Exciting news for people living with idiopathic pulmonary fibrosis (IPF). Two large clinical trials TETON-1 and TETON-2 showed that an inhaled medication called treprostinil (Tyvaso) slows the loss of lung function in patient with IPF.
These results are important because IPF is a progression lung disease that causes scarring of the lungs. Over time, the healthy lung gets replaced by scarr. This impairs the function of the lungs. We currently have no treatments that improve lung function. However, we have several treatments that can slow the accumulation of scar in the lungs.
What Is Treprostinil?
Treprostinil is a medication that has been used for years to treat certain types of pulmonary hypertension, a condition involving high blood pressure in the blood vessels of the lungs. It is taken as either a dry powder inhalation or a nebulizer four times daily.
Researchers have discovered that treprostinil may also help reduce the processes that cause lung scarring. This led to the TETON studies, which tested whether inhaled treprostinil could slow the progression of IPF.
What Did the Studies Find?
The TETON-1 and TETON-2 studies included nearly 1,200 patients with IPF. Patients received either inhaled treprostinil or a placebo (a treatment with no active medication) for year. Patients were allowed to be currently approved antifibrotic medications such as OFEV (nintedanib) or Esbriet (pirfenidone).
Researchers measured lung function using a test called forced vital capacity (FVC). This quick breathing test measures how much air a patient can blow out of their lungs after a big inhalation.
Both studies showed that patients receiving treprostinil lost significantly less lung function than those receiving the placebo.
When the two studies were combined, Tyvaso treated patients had 75% less loss of lung function over the year compared to placebo. Furthermore, the Tyvaso group had a 31% reduction in the risk clinical worsening and approximately 50% lower risk of an IPF exacerbation.
What About Side Effects?
The most common side effect was coughing. Other side effects included headache and diarrhea. Most were mild to moderate, but some patients stopped treatment because of side effects.
What Do These Results Mean for People with IPF?
The TETON studies offer real hope for people living with IPF. For the first time, two large clinical trials have shown that inhaled treprostinil can slow the loss of lung function in patients with IPF. Importantly, these benefits were seen in patients already being treated with currently approved antifibrotic medications.
However, treprostinil is not a cure for IPF. It has not been shown to reverse existing lung scarring, nor improve survival.
The Bottom Line
The TETON studies of Tyvaso in IPF moves the ball forward. We anticipate the FDA will expand the labeled indication soon to include treating IPF
For patients and families facing IPF, these findings offer hope that another effective treatment is likely to become available soon.
Patients should speak with their pulmonary fibrosis specialists about what these results may mean for their care.