Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, irreversible, and usually fatal interstitial lung disease of unknown cause [1, 2]. The aetiology of IPF is unknown, although identified risk factors for IPF include cigarette smoking, environmental exposures, microbial agents, age, male gender and gastroesophageal reflux disease (GERD). Genetic factors may also play a role in the aetiology of IPF as familial cases of IPF are described in approximately 5% of patients with IPF [2]. Nothing has shown significant anti-fibrotic activity in IPF patients and due to this high unmet medical need, numerous therapeutics are currently under clinical investigation. In this review, we shall focus on recombinant protein based approaches for the treatment of IPF, with a particular focus on pathophysiology of lung fibrosis using the bleomycin mouse model.
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