PROGRESSIVE AIRWAY OBLITERATION IN ADULTS AND ITS ASSOCIATION WITH RHEUMATOID DISEASE

  • 1 January 1977
    • journal article
    • research article
    • Vol. 46  (184) , 427-444
Abstract
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 5 of the 6 patients had classical rheumatoid arthritis, an association between the 2 diseases is suggested. The patients presented with rapidly developing breathlessness, and a high-pitched mid-inspiratory squeak was heard over the lung fields. Chest radiographs showed distended lungs, but were otherwise normal. Tests of lung function showed airflow obstruction, most marked at low lung volumes, with air trapping. The CO gas transfer coefficient, maximum static recoil pressure and static compliance were normal. In spite of treatment with antibiotics, bronchodilators and corticosteroids, 5 died in respiratory failure 5-18 mo. after first becoming breathless. Post-mortem examination in 4 patients showed an obliterative bronchiolitis, but no mucous gland hypertrophy or significant emphysema.