Idiopathic Bronchiolitis Obliterans with Organizing Pneumonia Manifesting as Acute Respiratory Distress Syndrome - A Case Report

原發性阻塞性細小支氣管炎併組織化肺炎或原因不明的組織化肺炎為一少見特殊的臨床病理症候群。其病理組織的特徵為鬆散的結締組織積聚在細小支氣管及肺泡。類固醇對其療效很好。大部分的病人發病的原因不明，但需先排除感染、藥物、放射線、自體免疫疾病等病因。它的病程通常是緩慢的，一般以乾咳、運動時氣喘、發燒及一些非特異性的身體不適表現。此疾病以猛暴、急性發作是很少見的表現，我們報告一個原發性阻塞性細小支氣管炎併有機化肺炎，以急性呼吸窘迫症表現並使用類固醇脈衝療法成功治療的病例。原發性阻塞性細小支氣管炎併組織化肺炎若以急性方式表現，有可能誤判為非典型肺炎或是急性間質性肺炎，而給予不恰當的治療。故當急性呼吸窘迫症的導因不明或是對藥物反應不良，應早期切片診斷是否為此病，並及早給予類固醇治療，以免錯過此可至癒的疾病。

關鍵字

原發性阻塞性細小支氣管炎併組織化肺炎；急性呼吸窘迫症；開胸組織切片

並列摘要

Bronchiolitis obliterans with organizing pneumonia (BOOP) is a clinicopathologic syndrome that appears with characteristic loose connective tissue obliterating in the respiratory bronchioles, alveolar duct, and alveoli. It responds excellently to corticosteroids, and has a favorable prognosis. The typical symptoms of idiopathic BOOP include dry cough, exertional dyspnea, fever, or fatigue. They usually occur in a chronic or subacute course. An acute life threatening manifestation of BOOP resembling acute respiratory distress syndrome (ARDS) has rarely been reported. Herein, we report the case of a female adult who suffered from rapidly progressive dyspnea and hypoxemic respiratory failure, with a course mimicking bacterial pneumonia with ARDS, in need of mechanical ventilation and with a poor response to antibiotic treatment. Idiopathic BOOP was diagnosed after open lung biopsy, and successfully treated with pulse methylprednisolone therapy. We highlight the importance of early intervention by open lung biopsy in a patient with idiopathic ARDS. Steroid therapy is effective in patients with idiopathic rapidly progressive BOOP.

並列關鍵字

idiopathic bronchiolitis obliterans with organizing pneumonia ； BOOP ； acute respiratory distress syndrome ； ARDS ； open lung biopsy

國際替代計量

Idiopathic Bronchiolitis Obliterans with Organizing Pneumonia Manifesting as Acute Respiratory Distress Syndrome - A Case Report

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