Kraft M, Mortenson R L, Colby T V, Newman L, Waldron J A, King T E
Department of Medicine, National Jewish Center for Immunology and Respiratory Medicine, University of Colorado School of Medicine, Denver 80206.
Am Rev Respir Dis. 1993 Oct;148(4 Pt 1):1093-101. doi: 10.1164/ajrccm/148.4_Pt_1.1093.
Four women with a chronic respiratory illness characterized by chronic cough, dyspnea, mild to severe physiologic abnormalities, relatively normal chest radiographs, and lack of response to bronchodilators or prednisone were identified and prospectively evaluated. Constrictive bronchiolitis, defined as concentric narrowing of the bronchiolar lumen, mural scarring, smooth muscle hyperplasia, and mucus stasis, was the major histologic finding on open lung biopsy in all cases. Each presented with an illness clinically distinct from asthma, connective tissue disorders, occupational or environmental lung disease, bronchiectasis, diffuse panbronchiolitis, cystic fibrosis, and emphysema. None of the patients smoked cigarettes. None had clinical evidence of a recent viral lower respiratory tract infection. The physical examinations were normal except for rales heard on chest examination in two patients. Chest radiographs showed increased bronchovascular markings in three patients. Lung function was normal in one patient, two of the patients had a reduced diffusing capacity associated with moderate hypoxemia and an obstructive ventilatory defect, and one patient exhibited a mixture of restrictive and obstructive defects. None have experienced significant progression of their disease over 1 to 5 yr of follow-up. However, complete return to normal function did not occur. We hypothesize that patients with the constellation of findings described represent a distinct and definable clinicopathologic entity and further clarifies the spectrum of "small airways disease." Establishing the diagnosis appears important for prognostic and possibly therapeutic reasons.
我们确定了四名患有慢性呼吸系统疾病的女性，其特征为慢性咳嗽、呼吸困难、轻至重度生理异常、胸部X光片相对正常，且对支气管扩张剂或泼尼松无反应，并对她们进行了前瞻性评估。缩窄性细支气管炎的定义为细支气管腔同心性狭窄、壁瘢痕形成、平滑肌增生和黏液淤滞，在所有病例的开胸肺活检中均为主要组织学发现。每位患者的临床表现均与哮喘、结缔组织病、职业或环境性肺病、支气管扩张、弥漫性泛细支气管炎、囊性纤维化和肺气肿不同。所有患者均不吸烟。均无近期病毒性下呼吸道感染的临床证据。除两名患者胸部检查闻及湿啰音外，体格检查均正常。三名患者胸部X光片显示支气管血管纹理增多。一名患者肺功能正常，两名患者弥散功能降低，伴有中度低氧血症和阻塞性通气功能障碍，一名患者表现为限制性和阻塞性缺陷并存。在1至5年的随访中，所有患者病情均无显著进展。然而，肺功能并未完全恢复正常。我们推测，具有上述一系列表现的患者代表一种独特且可明确的临床病理实体，并进一步明确了“小气道疾病”的范围。基于预后及可能的治疗原因，确立诊断似乎很重要。
