Clinical Management of Progressive Fibrosing ILD

Toby M. Maher, MD, PhD


May 18, 2021

Interstitial lung disease (ILD) and pulmonary fibrosis (PF) are umbrella terms for a broad range of lung disorders characterized by inflammation and fibrotic disorders of lung tissue.

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing type of interstitial lung disease that is both chronic and fatal, with an average survival rate of 5 years. IPF is the most typical presentation of progressive fibrosing ILD (PF-ILD). Patients with PF-ILD will eventually exhibit a progressive fibrosing phenotype.

Recent advances in understanding ILD phenotypes has led to novel antifibrotic therapies shown to reduce disease progression.

Toby M. Maher, director of the Interstitial Lung Disease Program at Keck School of Medicine in Los Angeles, discusses the current treatment options for PF-ILD, including pirfenidone, studied in the CAPACITY and ASCEND trials, and nintedanib, evaluated in the INPULSIS and INBUILD trials.


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