Edwards C, Cayton R, Bryan R
Department of Histopathology, East Birmingham Hospital.
J Clin Pathol. 1992 Nov;45(11):993-8. doi: 10.1136/jcp.45.11.993.
To investigate nine patients with histological changes at open lung biopsy compatible with so-called "small airways disease"; to define these changes and distinguish them from other types of bronchiolitis; and to correlate them with clinical features, respiratory physiology, and other laboratory investigations.
The open biopsy sections and the clinical records were reviewed.
There was chronic inflammation of the walls of centri-acinar respiratory bronchioles and peribronchiolar alveoli. Peripheral alveoli of affected acini were spared. Although lumens were narrowed and distorted, obstruction by granulation tissue plugs was not a feature. Clinical backgrounds included Hodgkin's disease, yellow nail syndrome, sicca syndrome, asthma and psoriasis. The main presenting symptoms were cough, dyspnoea and wheeze, often associated with basal crackles and basal shadowing. There was no consistent immunological or haematological abnormality. Sputum culture was negative in six patients, and a variety of organisms were cultured from three others. In most cases respiratory function tests revealed an irreversible obstructive defect (combined in some cases with a restrictive defect), gas trapping, and well preserved gas transfer. Lung volumes were normal. Bronchodilators, with steroids and azathioprine in more seriously affected patients, stabilised the disease process.
The histological changes may be distinguished from bronchiolitis obliterans and bronchiolitis obliterans organising pneumonia by the absence of intraluminal granulation tissue plugs, and from the obliterative bronchiolitis of rheumatoid disease and diffuse peribronchiolitis on clinical grounds. Although often striking, this lesion does not represent a clinico-pathological entity, and may occur in the distal lung in association with a number of different diseases. "Small airways disease" is often used by clinicians in a functional context, and may lead to confusion. It is suggested that "chronic transmural bronchiolitis" is a more appropriate term.
研究9例经开胸肺活检显示组织学改变符合所谓“小气道疾病”的患者；明确这些改变并将其与其他类型的细支气管炎相鉴别；并将这些改变与临床特征、呼吸生理学及其他实验室检查结果进行关联分析。
对开胸活检切片及临床记录进行回顾性分析。
终末细支气管中心周围呼吸性细支气管壁及细支气管周围肺泡存在慢性炎症。受累腺泡的外周肺泡未受累。尽管管腔变窄和变形，但未见肉芽组织堵塞导致的梗阻。临床背景包括霍奇金病、黄甲综合征、干燥综合征、哮喘和银屑病。主要表现症状为咳嗽、呼吸困难和喘息，常伴有肺底部湿啰音和肺底部阴影。未发现一致的免疫或血液学异常。6例患者痰培养阴性，另外3例培养出多种病原体。大多数情况下，呼吸功能测试显示为不可逆性阻塞性缺陷（部分病例合并限制性缺陷）、气体潴留，而气体交换功能保存良好。肺容积正常。支气管扩张剂，联合使用类固醇和硫唑嘌呤治疗病情较重的患者，可使病情稳定。
该组织学改变可通过无腔内肉芽组织堵塞与闭塞性细支气管炎及机化性肺炎相鉴别，并可基于临床理由与类风湿性疾病的闭塞性细支气管炎和弥漫性细支气管炎相鉴别。尽管该病变常很显著，但并不代表一种临床病理实体，可与多种不同疾病相关联发生于肺远端。临床医生常在功能背景下使用“小气道疾病”这一术语，可能会导致混淆。建议使用“慢性透壁性细支气管炎”这一术语更为合适。
