阻塞性細支氣管炎併器質性肺炎在自體免疫性疾病之臨床表現

Bronchiolitis Obliterans Organizing Pneumonia in Connective Tissue Disease

背景：阻塞性細支氣管炎併器質性肺炎在自體免疫性疾病的肺部表現中占有一定的比例，但是它的臨床症狀、治療及預後過去並沒有很詳細的描述。方法：我們收集高雄榮民總醫院過去兩年經由肺部切片證實為阻塞性細支氣管炎併器質性肺炎的病人，其中三人診斷為全身性紅斑狼瘡，一人診斷為皮肌炎，分析這些病人的臨床症狀、實驗室數據、放射線和病理檢查結果，以及藥物治療的反應。結果：全身性紅斑狼瘡的三個病人治療後臨床症狀和胸部X光片都有明顯的進步，但是另外一個皮肌炎的病人治療後病情仍逐漸惡化，後來產生了肺部纖維化的後遺症。結論：自體免疫性疾病的病人如果有肺部的病兆，經過一般的治療方式沒有明顯的進步時，最好能夠做肺部切片，如果診斷是阻塞性細支氣管炎併器質性肺炎，必須及早使用類固醇和免疫抑制劑治療，經過治療的病人大部份預後都很好，而且沒有復發的情形出現。

Background. To define the clinical course, radiology, histopathology and response to therapy of bronchiolitis obliterans organizing pneumonia (BOOP) in connective tissue disease. Methods. We describe four cases of BOOP in connective tissue disease-three cases of systemic lupus erythematosus and a case of dermatomyositis. Follow-up period was two years. All cases were diagnosed by an open lung biopsy, and all infectious studies were negative. All cases are treated with large dose corticosteroid and immunosuppressive agents. Results. Three cases show significant improvement following treatment, while the condition of one deteriorated resulting in pulmonary fibrosis and chronic pulmonary insufficiency. The response to treatment is good in the majority of patients. All patients are well tolerated to treatment. No relapses are noted during the follow-up period. Conclusion. BOOP is a manifestation of connective tissue disease in the lung. The prognosis is good in most patients. An open lung biopsy is necessary in order to make an accurate diagnosis. Early treatment with a combination of steroid and cytotoxic agents results in a low relapse rate.