The lung is involved in juvenile dermatomyositis
Identifieur interne : 000386 ( Istex/Corpus ); précédent : 000385; suivant : 000387The lung is involved in juvenile dermatomyositis
Auteurs : Guillaume Pouessel ; Antoine Deschildre ; Muriel Le Bourgeois ; Jean-Marie Cuisset ; Benoit Catteau ; Chantal Karila ; Véronique Nève ; Caroline Thumerelle ; Pierre Quartier ; Isabelle Tillie-LeblondSource :
- Pediatric Pulmonology [ 8755-6863 ] ; 2013-10.
English descriptors
- Teeft :
- Abnormality, Acute aspiration pneumonia, Adult patients, Adverse effects, Antoine deschildre, Arthritis rheum, Aspiration, Aspiration pneumonia, Assistance publique, Asymptomatic patients, Atelectasis, Autoimmune origin, Bronchial walls, Carbon monoxide, Chest hrct, Chest hrct scans, Childhood myositis assessment scale, Chronic aspiration pneumonia, Chronic idiopathic myopathy, Clinical manifestations, Cmas, Cmas score, Corticosteroid, Cyclo, Cyclophosphamide, Defect, Dermatomyositis, Disease activity, Dlco, Early stage, Expiratory, Expiratory volume, Femoral head, Foot osteomyelitis, Functional abnormalities, Gluteal region, High blood pressure, Hopital, Hopital jeanne, Hrct, Hrct results, Igiv, Inclusion duration, Intensive care unit, Interstitial, Interstitial lung disease, Interstitial pneumonia, Intravenous, Intravenous immunoglobulin, Intravenous methylprednisolone pulse therapy, Juvenile dermatomyositis, Lille, Linear atelectasis, Lower lobe, Lung biopsy, Lung disease, Lung involvement, Main cause, Maladies respiratoires rares, Maximal expiratory, Maximal expiratory pressure, Maximal inspiratory pressure, Months cyclo, Months hydroxychloroquine, Months igiv, Muscle function, Muscle involvement, Muscle strength, Mycophenolate mofetil, Myopathy, Nasal inspiratory pressure, National institute, Obstructive, Obstructive defect, Obstructive ventilatory defect, Oral corticosteroids, Pediatric, Pediatric pulmonology, Pneumonia, Polymyositis, Pulmonary function tests, Pulmonary involvement, Pulmonology, Radiological, Radiological abnormalities, Respir, Respiratory condition, Respiratory involvement, Respiratory manifestations, Respiratory muscle dysfunction, Respiratory muscle function, Respiratory muscle impairment, Respiratory muscle involvement, Respiratory muscle strength, Respiratory muscle tests, Respiratory muscle weakness, Respiratory status, Respiratory symptoms, Restrictive ventilatory defect, Rheum, Standard form, Systemic corticosteroids, Therapeutic approaches, Total lung capacity, Treatment regimens, Universite paris descartes, Ventilatory, Ventilatory defect, Vital capacity, Wiley periodicals.
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 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. Pediatr Pulmonol. 2013; 48:1016–1025. © 2012 Wiley Periodicals, Inc.
Url:
DOI: 10.1002/ppul.22742
Links to Exploration step
ISTEX:31A1C79BF240BB3958F08406044B25EC1F096D5ALe document en format XML
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Eblond, Isabelle" uniqKey="Tillie Eblond I" first="Isabelle" last="Tillie-Leblond">Isabelle Tillie-Leblond</name><affiliation><mods:affiliation>Service de Pneumologie et d'Immunologie‐Allergologie, Centre de Compétence des Maladies Pulmonaires Rares, Hôpital Calmette, CHRU de Lille, and INSERM U 1019, Institut Pasteur, Lille, France</mods:affiliation></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:31A1C79BF240BB3958F08406044B25EC1F096D5A</idno><date when="2013" year="2013">2013</date><idno type="doi">10.1002/ppul.22742</idno><idno type="url">https://api.istex.fr/ark:/67375/WNG-98DBKSM5-1/fulltext.pdf</idno><idno type="wicri:Area/Istex/Corpus">000386</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">000386</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main">The lung is involved in juvenile dermatomyositis</title><author><name sortKey="Pouessel, Guillaume" sort="Pouessel, Guillaume" uniqKey="Pouessel G" first="Guillaume" last="Pouessel">Guillaume Pouessel</name><affiliation><mods: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</mods:affiliation></affiliation><affiliation><mods:affiliation>Service de Pédiatrie, Pavillon Médico‐Chirurgical de Pédiatrie, Hôpital Victor Provo, 59056 Roubaix Cedex 1, France</mods:affiliation></affiliation></author><author><name sortKey="Deschildre, Antoine" sort="Deschildre, Antoine" uniqKey="Deschildre A" first="Antoine" last="Deschildre">Antoine Deschildre</name><affiliation><mods: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</mods:affiliation></affiliation><affiliation><mods:affiliation>E-mail: antoine.deschildre@chru-lille.fr</mods:affiliation></affiliation></author><author><name sortKey="Le Bourgeois, Muriel" sort="Le Bourgeois, Muriel" uniqKey="Le Bourgeois M" first="Muriel" last="Le Bourgeois">Muriel Le Bourgeois</name><affiliation><mods:affiliation>Service de Pneumologie et Allergologie Pédiatriques, Centre de Référence Des Maladies Respiratoires Rares de L'enfant, Hôpital Necker‐Enfants Malades, Université Paris Descartes, Assistance Publique des Hôpitaux de Paris, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Cuisset, Jean Arie" sort="Cuisset, Jean Arie" uniqKey="Cuisset J" first="Jean-Marie" last="Cuisset">Jean-Marie Cuisset</name><affiliation><mods:affiliation>Service de Neurologie Pédiatrique, Hôpital Salengro, CHRU de Lille, Lille, France</mods:affiliation></affiliation></author><author><name sortKey="Catteau, Benoit" sort="Catteau, Benoit" uniqKey="Catteau B" first="Benoit" last="Catteau">Benoit Catteau</name><affiliation><mods:affiliation>Service de Dermatologie, Hôpital Huriez, CHRU de