I. Malysse et al., COMPLETELY REVERSIBLE RESPIRATORY INSUFFICIENCY WITH PERSISTING ULTRASTRUCTURAL CILIARY ABNORMALITIES, The European respiratory journal, 7(8), 1994, pp. 1532-1536
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.