What rationale for treatment of occupational interstitial lung diseases with the drugs approved for idiopathic pulmonary fibrosis?

PURPOSE OF REVIEW: To critically discuss the rationale for the use of drugs approved for idiopathic pulmonary fibrosis (IPF) to treat occupational interstitial lung diseases (OILDs).

RECENT FINDINGS: Although IPF and OILDs share several clinical, radiological and probably pathogenetic features, currently, OILDs do not have a standard of care. In recent years, our knowledge and understanding of ILDs has improved substantially. Recently, the progressive pulmonary fibrosis (PPF) phenotype, which refers to non-IPF fibrotic ILDs that progress despite appropriate treatment, has been defined. OILDs may also be progressive. Nintedanib, initially approved for treatment of IPF, is also approved in patients with PPF. On the other hand, pirfenidone is approved in IPF but not in PPF, due to the lack of robust evidence of efficacy in this patient subset.

SUMMARY: OILDs are a large and highly heterogeneous group of conditions without a proper standard of care. Nintedanib may slow functional decline and disease progression in progressive OILDs, and new clinical trials are ongoing.