Completely reversible respiratory insufficiency with persisting ultrastructural ciliary abnormalities

A male nonsmoker, born in 1946, suffered from hypersecretory bronchitis and recurrent upper and lower respiratory tract infections since adolescence. In 1983 he developed pulmonary insufficiency. In 1984, some months after daily occupational exposure to printing inks and glues, forced expiratory volume in one second (FEV1) was 1.1 l (28% pred) and arterial oxygen tension (PaO2) 44 mmHg (5.9 kPa). Open lung biopsy showed a centri-acinar bronchiolitis-alveolitis suggestive of an airborne toxic pathology. Ultrastructural examination of the bronchial mucosa revealed microtubular abnormalities in about 10% of the cilia. Treatment consisted of stopping work from December 1985, oral corticosteroids until 1988, bronchodilators on a regular basis and intermittent antibiotics (1 week/month). The evolution was remarkable because: 1) from a severe pulmonary insufficiency, refractory to bronchodilators and corticosteroids for several years, lung function slowly improved to supernormal values, without residual functional signs of small airways disease; and 2) a repeat ultrastructural nasal mucosa examination in 1992 still showed persisting microtubular abnormalities in about 10% of the cilia, which disappeared on sequential monolayer-suspension culture, thus indicating secondary ciliary abnormalities.