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Antisynthetase Syndrome (AS) in Patients with Diffuse Interstitial Lung Disease (ILD)

Ana Maria Lopez Vazquez, Beatriz Soria Navarro,Carles Fabregat-Franco,Sherlyne Jaimes Diaz,Cristina Sabater Abad,Mercedes Ramon Capilla,Gustavo Juan Samper,Estrella Fernandez-Fabrellas, Juana Rodriguez Delgado, Araceli Carbonell Moncho

EUROPEAN RESPIRATORY JOURNAL(2015)

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摘要
Objectives: To determine the prevalence of SAS in patients with ILD and its clinical characteristics, evolution and specific manifestations linked to each antisynthetase antibodies and highlight the need for its screening in ILD. Methods: Retrospective analysis of patients registered in the last two years in our ILD unit, determining antisynthetase antibodies and analyzing clinical, radiological, functional data and illness evolution. Results: 119 ILD patients were included, 10.9% had antisynthetase antibodies, the most prevalent were Jo-1 (46.1%) and PL-12 (38.5%). In PL-12 patients inicial and respiratory syntoms were predominant, whereas in the Jo-1 patients were more frequent extrapulmonary manifestations and acute presentation. Whatever type of AS, 84.6% showed interstitial disease in HRCT, indistinguishable from other ILD patterns. Response to treatment with corticosteroids and / or immunosuppressive was good (defined by a smaller drop of 10% in FVC in one year) at 84.6%. Conclusions: The AS is common in patients with ILD and may occur without extrapulmonary manifestations, indistinguishable from idiopathic forms of ILD. The antisynthetase antibodies usually present with pulmonary involvement. Therefore we suggest a screening of antisythetase antibodies should be done in patients with ILD.
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关键词
Interstitial lung disease (connective tissue disease),Immunology,Interstitial lung disease
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