Idiopathic pulmonary fibrosis (IPF) is associated with a 50% all-cause mortality at 3 years if not treated with antifibrotics, according to the results of an observational study published in Frontiers in Medicine.

“These figures can serve as a historical control of the natural course of the disease,” Vincent Cottin, MD, PhD, and the coauthors of the study wrote.

Outside of patient registries, there is little real-world data available regarding outcomes in IPF, a chronic, progressive lung disease characterized by the buildup of fibrotic tissue in the pulmonary parenchyma.

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Here, a team of researchers led by Manon Belhassen, PhD, from Pharmaco Epidemiology Lyon in France used data from the French National Health System to report outcomes in patients diagnosed with IPF from 2015 to 2016 who were not treated with antifibrotics.

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The researchers identified 5360 patients who were diagnosed with IPF but were not treated with antifibrotics during this period. The mean age of the patients was 75.5 years, and slightly more than half (57.9%) were male.

During follow-up, 41.2% of the patients died. The researchers calculated that the cumulative incidence of death at 3 years was 50.2%. Approximately 35.3% of the patients had an acute respiratory-related hospitalization.

IPF is thought to affect 10 people per 100,000 every year. The median survival of patients after diagnosis is around 3 to 5 years. Currently, there is no cure for the disease, and available treatments can only help manage symptoms.

The antifibrotic medication pirfenidone can be prescribed, and clinical trial results have shown that it may reduce mortality. However, the drug is very expensive, and its effects on patient quality of life are unclear. Pirfenidone can also cause many adverse effects, including photosensitivity, fatigue, abdominal pain, and anorexia.


Cottin V, Spagnolo P, Bonniaud P, et al. Mortality and respiratory-related hospitalizations in idiopathic pulmonary fibrosis not treated with antifibrotics. Front Med (Lausanne). 2021;8:802989. doi:10.3389/fmed.2021.802989