Lille, Lille, France</mods:affiliation></affiliation></author><author><name sortKey="Karila, Chantal" sort="Karila, Chantal" uniqKey="Karila C" first="Chantal" last="Karila">Chantal Karila</name><affiliation><mods:affiliation>Service de Pneumologie et Allergologie Pédiatriques, Centre de Référence Des Maladies Respiratoires Rares de L'enfant, Hôpital Necker‐Enfants Malades, Université Paris Descartes, Assistance Publique des Hôpitaux de Paris, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Neve, Veronique" sort="Neve, Veronique" uniqKey="Neve V" first="Véronique" last="Nève">Véronique Nève</name><affiliation><mods:affiliation>Unité D'explorations Fonctionnelles Respiratoires Pédiatriques, Hôpital Jeanne de Flandre, CHRU de Lille, Lille, France</mods:affiliation></affiliation></author><author><name sortKey="Thumerelle, Caroline" sort="Thumerelle, Caroline" uniqKey="Thumerelle C" first="Caroline" last="Thumerelle">Caroline Thumerelle</name><affiliation><mods: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</mods:affiliation></affiliation></author><author><name sortKey="Quartier, Pierre" sort="Quartier, Pierre" uniqKey="Quartier P" first="Pierre" last="Quartier">Pierre Quartier</name><affiliation><mods:affiliation>Service d'Immunologie‐Hématologie et Rhumatologie Pédiatriques, Hôpital Necker‐Enfants Malades, Université Paris Descartes, Assistance Publique des Hôpitaux de Paris, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Tillie Eblond, Isabelle" sort="Tillie Eblond, Isabelle" uniqKey="Tillie Eblond I" first="Isabelle" last="Tillie-Leblond">Isabelle Tillie-Leblond</name><affiliation><mods:affiliation>Service de Pneumologie et d'Immunologie‐Allergologie, Centre de Compétence des Maladies Pulmonaires Rares, Hôpital Calmette, CHRU de Lille, and INSERM U 1019, Institut Pasteur, Lille, France</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">Pediatric Pulmonology</title><title level="j" type="alt">PEDIATRIC PULMONOLOGY</title><idno type="ISSN">8755-6863</idno><idno type="eISSN">1099-0496</idno><imprint><biblScope unit="vol">48</biblScope><biblScope unit="issue">10</biblScope><biblScope unit="page" from="1016">1016</biblScope><biblScope unit="page" to="1025">1025</biblScope><biblScope unit="page-count">10</biblScope><date type="published" when="2013-10">2013-10</date></imprint><idno type="ISSN">8755-6863</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">8755-6863</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>Abnormality</term><term>Acute aspiration pneumonia</term><term>Adult patients</term><term>Adverse effects</term><term>Antoine deschildre</term><term>Arthritis rheum</term><term>Aspiration</term><term>Aspiration pneumonia</term><term>Assistance publique</term><term>Asymptomatic patients</term><term>Atelectasis</term><term>Autoimmune origin</term><term>Bronchial walls</term><term>Carbon monoxide</term><term>Chest hrct</term><term>Chest hrct scans</term><term>Childhood myositis assessment scale</term><term>Chronic aspiration pneumonia</term><term>Chronic idiopathic myopathy</term><term>Clinical manifestations</term><term>Cmas</term><term>Cmas score</term><term>Corticosteroid</term><term>Cyclo</term><term>Cyclophosphamide</term><term>Defect</term><term>Dermatomyositis</term><term>Disease activity</term><term>Dlco</term><term>Early stage</term><term>Expiratory</term><term>Expiratory volume</term><term>Femoral head</term><term>Foot osteomyelitis</term><term>Functional abnormalities</term><term>Gluteal region</term><term>High blood pressure</term><term>Hopital</term><term>Hopital jeanne</term><term>Hrct</term><term>Hrct results</term><term>Igiv</term><term>Inclusion duration</term><term>Intensive care unit</term><term>Interstitial</term><term>Interstitial lung disease</term><term>Interstitial pneumonia</term><term>Intravenous</term><term>Intravenous immunoglobulin</term><term>Intravenous methylprednisolone pulse therapy</term><term>Juvenile dermatomyositis</term><term>Lille</term><term>Linear atelectasis</term><term>Lower lobe</term><term>Lung biopsy</term><term>Lung disease</term><term>Lung involvement</term><term>Main cause</term><term>Maladies respiratoires rares</term><term>Maximal expiratory</term><term>Maximal expiratory pressure</term><term>Maximal inspiratory pressure</term><term>Months cyclo</term><term>Months hydroxychloroquine</term><term>Months igiv</term><term>Muscle function</term><term>Muscle involvement</term><term>Muscle strength</term><term>Mycophenolate mofetil</term><term>Myopathy</term><term>Nasal inspiratory pressure</term><term>National institute</term><term>Obstructive</term><term>Obstructive defect</term><term>Obstructive ventilatory defect</term><term>Oral corticosteroids</term><term>Pediatric</term><term>Pediatric pulmonology</term><term>Pneumonia</term><term>Polymyositis</term><term>Pulmonary function tests</term><term>Pulmonary involvement</term><term>Pulmonology</term><term>Radiological</term><term>Radiological abnormalities</term><term>Respir</term><term>Respiratory condition</term><term>Respiratory involvement</term><term>Respiratory manifestations</term><term>Respiratory muscle dysfunction</term><term>Respiratory muscle function</term><term>Respiratory muscle impairment</term><term>Respiratory muscle involvement</term><term>Respiratory muscle strength</term><term>Respiratory muscle tests</term><term>Respiratory muscle weakness</term><term>Respiratory status</term><term>Respiratory symptoms</term><term>Restrictive ventilatory defect</term><term>Rheum</term><term>Standard form</term><term>Systemic corticosteroids</term><term>Therapeutic approaches</term><term>Total lung capacity</term><term>Treatment regimens</term><term>Universite paris descartes</term><term>Ventilatory</term><term>Ventilatory defect</term><term>Vital capacity</term><term>Wiley periodicals</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">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. Pediatr Pulmonol. 