Jb. Cailes et al., NEUTROPHIL ACTIVATION IN FIBROSING ALVEOLITIS - A COMPARISON OF LONE CRYPTOGENIC FIBROSING ALVEOLITIS AND SYSTEMIC-SCLEROSIS, The European respiratory journal, 9(5), 1996, pp. 992-999
Fibrosing alveolitis complicating systemic sclerosis (FASSc) carries a
better prognosis than lone cryptogenic fibrosing alveolitis (CPA), We
wanted to determine whether this improved prognosis is associated wit
h differential neutrophil migration and activation in the lower respir
atory tract. We therefore compared bronchoalveolar lavage (BAL) neutro
phil numbers and levels of neutrophil-derived enzymes in FASSc, CFA an
d normal individuals, Bronchoalveolar lavage was performed on 45 subje
cts (FASSc n=20; CFA n=15; normals n=10); cell counts and levels of ne
utrophil-derived enzymes, myeloperoxidase, elastase (total elastase an
d elastase/alpha(1)-antitrypsin complexes), collagenase and lactoferri
n were measured, Lung function testing was performed in subjects with
fibrosing alveolitis. Significant differences in the levels of collage
nase, myeloperoxidase and elastase/alpha(1)-antitrypsin complexes were
present in the BAL fluid from the three groups, Patients with CFA had
significantly higher neutrophil percentages and levels of collagenase
and myeloperoxidase than those with FASSc, Disease extent, as judged
by lung volumes and gas transfer, was comparable in the CFA and FASSc
groups. Forced vital capacity (% predicted) was significantly lower in
patients with evidence of increased neutrophil enzyme release than th
ose without, We conclude that: 1) increased neutrophil migration to th
e lung is accompanied by release both of primary and secondary granule
enzymes in cryptogenic fibrosing alveolitis; and 2) the lower amounts
of neutrophil products in fibrosing alveolitis complicating systemic
sclerosis may account for the improved prognosis, even when disease is
as extensive as in cryptogenic fibrosing alveolitis.