Harvard Medical School, Pulmonary & Critical Care Medicine, Brigham & Women's Hospital, 75 Francis Street, Boston, Massachusetts 02115, USA.
Expert Rev Respir Med. 2007 Aug;1(1):139-47. doi: 10.1586/17476348.1.1.139.
Constrictive bronchiolitis obliterans is an important respiratory illness because of its underlying irreversible fibrotic process, and is defined as concentric fibrosis in the bronchiolar submucosal layer with continual external circular scarring. The fibrotic and destructive nature of this lesion is the defining characteristic. Although patchy, the airway may become slit-like or obliterated from this fibrotic process, resulting in bronchiolitis obliterans. Constrictive bronchiolitis is limited to the bronchioles and does not extend into the alveoli. The clinical features of constrictive bronchiolitis consist of a chest radiograph that is often normal, early inspiratory crackles and irreversible airflow obstruction by pulmonary function testing. Chest CT scans show a mosaic pattern, air trapping by the expiratory film, bronchiolectasis and thickened small airway walls. Although idiopathic constrictive bronchiolitis is rare, the lesion is common among lung-transplant recipients. The lesion also occurs from certain types of toxic fumes, some of the connective tissue diseases, specific types of medications and post respiratory infection. Unusual exposures have also been described as a cause of constrictive bronchiolitis, such as consumption of the leafy vegetable Sauropus androgynus in far eastern Asia and from inhaling diacetyl, the ketone butter flavoring used in microwave popcorn production. Empirical treatment consists of corticosteroid and immunosuppressive agents. Antifibrotic agents may be successful in the future. This is generally a nonsteroid-responsive lesion and for disabling disease, lung transplantation can be a successful option.
缩窄性细支气管炎是一种重要的呼吸系统疾病，因为它具有潜在的不可逆转的纤维化过程，定义为细支气管黏膜下层的同心性纤维化，伴有持续的环状瘢痕形成。这种病变的纤维化和破坏性是其特征。尽管病变呈斑片状，但气道可能由于纤维化过程而变得像缝隙一样狭窄或闭塞，导致细支气管炎闭塞。缩窄性细支气管炎仅限于细支气管，不会延伸到肺泡。缩窄性细支气管炎的临床特征包括胸片通常正常、早期吸气性爆裂音和肺功能测试显示不可逆转的气流阻塞。胸部 CT 扫描显示马赛克模式、呼气片空气潴留、细支气管扩张和小气道壁增厚。虽然特发性缩窄性细支气管炎很少见，但在肺移植受者中很常见。这种病变也发生在某些类型的有毒烟雾、某些结缔组织疾病、某些类型的药物和呼吸道感染后。一些不常见的暴露也被描述为缩窄性细支气管炎的病因，例如食用远东地区的绿叶蔬菜 Sauropus androgynus 和吸入二乙酰，这是微波爆米花生产中使用的酮黄油调味料。经验性治疗包括皮质类固醇和免疫抑制剂。抗纤维化药物在未来可能会成功。这通常是一种非甾体反应性病变，对于致残性疾病，肺移植是一种成功的选择。
