AbstractPurpose of review
Therapeutic advances in the management of idiopathic pulmonary fibrosis (IPF) has led to improved outcomes with the use of the antifibrotic agents pirfenidone and nintedanib, with a number of randomized studies demonstrating benefits in slowing disease progression in IPF. However, treatment of other fibrosing interstitial lung diseases (ILD) remains challenging.Recent findings
Observational and uncontrolled studies investigating pirfenidone and nintedanib in non-IPF ILDs suggest potential benefits, although the data is weak. A number of randomized controlled trials evaluating pirfenidone and nintedanib in a variety of fibrosing ILDs are currently enrolling or planned.Summary
The review will discuss the rationale for use of established antifibrotic drugs approved for IPF for use in non-IPF ILD, describe supportive data from observational studies and ongoing clinical trials.