2013; 48:1016–1025. © 2012 Wiley Periodicals, Inc.</div></front></TEI><istex><corpusName>wiley</corpusName><keywords><teeft><json:string>dermatomyositis</json:string><json:string>hrct</json:string><json:string>abnormality</json:string><json:string>juvenile dermatomyositis</json:string><json:string>ventilatory</json:string><json:string>lille</json:string><json:string>pediatric</json:string><json:string>cyclo</json:string><json:string>expiratory</json:string><json:string>interstitial lung disease</json:string><json:string>hopital</json:string><json:string>obstructive</json:string><json:string>intravenous</json:string><json:string>pulmonology</json:string><json:string>corticosteroid</json:string><json:string>igiv</json:string><json:string>lung involvement</json:string><json:string>myopathy</json:string><json:string>dlco</json:string><json:string>radiological</json:string><json:string>cyclophosphamide</json:string><json:string>atelectasis</json:string><json:string>respir</json:string><json:string>linear atelectasis</json:string><json:string>respiratory muscle strength</json:string><json:string>respiratory muscle involvement</json:string><json:string>cmas</json:string><json:string>months cyclo</json:string><json:string>respiratory involvement</json:string><json:string>obstructive ventilatory defect</json:string><json:string>restrictive ventilatory defect</json:string><json:string>mycophenolate mofetil</json:string><json:string>aspiration pneumonia</json:string><json:string>pulmonary function tests</json:string><json:string>adverse effects</json:string><json:string>vital capacity</json:string><json:string>pediatric pulmonology</json:string><json:string>intravenous immunoglobulin</json:string><json:string>intravenous methylprednisolone pulse therapy</json:string><json:string>rheum</json:string><json:string>lung disease</json:string><json:string>arthritis rheum</json:string><json:string>hopital jeanne</json:string><json:string>acute aspiration pneumonia</json:string><json:string>respiratory symptoms</json:string><json:string>polymyositis</json:string><json:string>interstitial</json:string><json:string>pulmonary involvement</json:string><json:string>carbon monoxide</json:string><json:string>muscle function</json:string><json:string>lung biopsy</json:string><json:string>respiratory muscle tests</json:string><json:string>expiratory volume</json:string><json:string>respiratory muscle weakness</json:string><json:string>pneumonia</json:string><json:string>defect</json:string><json:string>aspiration</json:string><json:string>high blood pressure</json:string><json:string>respiratory muscle dysfunction</json:string><json:string>childhood myositis assessment scale</json:string><json:string>nasal inspiratory pressure</json:string><json:string>maximal inspiratory pressure</json:string><json:string>maximal expiratory pressure</json:string><json:string>clinical manifestations</json:string><json:string>respiratory status</json:string><json:string>bronchial walls</json:string><json:string>respiratory manifestations</json:string><json:string>chronic aspiration pneumonia</json:string><json:string>treatment regimens</json:string><json:string>respiratory muscle impairment</json:string><json:string>cmas score</json:string><json:string>ventilatory defect</json:string><json:string>autoimmune origin</json:string><json:string>maladies respiratoires rares</json:string><json:string>chronic idiopathic myopathy</json:string><json:string>assistance publique</json:string><json:string>universite paris descartes</json:string><json:string>standard form</json:string><json:string>femoral head</json:string><json:string>respiratory condition</json:string><json:string>gluteal region</json:string><json:string>foot osteomyelitis</json:string><json:string>main cause</json:string><json:string>months igiv</json:string><json:string>antoine deschildre</json:string><json:string>chest hrct</json:string><json:string>months hydroxychloroquine</json:string><json:string>muscle strength</json:string><json:string>total lung capacity</json:string><json:string>hrct results</json:string><json:string>systemic corticosteroids</json:string><json:string>intensive care unit</json:string><json:string>early stage</json:string><json:string>oral corticosteroids</json:string><json:string>lower lobe</json:string><json:string>asymptomatic patients</json:string><json:string>chest hrct scans</json:string><json:string>adult patients</json:string><json:string>interstitial pneumonia</json:string><json:string>inclusion duration</json:string><json:string>wiley periodicals</json:string><json:string>muscle involvement</json:string><json:string>obstructive defect</json:string><json:string>respiratory muscle function</json:string><json:string>therapeutic approaches</json:string><json:string>disease activity</json:string><json:string>national institute</json:string><json:string>functional abnormalities</json:string><json:string>maximal expiratory</json:string><json:string>radiological abnormalities</json:string></teeft></keywords><author><json:item><name>Guillaume Pouessel MD</name><affiliations><json:string>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</json:string><json:string>Service de Pédiatrie, Pavillon Médico‐Chirurgical de Pédiatrie, Hôpital Victor Provo, 59056 Roubaix Cedex 