title: [Pulmonary manifestations of antisynthetase syndrome].
abstract: Antisynthetase syndrome is an inflammatory myopathy frequently associated with pulmonary manifestations, especially interstitial lung diseases, and uncommonly pulmonary hypertension. In the context of a suggestive clinical and radiological picture, positive anti-RNA synthetase antibodies confirm the diagnosis. Anti-Jo1, anti-PL7, and anti-PL12 antibodies are the more commonly encountered. The presence of a number of extra-thoracic manifestations in association with pulmonary disease may suggest the diagnosis. These include: myalgia or muscular deficit, Raynaud's phenomenon, polyarthritis, fever, mechanics hands. Serum creatine kinase levels are usually increased. Electromyogram, muscular magnetic resonance imaging or muscle pathology are not mandatory to make the diagnosis. There is a high variability in symptoms and severity, between patients but also during the course of the disease in the same patient. The presence of an interstitial lung disease is a major prognostic factor and an indication for more intensive treatment, principally with systemic corticosteroids with or without immunosuppressive drugs. Improving respiratory physicians' knowledge of this disease, which is often revealed by its pulmonary manifestations, should help diagnosis, therapeutic management, and possibly prognosis.
authors.affiliation: Service de pneumologie, centre de compétences des maladies pulmonaires rares de Bretagne, hôpital Pontchaillou, 2, rue Henri-Le-Guilloux, 35033 Rennes cedex, France; IRSET UMR 1085, université de Rennes 1, Rennes, France. Electronic address: stephane.jouneau@chu-rennes.fr.
authors.forename: S
authors.initials: S
authors.lastname: Jouneau
authors.affiliation: Service de médecine interne et immunologie clinique, groupe hospitalier Pitié-Salpêtrière, AP-HP-UPMC, Paris, France.
authors.forename: B
authors.initials: B
authors.lastname: Hervier
authors.affiliation: Service de pneumologie, centre de référence de l'hypertension pulmonaire sévère, faculté de médecine, université Paris-Sud, hôpital de Bicêtre, AP-HP, département hospitalo-universitaire (DHU) Thorax Innovation (TORINO), 94270 Le-Kremlin-Bicêtre, France; UMR_S 999, université Paris-Sud, Inserm, laboratoire d'excellence (LabEx) en recherche sur le médicament et l'innovation thérapeutique (LERMIT), centre chirurgical Marie-Lannelongue, 92350 Le Plessis Robinson, France.
authors.forename: E-M
authors.initials: EM
authors.lastname: Jutant
authors.affiliation: Service de médecine interne, hôpital sud, Rennes, France; UMR CNRS 6290 IGDR, université de Rennes 1, Rennes, France.
authors.forename: O
authors.initials: O
authors.lastname: Decaux
authors.affiliation: Service d'anatomie pathologique, hôpital Avicenne, AP-HP, Bobigny, France.
authors.forename: M
authors.initials: M
authors.lastname: Kambouchner
authors.affiliation: Service de pneumologie, centre de référence de l'hypertension pulmonaire sévère, faculté de médecine, université Paris-Sud, hôpital de Bicêtre, AP-HP, département hospitalo-universitaire (DHU) Thorax Innovation (TORINO), 94270 Le-Kremlin-Bicêtre, France; UMR_S 999, université Paris-Sud, Inserm, laboratoire d'excellence (LabEx) en recherche sur le médicament et l'innovation thérapeutique (LERMIT), centre chirurgical Marie-Lannelongue, 92350 Le Plessis Robinson, France.
authors.forename: M
authors.initials: M
authors.lastname: Humbert
authors.affiliation: Service de pneumologie, centre de compétences des maladies pulmonaires rares de Bretagne, hôpital Pontchaillou, 2, rue Henri-Le-Guilloux, 35033 Rennes cedex, France; IRSET UMR 1085, université de Rennes 1, Rennes, France.
authors.forename: P
authors.initials: P
authors.lastname: Delaval
authors.affiliation: Service de pneumologie, centre de référence de l'hypertension pulmonaire sévère, faculté de médecine, université Paris-Sud, hôpital de Bicêtre, AP-HP, département hospitalo-universitaire (DHU) Thorax Innovation (TORINO), 94270 Le-Kremlin-Bicêtre, France; UMR_S 999, université Paris-Sud, Inserm, laboratoire d'excellence (LabEx) en recherche sur le médicament et l'innovation thérapeutique (LERMIT), centre chirurgical Marie-Lannelongue, 92350 Le Plessis Robinson, France.
authors.forename: D
authors.initials: D
authors.lastname: Montani
mesh_terms: D018450:Disease Progression; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D009220:Myositis / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D011379:Prognosis; D013902:Radiography, Thoracic; D011928:Raynaud Disease / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology
keywords: Anti-Jo1; Hypertension pulmonaire; Infiltrative lung disease; Inflammatory myopathy; Myopathie inflammatoire; Myosite; Myositis; Pneumopathie interstitielle diffuse; Pulmonary hypertension