Idiopathic Pulmonary Fibrosis

Pirfenidone for Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a condition in which scar tissue gradually builds up in the lungs. "Idiopathic" means no specific cause can be found. As the tissue around the air sacs thickens and stiffens, oxygen passes less easily into the blood. IPF mainly affects people over 60, is more common in men and in people who have smoked, and causes breathlessness on exertion that slowly worsens, a persistent dry cough, tiredness and sometimes rounded, swollen fingertips (clubbing). It is diagnosed by a specialist team using a high-resolution CT scan and lung function tests.

How Pirfenidone Works

Pirfenidone is an antifibrotic medicine. It dampens the growth signals that drive the formation of scar tissue and has anti-inflammatory effects. In clinical trials it slowed the decline in lung capacity compared with placebo. It does not reverse existing scarring or cure IPF, and many people do not feel better on it. The aim is to protect remaining lung function for longer. It is started and monitored by a respiratory specialist.

How It Is Taken

The internationally labeled regimen builds up over two weeks to reduce stomach side effects:

• Days 1–7: 267 mg three times a day
• Days 8–14: 534 mg three times a day
• Day 15 onwards: 801 mg three times a day (2,403 mg per day), the maximum recommended dose

Take each dose with food, spread through the day, to reduce nausea and dizziness. Pirfenex 200 mg and Pirfenidone 200 mg come as 200 mg tablets, so the number of tablets at each step differs from the schedule above; your specialist will tell you exactly how many to take. If you stop for 14 days or more, the build-up usually needs to start again.

Side Effects and Monitoring

Common side effects include nausea, indigestion, diarrhea, loss of appetite and weight, tiredness, dizziness and rash.

• Sun sensitivity: the skin can burn or develop a rash in sunlight. Avoid direct sun and sunbeds, use a high-protection sunscreen and cover up with clothing and a hat.
• Liver: blood tests are needed before starting, monthly for the first six months and every three months after that. Report yellowing of the skin or eyes, dark urine or pain on the right side of the abdomen.
• Get urgent help for swelling of the face, lips or tongue, difficulty breathing, or a widespread blistering or peeling rash.

Precautions

Do not smoke: smoking lowers pirfenidone levels and further harms the lungs. Fluvoxamine must not be taken with pirfenidone, and some other medicines, including ciprofloxacin, require a lower dose. Avoid grapefruit juice. Pirfenidone is not suitable for people with severe liver disease or severe kidney disease, and it is not recommended in pregnancy.

For other lung conditions and medicines, browse our respiratory range.

Frequently Asked Questions

Will pirfenidone improve my breathing?

Usually not noticeably. Its role is to slow further decline, so stable lung function tests are a sign it is doing its job.

How long is it taken for?

Treatment is usually long term, for as long as it is tolerated and your specialist feels it is helping.

This page provides general information and is not a substitute for guidance from your lung specialist or pharmacist. Pirfenidone is a prescription medicine that requires specialist supervision and regular blood tests.