Interferon-γ1b for the treatment of idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) represents a particularly aggressive disease, the aetiology of which still remains unknown. The natural history of the disease often leads to respiratory failure and death, with a mortality rate greater than some cancers. To date, no management approach has proven to be efficacious for the treatment of this disease. IPF has been characterised as an ‘epithelial–fibroblastic disorder’, characterised by abnormal wound healing with excessive fibrosis and minimal inflammation. These emerging data have focused attention on antifibrotic drugs. Interferon-γ1b (IFN-γ1b) has recently been proposed as a promising candidate for the treatment of IPF. The reason for this is that IFN-γ1b has the ability to modulate the Th1/Th2 imbalance and to suppress fibroblast activation. The view that IPF is untreatable at present requires reconsideration, as improved survival has been suggested in three controlled trials of IFN-γ1b in IPF therapy.