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一例抗合成酶综合征伴意识丧失患者的报告。

Report of a Patient With Anti-Jo-1 Syndrome With Loss of Consciousness.

作者信息

Chen Chun, Su Yu-Jang, Wong Chiong-Hee

机构信息

Mackay Memorial Hospital Department of Emergency Medicine Taipei Taiwan.

MacKay Medical College Department of Medicine New Taipei City Taiwan.

出版信息

J Acute Med. 2022 Jun 1;12(2):71-74. doi: 10.6705/j.jacme.202206_12(2).0004.

Abstract

A 51-year-old febrile woman presented to the emergency department because of loss of consciousness while worshipping at a temple. She experienced muscle weakness and blurred vision before fainting. She also felt pain in her left shoulder and suffered from dry cough and shortness of breath during the previous week. The chest radiograph (CXR) showed bilateral reticular infiltration, indicating interstitial lung disease (ILD), and chest computed tomography revealed reticular, nodular, and reticulonodular patterns of infiltration that were compatible with ILD. Blood tests showed a creatinine kinase level of 3,307 IU/L and an Anti-Jo-1 autoantibody level of 586 AU/mL. It was found via right thigh biopsy results 8 days later that she had polymyositis with perivascular inflammation and degenerative muscle fiber change. Pulse therapy with methylprednisolone (1 gm/day for 3 days) was administered. After 4 days, she felt better and was discharged. Around 25.0%-34.1% of myositis patients have anti-Jo-1 autoantibodies, and ILD was noted in 65%-68% of anti-Jo-1-positive patients. A ground glass pattern may be observed in the CXRs of anti-Jo-1 ILD patients, and some articles described multiple pulmonary nodules mimicking lung metastasis or concomitant with pleural effusion. The conditions of approximately 42%-66% of ILD patients are controlled or are not worsened after treatment with corticosteroid and immunosuppressive drug treatment. However, some rapidly progressive cases are not sensitive to corticosteroid therapy alone.

摘要

一名51岁发热女性因在寺庙礼拜时失去意识被送至急诊科。她在昏厥前出现肌肉无力和视力模糊。前一周她还感到左肩疼痛,并伴有干咳和呼吸急促。胸部X线片(CXR)显示双侧网状浸润,提示间质性肺疾病(ILD),胸部计算机断层扫描显示与ILD相符的网状、结节状和网状结节状浸润模式。血液检查显示肌酸激酶水平为3307 IU/L,抗Jo-1自身抗体水平为586 AU/mL。8天后通过右大腿活检结果发现她患有伴血管周围炎症和肌纤维变性改变的多发性肌炎。给予甲泼尼龙冲击治疗(1克/天,共3天)。4天后,她感觉好转并出院。约25.0%-34.1%的肌炎患者有抗Jo-1自身抗体,65%-68%的抗Jo-1阳性患者有ILD。抗Jo-1 ILD患者的CXR中可能观察到磨玻璃样改变,一些文章描述了多个肺结节,类似肺转移或伴有胸腔积液。约42%-66%的ILD患者在接受皮质类固醇和免疫抑制药物治疗后病情得到控制或未恶化。然而,一些快速进展的病例对单独的皮质类固醇治疗不敏感。

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