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[弥漫性间质性肺疾病揭示抗合成酶综合征:两例报告]

[Diffuse interstitial lung disease revealing anti-synthetase syndrome: about two cases].

作者信息

Bouardi Nizar El, Alaoui Amina, Haloua Meriem, Lamrani Youssef, Boubbou Meryem, Maaroufi Mustapha, Alami Baderdine

机构信息

Service de Radiologie, Centre Hospitalo-Universitaire Hassan II, Fès, Maroc.

Faculté de Médecine et de Pharmacie, Université Sidi Mohamed Ben Abdellah, Fès, Maroc.

出版信息

Pan Afr Med J. 2019 Jan 22;32:40. doi: 10.11604/pamj.2019.32.40.17903. eCollection 2019.

Abstract

Anti-synthetase syndrome (ASS) is an inflammatory myopathy commonly associated with pulmonary involvement, especially parenchymal (diffuse infiltrative pneumonitis). Extrathoracic manifestations associated with pulmonary involvement can give an indication to the diagnosis: myalgias, polyarthralgias, Raynaud's syndrome, erythematous palmar hyperkeratosis with fissures and fever. Given the suggestive clinical and radiological picture, the presence of aminoacyl-transfer RNA (tRNA) synthetase antibodies enables to confirm, in particular, Anti Jo-1 antibody activity. Pulmonary involvement is a major prognostic factor, hence the indication for intensive immunosuppressive therapy based on corticosteroids, immunosuppressive medications or the association among them. A better awareness about this disorder revealed by pulmonary manifestations could enable early and adequate management and to improve patient's prognosis.

摘要

抗合成酶综合征(ASS)是一种炎症性肌病,通常与肺部受累相关,尤其是实质性(弥漫性浸润性肺炎)。与肺部受累相关的胸外表现可为诊断提供线索:肌痛、多关节痛、雷诺综合征、伴有裂隙的手掌红斑性角化过度和发热。鉴于提示性的临床和影像学表现,氨酰基转移RNA(tRNA)合成酶抗体的存在尤其能够确认抗Jo - 1抗体活性。肺部受累是一个主要的预后因素,因此有基于皮质类固醇、免疫抑制药物或它们之间联合使用的强化免疫抑制治疗的指征。对这种由肺部表现揭示的疾病有更好的认识可以实现早期和充分的管理,并改善患者的预后。

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