Literature DB >> 26231412

[Pulmonary manifestations of antisynthetase syndrome].

S Jouneau1, B Hervier2, E-M Jutant3, O Decaux4, M Kambouchner5, M Humbert3, P Delaval6, D Montani3.   

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.
Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Entities:  

Keywords:  Anti-Jo1; Hypertension pulmonaire; Infiltrative lung disease; Inflammatory myopathy; Myopathie inflammatoire; Myosite; Myositis; Pneumopathie interstitielle diffuse; Pulmonary hypertension

Mesh:

Year:  2014        PMID: 26231412     DOI: 10.1016/j.rmr.2014.07.013

Source DB:  PubMed          Journal:  Rev Mal Respir        ISSN: 0761-8425            Impact factor:   0.622


  2 in total

Review 1.  Secondary myopathy due to systemic diseases.

Authors:  J Finsterer; W N Löscher; J Wanschitz; S Quasthoff; W Grisold
Journal:  Acta Neurol Scand       Date:  2016-02-25       Impact factor: 3.209

2.  Investigating Idiopathic Inflammatory Myopathy; Initial Cross Speciality Experience with Use of the Extended Myositis Antibody Panel.

Authors:  Antoinette O'Connor; Jennifer Mulhall; Sinead M J Harney; John G Ryan; Grainne Murphy; Michael T Henry; Peter Annis; Vincent Tormey; Aisling M Ryan
Journal:  Clin Pract       Date:  2017-05-04
  2 in total

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