriques, pôle enfant, hôpital Jeanne-de-Flandre, CHRU de Lille, avenue Avinée, 59037 Lille, France.
authors.forename: C
authors.initials: C
authors.lastname: Thumerelle
authors.affiliation: Unité d'exploration fonctionnelle respiratoire pédiatrique, hôpital Jeanne-de-Flandre, CHRU de Lille, avenue Avinée, 59037 Lille, France.
authors.forename: V
authors.initials: V
authors.lastname: Nève
authors.affiliation: Service d'imagerie thoracique, hôpital Calmette, CHRU, 59037 Lille cedex, France.
authors.forename: T
authors.initials: T
authors.lastname: Santangelo
authors.affiliation: Unité de pneumologie et allergologie pédiatriques, pôle enfant, hôpital Jeanne-de-Flandre, CHRU de Lille, avenue Avinée, 59037 Lille, France; Service de pédiatrie, hôpital Victor-Provo, 59100 Roubaix, France.
authors.forename: S
authors.initials: S
authors.lastname: Flammarion
authors.affiliation: Unité de rhumatologie pédiatrique, pôle enfant, hôpital Jeanne-de-Flandre, CHRU de Lille, avenue Avinée, 59037 Lille cedex, France.
authors.forename: I
authors.initials: I
authors.lastname: Pruvot
authors.affiliation: Service de pneumologie et d'immuno-allergologie, hôpital Calmette, CHRU, 59037 Lille cedex, France.
authors.forename: I
authors.initials: I
authors.lastname: Tillie-Leblond
authors.affiliation: Unité de pneumologie et allergologie pédiatriques, pôle enfant, hôpital Jeanne-de-Flandre, CHRU de Lille, avenue Avinée, 59037 Lille, France. Electronic address: antoine.deschildre@chru-lille.fr.
authors.forename: A
authors.initials: A
authors.lastname: Deschildre
mesh_terms: D000328:Adult; D002648:Child; D003882:Dermatomyositis / Q000150:complications* / Q000188:drug therapy; D018450:Disease Progression; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008171:Lung Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D012129:Respiratory Function Tests
chemical_list: D007166:Immunosuppressive Agents
keywords: Child; Dermatomyosite; Dermatomyositis; Diaphragm; Diaphragme; Enfant; Interstitial lung disease; Pneumopathie infiltrante diffuse