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以咯血为表现的呼吸性细支气管炎伴间质性肺疾病(RB-ILD)。

Respiratory bronchiolitis associated interstitial lung disease (RB-ILD) presenting with haemoptysis.

作者信息

McWilliams A M, Lake F R

机构信息

Department of Respiratory Medicine, Royal Perth Hospital, Western Australia, Australia.

出版信息

Respirology. 2000 Dec;5(4):385-7.

Abstract

Respiratory bronchiolitis associated interstitial lung disease is an uncommon condition in current or ex-smokers. The presentation is non-specific, but haemoptysis is uncommonly reported in this condition. We report the case of a 25-year-old woman who presented with significant haemoptysis, dyspnoea, reduced transfer factor and normal clinical examination. In addition, a Medline literature search was performed to review the clinical features and prognosis of this disease. Other causes of haemoptysis were excluded with extensive investigation. The diagnosis was made on thoracoscopic lung biopsy. The patient had significant postoperative complications of prolonged air leak and hydropneumothorax requiring further surgery and prolonged hospital stay. Advice regarding smoking cessation was given. Her pulmonary physiology remains abnormal on follow up but symptoms have improved. Respiratory bronchiolitis-ILD may present with normal examination and radiology. Haemoptysis in this case may have been associated with the underlying disease but could have been incidental. Diagnosis, in general, requires lung biopsy. As in this patient, lung function does not appear to improve significantly on follow up.

摘要

呼吸性细支气管炎相关间质性肺疾病在现吸烟者或既往吸烟者中并不常见。其表现不具特异性,但咯血在该疾病中较少见。我们报告了一名25岁女性病例,该患者出现大量咯血、呼吸困难、肺弥散功能降低,而临床检查正常。此外,还进行了医学文献检索以回顾该疾病的临床特征和预后。通过广泛检查排除了其他咯血原因。诊断通过胸腔镜肺活检确定。该患者术后出现了严重的并发症,即持续漏气和血气胸,需要进一步手术并延长住院时间。已给予戒烟建议。随访时其肺功能仍异常,但症状有所改善。呼吸性细支气管炎相关间质性肺疾病可能临床检查和影像学表现正常。该病例中的咯血可能与基础疾病有关,但也可能是偶然发生。一般来说,诊断需要肺活检。正如该患者一样,随访时肺功能似乎并未显著改善。

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