1, France</json:string></affiliations></json:item><json:item><name>Antoine Deschildre MD</name><affiliations><json:string>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</json:string><json:string>E-mail: antoine.deschildre@chru-lille.fr</json:string></affiliations></json:item><json:item><name>Muriel Le Bourgeois MD</name><affiliations><json:string>Service de Pneumologie et Allergologie Pédiatriques, Centre de Référence Des Maladies Respiratoires Rares de L'enfant, Hôpital Necker‐Enfants Malades, Université Paris Descartes, Assistance Publique des Hôpitaux de Paris, Paris, France</json:string></affiliations></json:item><json:item><name>Jean‐Marie Cuisset MD</name><affiliations><json:string>Service de Neurologie Pédiatrique, Hôpital Salengro, CHRU de Lille, Lille, France</json:string></affiliations></json:item><json:item><name>Benoit Catteau MD</name><affiliations><json:string>Service de Dermatologie, Hôpital Huriez, CHRU de Lille, Lille, France</json:string></affiliations></json:item><json:item><name>Chantal Karila MD</name><affiliations><json:string>Service de Pneumologie et Allergologie Pédiatriques, Centre de Référence Des Maladies Respiratoires Rares de L'enfant, Hôpital Necker‐Enfants Malades, Université Paris Descartes, Assistance Publique des Hôpitaux de Paris, Paris, France</json:string></affiliations></json:item><json:item><name>Véronique Nève MD</name><affiliations><json:string>Unité D'explorations Fonctionnelles Respiratoires Pédiatriques, Hôpital Jeanne de Flandre, CHRU de Lille, Lille, France</json:string></affiliations></json:item><json:item><name>Caroline Thumerelle MD</name><affiliations><json:string>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</json:string></affiliations></json:item><json:item><name>Pierre Quartier MD</name><affiliations><json:string>Service d'Immunologie‐Hématologie et Rhumatologie Pédiatriques, Hôpital Necker‐Enfants Malades, Université Paris Descartes, Assistance Publique des Hôpitaux de Paris, Paris, France</json:string></affiliations></json:item><json:item><name>Isabelle Tillie‐Leblond MD, PhD</name><affiliations><json:string>Service de Pneumologie et d'Immunologie‐Allergologie, Centre de Compétence des Maladies Pulmonaires Rares, Hôpital Calmette, CHRU de Lille, and INSERM U 1019, Institut Pasteur, Lille, France</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>juvenile dermatomyositis</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>lung</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>diaphragm</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>interstitial lung disease</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>child</value></json:item></subject><articleId><json:string>PPUL22742</json:string></articleId><arkIstex>ark:/67375/WNG-98DBKSM5-1</arkIstex><language><json:string>eng</json:string></language><originalGenre><json:string>article</json:string></originalGenre><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 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. Pediatr Pulmonol. 2013; 48:1016–1025. © 2012 Wiley Periodicals, Inc.</abstract><qualityIndicators><score>10</score><pdfWordCount>5230</pdfWordCount><pdfCharCount>35244</pdfCharCount><pdfVersion>1.3</pdfVersion><pdfPageCount>10</pdfPageCount><pdfPageSize>593.972 x 792 pts</pdfPageSize><pdfWordsPerPage>523</pdfWordsPerPage><pdfText>true</pdfText><refBibsNative>true</refBibsNative><abstractWordCount>309</abstractWordCount><abstractCharCount>2235</abstractCharCount><keywordCount>5</keywordCount></qualityIndicators><title>The lung is involved in juvenile dermatomyositis</title><pmid><json:string>23281200</json:string></pmid><genre><json:string>article</json:string></genre><host><title>Pediatric Pulmonology</title><language><json:string>unknown</json:string></language><doi><json:string>10.1002/(ISSN)1099-0496</json:string></doi><issn><json:string>8755-6863</json:string></issn><eissn><json:string>1099-0496</json:string></eissn><publisherId><json:string>PPUL</json:string></publisherId><volume>48</volume><issue>10</issue><pages><first>1016</first><last>1025</last><total>10</total></pages><genre><json:string>journal</json:string></genre><subject><json:item><value>Original Article: Other</value></json:item><json:item><value>Original Articles: Others</value></json:item></subject></host><namedEntities><unitex><date><json:string>2000</json:string><json:string>1975</json:string><json:string>from March 2008 to May 2009</json:string><json:string>1017</json:string><json:string>2013-09-08</json:string></date><geogName></geogName><orgName><json:string>UK and Ireland</json:string><json:string>Pediatric Rheumatology International Trials Organisation</json:string><json:string>National Registry 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uri="https://api.istex.fr/ark:/67375/WNG-98DBKSM5-1/fulltext.tei"><teiHeader><fileDesc><titleStmt><title level="a" type="main">The lung is involved in juvenile dermatomyositis</title><title level="a" type="short">Lung Involvement in Juvenile Dermatomyositis</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher ref="https://scientific-publisher.data.istex.fr/ark:/67375/H02-QW5Q88H5-V">Wiley Publishing Ltd</publisher><availability><licence>© 2012 Wiley Periodicals, Inc.