Esbriet Stops Lung Fibrosis By Improving Functioning of Mitochondria, Study Shows

Esbriet Stops Lung Fibrosis By Improving Functioning of Mitochondria, Study Shows

Esbriet (pirfenidone) stops the lung scarring in idiopathic pulmonary fibrosis (IPF) by improving the functioning of mitochondria, cell components that convert food to energy and regulate cell death, according to a study.

The therapy centers on mitophagy, the process by which the body purges defective mitochondria. Mitophagy is a mitochondria quality control mechanism, and its impairment is associated with a number of diseases. Recent studies indicate pulmonary fibrosis is one.

Faulty mitophagy promotes myofibroblast differentiation, an impairment that contributes to IPF.  A myofibroblast is a cell between a fibroblast — a connective tissue that plays a crucial role in wound healing — and a muscle cell. Myofibroblast impairment inhibits the healing of lung scarring, or fibrosis.

The study, “Pirfenidone inhibits myofibroblast differentiation and lung fibrosis development during insufficient mitophagy,” was published in the journal Respiratory Research.

Esbriet is an approved anti-fibrotic drug that Genentech developed to treat IPF. It significantly halts the decline in a lung function measure known as forced vital capacity. And studies in mouse models of IPF have shown it to have anti-inflammatory and anti-fibrotic properties.

The mechanism that Esbriet uses to slow the progression of lung fibrosis progression was unknown until recently.

Researchers discovered that it promotes mitophagy in fibroblasts isolated from IPF lung tissue in a lab. They also learned that Esbriet-triggered mitophagy can inhibit myofibroblast differentiation.

To shed more light on Esbriet’s anti-fibrotic mechanisms, researchers analyzed its anti-oxidative properties. They had previously shown that when mitophagy was impaired, mitochondria produce more reactive oxygen species, molecules that can damage organs and tissue. The increase in reactive oxygen species contributed to myofibroblast differentiation, and thus fibrosis.

Researchers also discovered that when mitophagy was impaired, Esbriet suppressed mitochondria’s production of reactive oxygen species in lung fibroblasts. This stopped the fibrotic process.

The results were further confirmed in mice. Esbriet slowed the development of fibrosis in the lungs of bleomycin-treated mice, an established model for IPF.

Overall, the study helped clarify Esbriet’s mechanism of action. It showed that the drug “inhibits lung fibrosis development in the setting of insufficient mitophagy, which may at least partly explain the anti-fibrotic mechanisms of PFD [pirfenidone] for IPF treatment,” the team concluded.

9 comments

  1. Richard Groat says:

    Was on this for three months, did nothing to slow down process, told my doctor I am not taking them any longer. Can’t comment if they didn’t any good, but I sure feel a whole lot better since going off the medication.

    I hope this medicine helps others, did nothing for me.

    • Anne says:

      Really? I am takeing ofev and it has helped me alot. Has slowed down the disease alot for me.Have you tried the ofev? I thought they were both suppose to work the same way.

  2. RUSSELL G VAN SWERINGEN says:

    ESBPRIET IS TOO EXPENSIVE FOR MOST PEOPLE TO USE ON A REGULAR BASIS. IS THERE MUCH OF A CHANCE IN THE NEAR FUTURE THAT THIS MEDICINE WILL BECOME MORE AFFORDABLE ??

    • Anne says:

      It is expensive. Didn’t your doctor see about getting it paid for for you? Its thousands of dollars who can afford it.? They automatically just did it for me and it comes in the mail for me. They call each month when it’s time to re-order. They keep in touch with you if u need any help or have any questions about anything. They are very helpful.Where do u live? They didn’t even expect me to pay for the pills . They did it all for me then contacted me about it. Pill does work very well for me. I am takeing the ofev My doctor told me they r suppose to work the same way. Each have a few different side effects my doctor says.

    • Anne says:

      Did your doctor say that was long enough to see a difference? I am so sorry it didn’t work. I take the ofev and it works wonders for me. Did u have breathing tests every month or two to see if the pill was slowing the disease down? Did u try the ofev I thought they were suppose to work the same. When were u diagnosed with the disease? I am very thankful doctors put me on the ofev it works wonders for me. Did your doctor suggest you trying the of ev?

  3. William Newton says:

    Was on ofev for about one year and switched to espriet and shortly thereafter developed severe nose bleed ending up in emergency room . Switched back to ofev with no more nose bleeds.Had not had a nosebleed since childhood. I am 79 yrs old. IPF is stable.

  4. Robert Bell says:

    I am just starting on Espriet. Thanks for the comments. I am going to keep close watch instead of assuming this stuff is working.

  5. Robert says:

    Appears that some of you did not take the drug long enough to see what changes occurred in your lung function test (which is one of the best methods to determine if the drug is having a positive impact). Keep in mind that meds have to be used in conjunction with increased exercise in order for them to be effective. The drugs have little chance of being effective if one take them and then sits around to see if they work. You must exercise like your life depended on it (which it does). Walk walk walk.

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