COMPLETELY REVERSIBLE RESPIRATORY INSUFFICIENCY WITH PERSISTING ULTRASTRUCTURAL CILIARY ABNORMALITIES

A male nonsmoker, born in 1946, suffered from hypersecretory bronchiti s and recurrent upper and lower respiratory tract infections since ado lescence. In 1983 he developed pulmonary insufficiency. In 1984, some months after daily occupational exposure to printing inks and glues, f orced expiratory volume in one second (FEV(1)) was 1.1 l(28% pred) and arterial oxygen tension (Pao(2)) 44 mmHg (5.9 kPa). Open lung biopsy showed a centri-acinar bronchiolitis-alveolitis suggestive of an airbo rne toxic pathology. Ultrastructural examination of the bronchial muco sa revealed microtubular abnormalities in about 10% of the cilia. Trea tment consisted of stopping work from December 1985, oral corticostero ids until 1988, bronchodilators on a regular basis and intermittent an tibiotics (1 week/month). The evolution was remarkable because: 1) fro m a severe pulmonary insufficiency, refractory to bronchodilators and corticosteroids for several years, lung function slowly improved to su pernormal values, without residual functional signs of small airways d isease; and 2) a repeat ultrastructural nasal mucosa examination in 19 99 still showed persisting microtubular abnormalities in about 10% of the cilia, which disappeared on sequential monolayer-suspension cultur e, thus indicating secondary ciliary abnormalities.