</licence></availability><date type="published" when="2013-10"></date></publicationStmt><notesStmt><note type="content-type" subtype="article" source="article" scheme="https://content-type.data.istex.fr/ark:/67375/XTP-6N5SZHKN-D">article</note><note type="publication-type" subtype="journal" scheme="https://publication-type.data.istex.fr/ark:/67375/JMC-0GLKJH51-B">journal</note></notesStmt><sourceDesc><biblStruct type="article"><analytic><title level="a" type="main">The lung is involved in juvenile dermatomyositis</title><title level="a" type="short">Lung Involvement in Juvenile Dermatomyositis</title><author xml:id="author-0000"><persName><forename type="first">Guillaume</forename><surname>Pouessel</surname><roleName type="degree">MD</roleName></persName><affiliation><orgName type="division">Unité de Pneumologie et Allergologie</orgName><orgName type="institution">Pôle de Pédiatrie, Centre de Compétence des Maladies Respiratoires Rares de L'enfant, Hôpital Jeanne de Flandre et Université Lille 2</orgName><address><street>CHRU de Lille</street><settlement>Lille</settlement><country key="FR" xml:lang="en">FRANCE</country></address></affiliation><affiliation><orgName type="division">Service de Pédiatrie</orgName><orgName type="institution">Pavillon Médico‐Chirurgical de Pédiatrie, Hôpital Victor Provo</orgName><address><street>59056 Roubaix Cedex 1</street><country key="FR" xml:lang="en">FRANCE</country></address></affiliation></author><author xml:id="author-0001" role="corresp"><persName><forename type="first">Antoine</forename><surname>Deschildre</surname><roleName type="degree">MD</roleName></persName><affiliation><orgName type="division">Unité de Pneumologie et Allergologie</orgName><orgName type="institution">Pôle de Pédiatrie, Centre de Compétence des Maladies Respiratoires Rares de L'enfant, Hôpital Jeanne de Flandre et Université Lille 2</orgName><address><street>CHRU de Lille</street><settlement>Lille</settlement><country key="FR" xml:lang="en">FRANCE</country></address></affiliation></author><author xml:id="author-0002"><persName><forename type="first">Muriel</forename><surname>Le Bourgeois</surname><roleName type="degree">MD</roleName></persName><affiliation><orgName type="division">Service de Pneumologie et Allergologie Pédiatriques</orgName><orgName type="institution">Centre de Référence Des Maladies Respiratoires Rares de L'enfant, Hôpital Necker‐Enfants Malades, Université Paris Descartes, Assistance Publique des Hôpitaux de Paris</orgName><address><settlement>Paris</settlement><country key="FR" xml:lang="en">FRANCE</country></address></affiliation></author><author xml:id="author-0003"><persName><forename type="first">Jean‐Marie</forename><surname>Cuisset</surname><roleName type="degree">MD</roleName></persName><affiliation><orgName type="division">Service de Neurologie Pédiatrique</orgName><orgName type="institution">Hôpital Salengro, CHRU de Lille</orgName><address><settlement>Lille</settlement><country key="FR" xml:lang="en">FRANCE</country></address></affiliation></author><author xml:id="author-0004"><persName><forename type="first">Benoit</forename><surname>Catteau</surname><roleName type="degree">MD</roleName></persName><affiliation><orgName type="division">Service de Dermatologie</orgName><orgName type="institution">Hôpital Huriez, CHRU de Lille</orgName><address><settlement>Lille</settlement><country key="FR" xml:lang="en">FRANCE</country></address></affiliation></author><author xml:id="author-0005"><persName><forename type="first">Chantal</forename><surname>Karila</surname><roleName type="degree">MD</roleName></persName><affiliation><orgName type="division">Service de Pneumologie et Allergologie Pédiatriques</orgName><orgName type="institution">Centre de Référence Des Maladies Respiratoires Rares de L'enfant, Hôpital Necker‐Enfants Malades, Université Paris Descartes, Assistance Publique des Hôpitaux de Paris</orgName><address><settlement>Paris</settlement><country key="FR" xml:lang="en">FRANCE</country></address></affiliation></author><author xml:id="author-0006"><persName><forename type="first">Véronique</forename><surname>Nève</surname><roleName type="degree">MD</roleName></persName><affiliation><orgName type="division">Unité D'explorations Fonctionnelles Respiratoires Pédiatriques</orgName><orgName type="institution">Hôpital Jeanne de Flandre, CHRU de Lille</orgName><address><settlement>Lille</settlement><country key="FR" xml:lang="en">FRANCE</country></address></affiliation></author><author xml:id="author-0007"><persName><forename type="first">Caroline</forename><surname>Thumerelle</surname><roleName type="degree">MD</roleName></persName><affiliation><orgName type="division">Unité de Pneumologie et Allergologie</orgName><orgName type="institution">Pôle de Pédiatrie, Centre de Compétence des Maladies Respiratoires Rares de L'enfant, Hôpital Jeanne de Flandre et Université Lille 2</orgName><address><street>CHRU de Lille</street><settlement>Lille</settlement><country key="FR" xml:lang="en">FRANCE</country></address></affiliation></author><author xml:id="author-0008"><persName><forename type="first">Pierre</forename><surname>Quartier</surname><roleName type="degree">MD</roleName></persName><affiliation><orgName type="division">Service d'Immunologie‐Hématologie et Rhumatologie Pédiatriques</orgName><orgName type="division">Hôpital Necker‐Enfants Malades</orgName><orgName type="institution">Université Paris Descartes, Assistance Publique des Hôpitaux de Paris</orgName><address><settlement>Paris</settlement><country key="FR" xml:lang="en">FRANCE</country></address></affiliation></author><author xml:id="author-0009"><persName><forename type="first">Isabelle</forename><surname>Tillie‐Leblond</surname><roleName type="degree">MD, PhD</roleName></persName><affiliation><orgName type="division">Service de Pneumologie et d'Immunologie‐Allergologie</orgName><orgName type="institution">Centre de Compétence des Maladies Pulmonaires Rares, Hôpital Calmette, CHRU de Lille, and INSERM U 1019, Institut Pasteur</orgName><address><settlement>Lille</settlement><country key="FR" xml:lang="en">FRANCE</country></address></affiliation></author><idno type="istex">31A1C79BF240BB3958F08406044B25EC1F096D5A</idno><idno type="ark">ark:/67375/WNG-98DBKSM5-1</idno><idno type="DOI">10.1002/ppul.22742</idno><idno type="unit">PPUL22742</idno><idno 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<ref target="http://www.tei-c.org/">We use TEI</ref></desc></application></appInfo></encodingDesc><profileDesc><abstract xml:lang="en" style="main"><head>Abstract</head><head>Background</head><p>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.