Non-steroid agents for idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis is a form of progressive lung disease which ultimately leads to death. The cause is unknown, but the disease is characterised by scar tissue in the lungs. This prevents the lungs from working effectively. Standard treatment uses oral corticosteroids in association with immunosuppressors, but there is uncertainty as to whether this treatment is effective. Immunosuppressive agents such as azathioprine and cyclophosphamide have been used to treat the disease because it is thought they might prevent inflammation. The review found 15 high quality trials of non-steroid drugs tested in idiopathic pulmonary fibrosis patients. Notwithstanding the encouraging results of a first small study included in the first version of this review, the effects of interferon gamma-1beta, as assessed by combining two subsequent large trials, were disappointing and failed to show an effect on improving survival. Four studies did evaluate pirfenidone, an anti-fibrotic oral drug, on a large number of patients: although two of these studies have only been presented in conferences,combining the published and unpublished data showed a significant improvement of pirfenidone on progression-free survival and a small increase in pulmonary function. Current evidence suggests a possible role for pirfenidone in the treatment of idiopathic pulmonary fibrosis, though data on survival are now needed. However, trials with other non-steroid agents are currently ongoing and new evidence may become available soon.