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.
- Kreuter, M.
- Walscher, J.
- Behr, J.
Keywords
- Disease Progression
- Enzyme Inhibitors/pharmacology
- Humans
- Indoles/*pharmacology
- *Lung Diseases, Interstitial/diagnosis/drug therapy
- Observational Studies as Topic
- Pyridones/*pharmacology
- Randomized Controlled Trials as Topic
- Treatment Outcome
