Geddes D M, Corrin B, Brewerton D A, Davies R J, Turner-Warwick M
Q J Med. 1977 Oct;46(184):427-44.
Six patients with rapidly progressive airway obliteration in the absence of chronic bronchitis or emphysema are reported. Because this pattern of lung disease is very uncommon and five of the six patients had classical rheumatoid arthritis an association between the two diseases is suggested. The patients presented with rapidly developing breathlessness, and râles and a high-pitched mid-inspiratory squeak were heard over the lung fields. Chest radiographs showed distended lungs but were otherwise normal. Tests of lung fuction showed airflow obstruction, most marked at low lung volumes, with air trapping. The carbon monoxide gas transfer coefficient, maximum static recoil pressure and static compliance were normal. In spite of treatment with antibiotics, bronchodilators and corticosteroids, five died in respiratory failure five to 18 months after first becoming breathless. Post-mortem examination in four patients showed an obliterative bronchiolitis but no mucous gland hypertrophy or significant emphysema.
报告了6例在无慢性支气管炎或肺气肿情况下出现快速进行性气道闭塞的患者。由于这种肺部疾病模式非常罕见，且6例患者中有5例患有典型的类风湿性关节炎，因此提示这两种疾病之间存在关联。患者表现为迅速发展的呼吸困难，肺部可闻及啰音和高调的吸气中期哮鸣音。胸部X线片显示肺扩张，但其他方面正常。肺功能测试显示气流受限，在低肺容量时最为明显，并伴有气体潴留。一氧化碳气体转移系数、最大静态回缩压力和静态顺应性均正常。尽管使用了抗生素、支气管扩张剂和皮质类固醇进行治疗，但5例患者在首次出现呼吸困难后5至18个月死于呼吸衰竭。4例患者的尸检显示为闭塞性细支气管炎，但无黏液腺肥大或明显的肺气肿。
