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Polymyositis with Lung Involvement Presenting as Bronchiolitis Obliterans Organizing Pneumonia-A Case Report

多發性肌炎導致阻塞性細支氣管炎合併器質化肺炎-病例報告

摘要


多發性肌炎的病患中產生間質性肺炎的比例並不高,而其中組織型態以阻塞性細支氣管炎合併器質化肺炎表現者則更為稀少。患者若產生間質性肺炎通常代表較差的預後,但其中以阻塞性細支氣管炎合併器質化肺炎表現者除外。 本病例為一位六十一歲健康男性,無特殊病史。入院主訴為四天前開始發燒、咳嗽及呼吸困難。胸部X光浸潤、白血球及C反應蛋白升高符合肺炎診斷。但抗生素治療無效且病情進展至呼吸衰竭需使用呼吸器。幾天後,病患產生肌肉疼痛及無力的症狀且血清肌酐酸上升。經過一系列的追蹤檢查確定患者為多發性肌炎導致阻塞性細支氣管炎合併器質化肺炎。經施予類固醇治療後,病患病情迅速改善並成功脫離呼吸器,治癒出院。

並列摘要


The incidence of interstitial lung disease (ILD) in polymyositis (PM) is low, and the histological pattern of ILD as bronchiolitis obliterans with organizing pneumonia (BOOP) is even less. ILD in PM usually indicates a poor prognosis, unless the histological presentation is BOOP. We report a 61-year-old male without a history of cigarette smoking or systemic disease, who presented with fever, cough and dyspnea for 4 days before admission. Leukocytosis and elevated C-reactive protein were observed. Chest X-ray (CXR) revealed left lower lobe infiltrates. He was initially treated for pneumonia, but with a clinically poor response to antibiotics. Respiratory failure occurred and the CXR showed disease progression. Muscle weakness, tenderness, and elevated creatinine kinase developed after a few days. Upon completion of a series of studies, the diagnosis of PM with lung involvement presenting as BOOP was confirmed. Antibiotic was discontinued and steroid prescribed. The disease had a dramatic response to steroid therapy. The patient was then successfully weaned from the ventilator and later discharged.

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