Antifibrotic drugs as treatment of nonidiopathic pulmonary fibrosis interstitial pneumonias: the time is now (?).

PURPOSE 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.