</p><head>Methods and patients</head><p>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.</p><head>Results</head><p>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.</p><head>Conclusion</head><p>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. <hi rend="bold">Pediatr Pulmonol. 2013; 48:1016–1025.</hi> © 2012 Wiley Periodicals, Inc.</p></abstract><textClass><keywords xml:lang="en"><term xml:id="ppul22742-kwd-0001">juvenile dermatomyositis</term><term xml:id="ppul22742-kwd-0002">lung</term><term xml:id="ppul22742-kwd-0003">diaphragm</term><term xml:id="ppul22742-kwd-0004">interstitial lung disease</term><term xml:id="ppul22742-kwd-0005">child</term></keywords><keywords rend="articleCategory"><term>Original Article: Other</term></keywords><keywords rend="tocHeading1"><term>Original Articles: Others</term></keywords></textClass><langUsage><language ident="en"></language></langUsage></profileDesc><revisionDesc><change when="2019-12-20" who="#istex" xml:id="pub2tei">formatting</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><extension>txt</extension><original>false</original><mimetype>text/plain</mimetype><uri>https://api.istex.fr/ark:/67375/WNG-98DBKSM5-1/fulltext.txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Wiley, elements deleted: body"><istex:xmlDeclaration>version="1.0" encoding="UTF-8" standalone="yes"</istex:xmlDeclaration><istex:document><component version="2.0" xml:lang="en" type="serialArticle" xml:id="ppul22742"><header><publicationMeta level="product"><doi origin="wiley" registered="yes">10.1002/(ISSN)1099-0496</doi><issn type="print">8755-6863</issn><issn type="electronic">1099-0496</issn><idGroup><id type="product" value="PPUL"></id></idGroup><titleGroup><title type="main" sort="PEDIATRIC PULMONOLOGY">Pediatric Pulmonology</title><title type="short">Pediatr Pulmonol.</title></titleGroup></publicationMeta><publicationMeta level="part" position="100"><doi registered="yes">10.1002/ppul.v48.10</doi><copyright ownership="publisher">© 2012 Wiley Periodicals, Inc.</copyright><numberingGroup><numbering type="journalVolume" number="48">48</numbering><numbering type="journalIssue">10</numbering></numberingGroup><coverDate startDate="2013-10">October 2013</coverDate></publicationMeta><publicationMeta level="unit" position="100" type="article" status="forIssue"><doi>10.1002/ppul.22742</doi><idGroup><id type="unit" value="PPUL22742"></id></idGroup><countGroup><count type="pageTotal" number="10"></count></countGroup><titleGroup><title type="articleCategory">Original Article: Other</title><title type="tocHeading1">Original Articles: Others</title></titleGroup><copyright ownership="publisher">© 2012 Wiley Periodicals, Inc.</copyright><eventGroup><event type="manuscriptReceived" date="2012-03-27"></event><event type="manuscriptRevised" date="2012-07-18"></event><event type="manuscriptAccepted" date="2012-09-15"></event><event type="xmlCreated" agent="Thomson Digital" date="2013-09-08"></event><event type="publishedOnlineEarlyUnpaginated" date="2012-12-31"></event><event type="firstOnline" date="2012-12-31"></event><event type="publishedOnlineFinalForm" date="2013-09-09"></event><event type="xmlConverted" agent="Converter:WILEY_ML3G_TO_WILEY_ML3GV2 version:3.8.8" date="2014-02-07"></event><event type="xmlConverted" agent="Converter:WML3G_To_WML3G version:4.6.4 mode:FullText" date="2015-10-01"></event></eventGroup><numberingGroup><numbering type="pageFirst">1016</numbering><numbering type="pageLast">1025</numbering></numberingGroup><correspondenceTo>Correspondence to: Antoine Deschildre, MD, Unité de Pneumologie et Allergologie, Pôle de Pédiatrie, Hôpital Jeanne de Flandre, CHRU de Lille, Avenue Eugène Avinée, 59037 Lille Cedex, France. E‐mail: <email>antoine.deschildre@chru-lille.fr</email></correspondenceTo><linkGroup><link type="toTypesetVersion" href="file:PPUL.PPUL22742.pdf"></link></linkGroup></publicationMeta><contentMeta><titleGroup><title type="main">The lung is involved in juvenile dermatomyositis</title><title type="short">Lung Involvement in Juvenile Dermatomyositis</title><title type="shortAuthors">Pouessel et al.</title></titleGroup><creators><creator xml:id="ppul22742-cr-0001" creatorRole="author" affiliationRef="#ppul22742-aff-0001 #ppul22742-aff-0002"><personName><givenNames>Guillaume</givenNames> <familyName>Pouessel</familyName><degrees>MD</degrees></personName></creator><creator xml:id="ppul22742-cr-0002" creatorRole="author" affiliationRef="#ppul22742-aff-0001" corresponding="yes"><personName><givenNames>Antoine</givenNames> <familyName>Deschildre</familyName><degrees>MD</degrees></personName></creator><creator xml:id="ppul22742-cr-0003" creatorRole="author" affiliationRef="#ppul22742-aff-0003"><personName><givenNames>Muriel</givenNames> <familyName>Le Bourgeois</familyName><degrees>MD</degrees></personName></creator><creator xml:id="ppul22742-cr-0004" creatorRole="author" affiliationRef="#ppul22742-aff-0004"><personName><givenNames>Jean‐Marie</givenNames> <familyName>Cuisset</familyName><degrees>MD</degrees></personName></creator><creator