Untreated IPF Linked to 50% Mortality Rate at 3 Years

Yedida Y Bogachkov PhD avatar

by Yedida Y Bogachkov PhD |

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If left untreated by anti-fibrotic medications, idiopathic pulmonary fibrosis (IPF) has a 50% mortality rate after three years, according to a recent observational study using real-world data.

This is compared with a 26–31% mortality rate among IPF patients taking available anti-fibrotics, such as Esbriet (pirfenidone) and Ofev (nintedanib).

Additionally, the study found that patients who don’t take such medications face a more than 40% rate of respiratory-related hospitalizations.

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The study, “Mortality and Respiratory-Related Hospitalizations in Idiopathic Pulmonary Fibrosis Not Treated With Antifibrotics,” was published in the journal Frontiers in Medicine.

IPF is a progressive lung disease of unknown origin characterized by lung fibrosis, or scarring. There remains no cure, as yet, but two currently approved anti-fibrotic medications, Ofev and Esbriet, have been shown to help slow lung function decline in patients, and reduce their risk of respiratory-related hospitalizations.

However, many people with IPF often end up not being treated with either of these medications, according to the researchers.

When left untreated, the condition may lead to death within two to five years. But “real-world data regarding outcomes of idiopathic pulmonary fibrosis (IPF) are scarce, outside of registries,” the researchers wrote.

Now, these investigators used claims data from the French National Health System to conduct a population-based, observational study analyzing the mortality of patients with IPF.  The Système National des Données de Santé is a comprehensive, digital healthcare data resource covering 98.8% of the population in France — “one of the largest data repositories worldwide,” the team wrote.

A total of 5,360 patients diagnosed with IPF and not treated with anti-fibrotic therapies were included in the study. These individuals had a mean age of 75.5 and more than half (57.9%) were male.

In the year before the study’s start, 47.3% of the patients followed had scored a 5 or higher on the Charlson Comorbidity index, a tool that predicts a patient’s risk of death based on co-existing conditions — each assigned a weight from 1 to 6. A score equal to or greater than 5 is indicative of high morbidity.

Further, 74% of the patients had visited an office-based lung specialist or hospital practitioner at least once in the year before the study’s start, with the vast majority (81.7%) having experienced at least one acute respiratory-related hospitalization in that prior year. A total of 19.4% — more than 1,000 patients — also had needed to use supplemental oxygen, data showed.

After a mean of just over seven months — 223.2 days — following the study’s launch, 35.3% of the included patients had experienced an acute respiratory-related hospitalization. After three years, 41.5% had been hospitalized for respiratory-related events.

Over the follow-up period, at a mean of 236.2 days (nearly eight months) from study inclusion, 41.2% of the patients had died. By the three-year point, the mortality rate was 50.2%.

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“In summary, IPF patients untreated with antifibrotic therapies had poor outcomes, with a 50% all-cause mortality and a 42% cumulative incidence rate of acute respiratory-related hospitalization at 3 years,” the researchers wrote.

The team then compared these outcomes with those of IPF patients given anti-fibrotic therapies.

“Using the same dataset, we previously found cumulative incidence rates at 3 years of 26 and 31% for all-cause mortality, and of 48 and 44% for acute respiratory-related hospitalization, in patients newly treated with pirfenidone [Esbriet] and nintedanib [Ofev], respectively,” the researchers wrote.

These findings confirm the progressive, and ultimately fatal nature of IPF, in agreement with previous studies, the team suggested, while also noting the potential impact of anti-fibrotics such as Esbriet and Ofev.

Yet, the researchers noted their study had some limitations, including the diagnosis codes used to identify IPF and age limitations above 50, which were used in an attempt to exclude other fibrotic lung diseases. However, these methods may have excluded those who are younger and may have genetic forms of IPF, the team said.

“This observational study that included over 5,000 IPF patients showed that, if untreated with antifibrotic therapies, the disease is associated with a 50% all-cause mortality at 3 years,” the researchers wrote, adding that “these figures can serve as a historical control of the natural course of the disease.”