Diffuse interstitial lung disease (DLD) is characterised by varying degrees
of inflammation and fibrosis that result in derangement of the gas-exchang
ing units of the lung. A hallmark of these diseases is the abnormal deposit
ion of collagen, which is a prime determinant of clinical course. This revi
ew considers the current information concerning collagen turnover in diffus
e fibrotic lung disease. Considerable experimental evidence implicates both
increased collagen production and reduced degradation in these diseases. R
educed degradation may result from both reduced production of collagenases
and increased inhibition by tissue inhibitors. The known effects upon colla
gen turnover of novel therapeutic agents for pulmonary fibrosis (pirfenidon
e and interferon gamma) are discussed.