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严重呼吸衰竭作为与抗合成酶综合征(ASS)相关的间质性肺疾病的首发特征。

Severe respiratory failure as a presenting feature of an interstitial lung disease associated with anti-synthetase syndrome (ASS).

作者信息

Piroddi Ines Maria Grazia, Ferraioli Gianluca, Barlascini Cornelius, Castagneto Corrado, Nicolini Antonello

机构信息

Respiratory Diseases Unit ASL4 Chiavarese, Via Terzi 43, 16039 Sestri Levante, Italy.

Emergency and Internal Medicine Department ASL4 Chiavarese, Italy.

出版信息

Respir Investig. 2016 Jul;54(4):284-8. doi: 10.1016/j.resinv.2016.01.005. Epub 2016 Mar 8.

DOI:10.1016/j.resinv.2016.01.005
PMID:27424829
Abstract

Anti-synthetase syndrome (ASS) is defined as a heterogeneous connective tissue disorder characterized by the association of an interstitial lung disease (ILD) with or without inflammatory myositis with the presence of anti-aminoacyl-tRNA-synthetase antibodies. ILD is one of the major extra-muscular manifestations of polymyositis and dermatomyositis. We report a case of a patient with dyspnea, cough, and intermittent fever as well as ILD associated ASS in the absence of muscular involvement. This patient was admitted to the emergency department with severe respiratory failure requiring non-invasive ventilation. Our patient's case demonstrates that the diagnosis of ASS may not be obvious. However, its diagnosis leads to appropriate and potentially life-saving treatment.

摘要

抗合成酶综合征(ASS)被定义为一种异质性结缔组织疾病,其特征为间质性肺疾病(ILD)与炎性肌炎(有或无)并存,并伴有抗氨酰 - tRNA合成酶抗体。ILD是多发性肌炎和皮肌炎的主要肌肉外表现之一。我们报告一例患者,该患者有呼吸困难、咳嗽、间歇性发热,以及在无肌肉受累情况下与ILD相关的ASS。该患者因严重呼吸衰竭需无创通气而入住急诊科。我们患者的病例表明,ASS的诊断可能并不明显。然而,其诊断能带来恰当且可能挽救生命的治疗。

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