xml:id="ppul22742-cr-0005" creatorRole="author" affiliationRef="#ppul22742-aff-0005"><personName><givenNames>Benoit</givenNames> <familyName>Catteau</familyName><degrees>MD</degrees></personName></creator><creator xml:id="ppul22742-cr-0006" creatorRole="author" affiliationRef="#ppul22742-aff-0003"><personName><givenNames>Chantal</givenNames> <familyName>Karila</familyName><degrees>MD</degrees></personName></creator><creator xml:id="ppul22742-cr-0007" creatorRole="author" affiliationRef="#ppul22742-aff-0006"><personName><givenNames>Véronique</givenNames> <familyName>Nève</familyName><degrees>MD</degrees></personName></creator><creator xml:id="ppul22742-cr-0008" creatorRole="author" affiliationRef="#ppul22742-aff-0001"><personName><givenNames>Caroline</givenNames> <familyName>Thumerelle</familyName><degrees>MD</degrees></personName></creator><creator xml:id="ppul22742-cr-0009" creatorRole="author" affiliationRef="#ppul22742-aff-0007"><personName><givenNames>Pierre</givenNames> <familyName>Quartier</familyName><degrees>MD</degrees></personName></creator><creator xml:id="ppul22742-cr-0010" creatorRole="author" affiliationRef="#ppul22742-aff-0008"><personName><givenNames>Isabelle</givenNames> <familyName>Tillie‐Leblond</familyName><degrees>MD, PhD</degrees></personName></creator></creators><affiliationGroup><affiliation xml:id="ppul22742-aff-0001" countryCode="FR"><orgDiv>Unité de Pneumologie et Allergologie</orgDiv><orgName>Pôle de Pédiatrie, Centre de Compétence des Maladies Respiratoires Rares de L'enfant, Hôpital Jeanne de Flandre et Université Lille 2</orgName><address><street>CHRU de Lille</street><city>Lille</city><country>France</country></address></affiliation><affiliation xml:id="ppul22742-aff-0002" countryCode="FR"><orgDiv>Service de Pédiatrie</orgDiv><orgName>Pavillon Médico‐Chirurgical de Pédiatrie, Hôpital Victor Provo</orgName><address><street>59056 Roubaix Cedex 1</street><country>France</country></address></affiliation><affiliation xml:id="ppul22742-aff-0003" countryCode="FR"><orgDiv>Service de Pneumologie et Allergologie Pédiatriques</orgDiv><orgName>Centre de Référence Des Maladies Respiratoires Rares de L'enfant, Hôpital Necker‐Enfants Malades, Université Paris Descartes, Assistance Publique des Hôpitaux de Paris</orgName><address><city>Paris</city><country>France</country></address></affiliation><affiliation xml:id="ppul22742-aff-0004" countryCode="FR"><orgDiv>Service de Neurologie Pédiatrique</orgDiv><orgName>Hôpital Salengro, CHRU de Lille</orgName><address><city>Lille</city><country>France</country></address></affiliation><affiliation xml:id="ppul22742-aff-0005" countryCode="FR"><orgDiv>Service de Dermatologie</orgDiv><orgName>Hôpital Huriez, CHRU de Lille</orgName><address><city>Lille</city><country>France</country></address></affiliation><affiliation xml:id="ppul22742-aff-0006" countryCode="FR"><orgDiv>Unité D'explorations Fonctionnelles Respiratoires Pédiatriques</orgDiv><orgName>Hôpital Jeanne de Flandre, CHRU de Lille</orgName><address><city>Lille</city><country>France</country></address></affiliation><affiliation xml:id="ppul22742-aff-0007" countryCode="FR"><orgDiv>Service d'Immunologie‐Hématologie et Rhumatologie Pédiatriques</orgDiv><orgDiv>Hôpital Necker‐Enfants Malades</orgDiv><orgName>Université Paris Descartes, Assistance Publique des Hôpitaux de Paris</orgName><address><city>Paris</city><country>France</country></address></affiliation><affiliation xml:id="ppul22742-aff-0008" countryCode="FR"><orgDiv>Service de Pneumologie et d'Immunologie‐Allergologie</orgDiv><orgName>Centre de Compétence des Maladies Pulmonaires Rares, Hôpital Calmette, CHRU de Lille, and INSERM U 1019, Institut Pasteur</orgName><address><city>Lille</city><country>France</country></address></affiliation></affiliationGroup><keywordGroup type="author" xml:lang="en"><keyword xml:id="ppul22742-kwd-0001">juvenile dermatomyositis</keyword><keyword xml:id="ppul22742-kwd-0002">lung</keyword><keyword xml:id="ppul22742-kwd-0003">diaphragm</keyword><keyword xml:id="ppul22742-kwd-0004">interstitial lung disease</keyword><keyword xml:id="ppul22742-kwd-0005">child</keyword></keywordGroup><fundingInfo><fundingAgency>none reported</fundingAgency></fundingInfo><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><section xml:id="ppul22742-sec-0001"><title type="main">Background</title><p>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.</p></section><section xml:id="ppul22742-sec-0002"><title type="main">Methods and patients</title><p>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.</p></section><section xml:id="ppul22742-sec-0003"><title type="main">Results</title><p>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.</p></section><section xml:id="ppul22742-sec-0004"><title type="main">Conclusion</title><p>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. <b>Pediatr Pulmonol. 2013; 48:1016–1025.</b> © 2012 Wiley Periodicals, Inc.</p></section></abstract></abstractGroup></contentMeta><noteGroup><note xml:id="ppul22742-note-0010" numbered="no">Conflicts of interest: The authors declare that they have no competing interests.