Combination treatment extends survival in PH, pulmonary fibrosis
Pulmonary vasodilators with antifibrotics may put off need for transplant
Combining pulmonary vasodilators, medications that widen blood vessels, and antifibrotics, which slow the progression of scarring, may prolong survival and time without needing a transplant in people with both pulmonary hypertension (PH) and pulmonary fibrosis, a study suggested.
“Antifibrotic and pulmonary vasodilator therapy could improve transplant-free survival in patients with pulmonary hypertension related to pulmonary fibrosis,” wrote three researchers from Temple University Hospital in Philadelphia.
The study, “Antifibrotic therapy combined with pulmonary vasodilator therapy may improve survival in patients with pulmonary fibrosis and pulmonary hypertension: a retrospective cohort study,” was published in Therapeutic Advances in Respiratory Disease.
Pulmonary hypertension occurs when blood pressure in the vessels that carry blood to the lungs becomes abnormally high. This makes it harder for the heart to pump blood to the lungs, leading to PH symptoms such as shortness of breath. Many patients also have pulmonary fibrosis, which occurs when inflammation or damage causes scar tissue to form in the lungs.
To treat pulmonary hypertension, doctors typically prescribe pulmonary vasodilators, which help lower blood pressure in the lungs. To slow scarring, they use antifibrotics. While these medications have shown benefits on their own, it remains unclear how well they work when used together in patients with both diseases.
Combination treatment shows benefits
To learn more, the researchers looked back at medical records of 155 patients who had both pulmonary hypertension and pulmonary fibrosis. Twenty-four of these patients were treated with both types of medication, while the other 131 patients received only antifibrotics.
The proportion of patients with severe pulmonary hypertension was about twice as high in the group that received both pulmonary vasodilators and antifibrotics compared with those who only received antifibrotics (50% vs. 25.2%).
However, they lived significantly longer without needing lung transplants compared with those who only received antifibrotics (19.1 months vs. 9.4 months). Transplant-free survival was also significantly higher in patients who received both types of medication (25% vs. 2.3%).
“[Patients] treated with combination therapy with antifibrotics and pulmonary vasodilators may have improved transplant-free survival when compared to those treated with antifibrotic therapy alone, despite the combination therapy group having more severe [pulmonary hypertension] at baseline,” the researchers wrote.
Combining pulmonary vasodilators and antifibrotics reduced the risk of death or needing a lung transplant by 76%, even after adjusting for lung function and other clinical measures. Patients who received both types of medication tended to walk farther in a six-minute walk test, a standard test of exercise capacity, but this was not statistically significant.
While the study suggests benefits of combining the two types of medication, “randomized studies are needed to confirm these findings and further explore the long-term outcomes of combination therapy in appropriate patients with pulmonary fibrosis and [pulmonary hypertension],” the researchers wrote.
The relatively small number of participants, particularly in the combination treatment group, was among the study’s limitations, they said.
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