Pulmonary fibrosis commonly develops in systemic sclerosis. We assesse
d the role of thrombin in promoting fibroblast proliferation in the lu
ngs in this disorder. Bronchoalveolar lavage fluid (BALF) thrombin con
centrations were higher in ten patients with systemic sclerosis than i
n 12 healthy controls (14.6 vs 3.6 nmol/L, p<0.02), but values in pati
ents with cryptogenic fibrosing alveolitis (n=10) or sarcoidosis (n=10
) were not increased. BALF from all patients induced fibroblast prolif
eration. This proliferation was attenuated by thrombin inhibitors for
BALF from systemic sclerosis patients only. We suggest thrombin contri
butes to lung fibroblast proliferation in this disorder.