</note></noteGroup></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>The lung is involved in juvenile dermatomyositis</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Lung Involvement in Juvenile Dermatomyositis</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>The lung is involved in juvenile dermatomyositis</title></titleInfo><name type="personal"><namePart type="given">Guillaume</namePart><namePart type="family">Pouessel</namePart><namePart type="termsOfAddress">MD</namePart><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</affiliation><affiliation>Service de Pédiatrie, Pavillon Médico‐Chirurgical de Pédiatrie, Hôpital Victor Provo, 59056 Roubaix Cedex 1, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Antoine</namePart><namePart type="family">Deschildre</namePart><namePart type="termsOfAddress">MD</namePart><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</affiliation><affiliation>E-mail: antoine.deschildre@chru-lille.fr</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Muriel</namePart><namePart type="family">Le Bourgeois</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service de Pneumologie et Allergologie Pédiatriques, Centre de Référence Des Maladies Respiratoires Rares de L'enfant, Hôpital Necker‐Enfants Malades, Université Paris Descartes, Assistance Publique des Hôpitaux de Paris, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Jean‐Marie</namePart><namePart type="family">Cuisset</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service de Neurologie Pédiatrique, Hôpital Salengro, CHRU de Lille, Lille, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Benoit</namePart><namePart type="family">Catteau</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service de Dermatologie, Hôpital Huriez, CHRU de Lille, Lille, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Chantal</namePart><namePart type="family">Karila</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service de Pneumologie et Allergologie Pédiatriques, Centre de Référence Des Maladies Respiratoires Rares de L'enfant, Hôpital Necker‐Enfants Malades, Université Paris Descartes, Assistance Publique des Hôpitaux de Paris, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Véronique</namePart><namePart type="family">Nève</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Unité D'explorations Fonctionnelles Respiratoires Pédiatriques, Hôpital Jeanne de Flandre, CHRU de Lille, Lille, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Caroline</namePart><namePart type="family">Thumerelle</namePart><namePart type="termsOfAddress">MD</namePart><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</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Pierre</namePart><namePart type="family">Quartier</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service d'Immunologie‐Hématologie et Rhumatologie Pédiatriques, Hôpital Necker‐Enfants Malades, Université Paris Descartes, Assistance Publique des Hôpitaux de Paris, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Isabelle</namePart><namePart type="family">Tillie‐Leblond</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>Service de Pneumologie et d'Immunologie‐Allergologie, Centre de Compétence des Maladies Pulmonaires Rares, Hôpital Calmette, CHRU de Lille, and INSERM U 1019, Institut Pasteur, Lille, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="article" displayLabel="article" authority="ISTEX" authorityURI="https://content-type.data.istex.fr" valueURI="https://content-type.data.istex.fr/ark:/67375/XTP-6N5SZHKN-D">article</genre><originInfo><publisher>Blackwell Publishing Ltd</publisher><dateIssued encoding="w3cdtf">2013-10</dateIssued><dateCreated encoding="w3cdtf">2013-09-08</dateCreated><dateCaptured encoding="w3cdtf">2012-03-27</dateCaptured><dateValid encoding="w3cdtf">2012-09-15</dateValid><copyrightDate encoding="w3cdtf">2013</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><abstract lang="en">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. Pediatr Pulmonol. 2013; 48:1016–1025. © 2012 Wiley Periodicals, Inc.</abstract><note type="funding">none reported</note><subject lang="en"><genre>keywords</genre><topic>juvenile dermatomyositis</topic><topic>lung</topic><topic>diaphragm</topic><topic>interstitial lung disease</topic><topic>child</topic></subject><relatedItem type="host"><titleInfo><title>Pediatric Pulmonology</title></titleInfo><titleInfo type="abbreviated"><title>Pediatr Pulmonol.</title></titleInfo><genre type="journal" authority="ISTEX" authorityURI="https://publication-type.data.istex.fr" valueURI="https://publication-type.data.istex.fr/ark:/67375/JMC-0GLKJH51-B">journal</genre><subject><genre>article-category</genre><topic>Original Article: Other</topic><topic>Original Articles: Others</topic></subject><identifier type="ISSN">8755-6863</identifier><identifier type="eISSN">1099-0496</identifier><identifier type="DOI">10.1002/(ISSN)1099-0496</identifier><identifier type="PublisherID">PPUL</identifier><part><date>2013</date><detail type="volume"><caption>vol.</caption><number>48</number></detail><detail type="issue"><caption>no.</caption><number>10</number></detail><extent unit="pages"><start>1016</start><end>1025</end><total>10</total></extent></part></relatedItem><relatedItem type="references" displayLabel="ppul22742-cit-0001"><titleInfo><title>Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood</title></titleInfo><name type="personal"><namePart type="given">BM</namePart><namePart type="family">Feldman</namePart><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">LG</namePart><namePart type="family">Rider</namePart><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">AM</namePart><namePart type="family">Reed</namePart><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">LM</namePart><namePart type="family">Pachman</namePart><role><roleTerm type="text">author</roleTerm></role></name><genre>journal-article</genre><note type="citation/reference">Feldman BM, Rider LG, Reed AM, Pachman LM. 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