瀰漫性泛細支氣管炎：病例報告及文獻回顧

Diffuse Panbronchiolitis: A Case Report and Review of the Literature

瀰漫性泛細支氣管炎(diffuse panbronchiolitis)是一種少見的特發性、漸進性、化膿性呼吸性支氣管和終末支氣管疾病，有獨特的放射診斷及臨床病理特徵，若不加以治療，經常會進展成支氣管擴張症、呼吸衰竭，甚至死亡。在非日本國家更是不多見的支氣管性疾病，經常被忽略診斷及治療。此疾病若長期施予低劑量的紅黴素治療，常可明顯改善症狀及存活率。本病例是一位70歲男性，有與先前文獻報告相同的臨床症狀及影像學診斷，且肺功能呈現限制型通氣障礙併低血氧，開始每日給予低劑量紅黴素500mg治療，目前已在門診追蹤3個月，臨床表徵及放射線診斷皆有明顯的改善。希望能藉此病例報告，提醒基層醫師，在面對一位長時間咳嗽有濃痰、走路會喘的老年病人，不要僅想到慢性阻塞性肺病或一般的肺炎，應該對此疾病有所警覺，否則將成為致命的疾病。如有懷疑，應轉診或會診相關專科醫師作進一步診斷及治療，以免延誤診斷與治療的黃金時間。

Diffuse panbronchiolitis is a rare respiratory bronchial and terminal bronchial disease which is idiopathic, progressive and suppurative. It has unique features of radiology and pathogenesis. If left untreated, it usually progresses to bronchiectasis, respiratory failure, and even death. This disease is not common in non-Japanese countries. The diagnosis and treatment are often ignored. Herein, we report a case of a 70-year-old man who had the same clinical symptoms and radiological features which were compatible with what in the literature and his pulmonary function test showed restrictive ventilatory impairment associated with hypoxemia. So we started to use low-dose erythromycin (500mg/day). The patient's physical condition and radiological features improved significantly after three months of erythromycin treatment. The purpose of this article is to remind those primary care physicians not to focus solely on chronic obstructive pulmonary disease or ordinary pneumonia when treat an old aged patient who has cough with purulent sputum and exertional dyspnea for a long time. It is suggest that the primary care physicians should be aware of the disease which maybe fatal, or it may be a deadly disease. If the physicians cannot confirm the disease, it is better to refer the patient to the more expert practitioner in order to have reasonable management without delay.