Respiratory
Antifibrotic medicines
Pirfenidone and nintedanib (pulmonary fibrosis) — Tablets that slow the scarring of the lungs in idiopathic pulmonary fibrosis and related conditions.
Education and reference only. This is a plain-language class overview — it deliberately contains no doses. Always check the current Summary of Product Characteristics (SmPC), the BNF and your local formulary before prescribing or administering any medicine.
Quick answer
What is Antifibrotic medicines?
Antifibrotic medicines (pirfenidone and nintedanib) are specialist tablets that slow the scarring of the lungs in idiopathic pulmonary fibrosis and related progressive fibrosing lung diseases. They slow the loss of lung function rather than reversing damage.
- How it works: In pulmonary fibrosis the lung tissue becomes progressively scarred and stiff, making breathing harder over time.
- In practice: In practice antifibrotic medicines (pirfenidone and nintedanib) are specialist treatments that slow the progressive lung scarring of idiopathic pulmonary fibrosis (IPF) and some other progressive fibrosing lung diseases.
What it is
Antifibrotic medicines (pirfenidone and nintedanib) are specialist tablets that slow the scarring of the lungs in idiopathic pulmonary fibrosis and related progressive fibrosing lung diseases. They slow the loss of lung function rather than reversing damage.
How it works
In pulmonary fibrosis the lung tissue becomes progressively scarred and stiff, making breathing harder over time. These medicines interfere with the signalling that drives that scarring — pirfenidone broadly damps the fibrotic and inflammatory process, while nintedanib blocks growth-factor pathways (and blood-vessel signalling) involved in fibrosis — so the lungs scar more slowly.
In practice
In practice antifibrotic medicines (pirfenidone and nintedanib) are specialist treatments that slow the progressive lung scarring of idiopathic pulmonary fibrosis (IPF) and some other progressive fibrosing lung diseases. They do not reverse existing scarring or cure the disease, but they slow the decline in lung function and are an important advance for a condition that was previously untreatable. They are started and supervised by specialist interstitial-lung-disease centres, usually based on lung-function criteria. The two have different side-effect profiles that guide choice. Pirfenidone commonly causes nausea, loss of appetite, tiredness and notable photosensitivity (so strict sun protection is needed), and can affect the liver. Nintedanib commonly causes diarrhoea (often manageable with prompt treatment and dose adjustment), nausea and liver enzyme changes, and — because it also affects blood vessels — carries a small increased bleeding risk and is used cautiously with anticoagulants and around surgery. Both need liver-function monitoring, both are affected by smoking (which reduces nintedanib levels) and various drug interactions, and both are generally avoided in pregnancy. They sit within a wider plan that includes oxygen, pulmonary rehabilitation, vaccination, treatment of reflux and cough, and consideration of transplant.
Examples
Practical use
How to take it & use it well
- Take it steadily as prescribed and with food, as taking it with meals helps both how well it works and how well you tolerate it; it slows the scarring of your lungs over time.
- Understand that it slows the decline in lung function rather than reversing scarring or curing the disease, so steady long-term use is what matters.
- If you take pirfenidone, protect your skin carefully from the sun, using clothing that covers up and a high-factor sunscreen, as it makes your skin much more sensitive to sunlight.
- If you take nintedanib, treat any diarrhoea early with plenty of fluids and the advice you have been given, and tell your team, as it is common but usually manageable.
- Keep up your liver blood tests, as both medicines can affect the liver and these checks guide whether the dose is safe.
- Tell your team about any new medicines, any unusual bleeding or bruising, and any planned surgery, especially if you take nintedanib.
Common uses
- Idiopathic pulmonary fibrosis (IPF)
- Other progressive fibrosing interstitial lung diseases
- Slowing decline in lung function (specialist-supervised)
Monitoring
- Lung function (to judge benefit) and symptoms
- Liver-function blood tests on a regular schedule
- Tolerability — nausea, weight, diarrhoea; bleeding risk and skin (sun) reactions
Weighing it up
Advantages & disadvantages
Advantages
- They slow the progressive lung scarring of idiopathic pulmonary fibrosis, a condition that was previously untreatable.
- They slow the decline in lung function, which can help preserve breathing for longer.
- Having two options with different side-effect profiles means treatment can be matched to the person.
- They are an important advance and are started and supervised by specialist interstitial-lung-disease centres.
- They fit within a wider plan that includes oxygen, pulmonary rehabilitation, vaccination and other support.
Disadvantages
- They slow but do not reverse or cure the scarring, so they manage rather than fix the condition.
- Pirfenidone commonly causes nausea, loss of appetite, tiredness and marked sensitivity to sunlight needing strict sun protection.
- Nintedanib commonly causes diarrhoea, along with nausea and liver enzyme changes.
- Nintedanib slightly increases the risk of bleeding, so it is used cautiously with blood-thinners and around surgery.
- Both need liver-function monitoring and are generally avoided in pregnancy, and smoking lowers nintedanib levels.
Key safety principles
What to watch for
- They slow, but do not reverse or cure, lung scarring — and need liver-function monitoring; both are generally avoided in pregnancy.
- Pirfenidone: nausea, appetite loss and marked photosensitivity — strict sun protection (clothing and high-factor sunscreen) is essential.
- Nintedanib: diarrhoea is common (treat promptly); it slightly increases bleeding risk, so caution with anticoagulants and around surgery. Smoking lowers its levels.
Key interactions
What to avoid or check alongside
- Nintedanib slightly increases the bleeding risk, so it is used cautiously with anticoagulants and other blood-thinners, and is often paused around surgery.
- Smoking reduces the level of nintedanib in the body, so stopping smoking matters both for your lungs and for the medicine working properly.
- Both medicines can affect the liver, so liver-function tests are monitored and other liver-affecting drugs are taken into account.
- Pirfenidone causes marked photosensitivity, so strong sun and sunbeds are avoided and sun protection is essential while taking it.
- Tell your team about all your medicines, including those bought without prescription, as various drug interactions can change the levels of these treatments.
Patient & carer advice
- These slow the scarring of your lungs over time — taking them steadily, with food, helps both how they work and how well you tolerate them
- With pirfenidone, protect your skin carefully from the sun; with nintedanib, treat any diarrhoea early and tell your team
- Keep up your liver blood tests, and tell your team about any new medicines, bleeding, or planned surgery
Use with
Related clinical calculators
Dose and risk decisions for this class often depend on renal function, weight or bleeding/stroke risk. These tools help:
Answers
Antifibrotic medicines: frequently asked questions
What do antifibrotic medicines do in pulmonary fibrosis?
Pirfenidone and nintedanib are specialist treatments that slow the progressive lung scarring of idiopathic pulmonary fibrosis and some related conditions. They do not reverse existing scarring or cure the disease, but they slow the decline in lung function, which is an important advance for a condition that previously had no treatment. They are started and supervised by specialist centres.
Why is sun protection so important with pirfenidone?
Pirfenidone makes your skin much more sensitive to sunlight, so you can burn easily even on a dull day. Strict sun protection is essential: cover up with clothing, wear a hat and use a high-factor sunscreen, and avoid sunbeds. This is one of the most important practical points with this medicine, so take it seriously all year round.
How do I cope with diarrhoea from nintedanib?
Diarrhoea is common with nintedanib, but it is usually manageable. Treat it early by keeping up your fluids and using the anti-diarrhoea advice your team has given you, and let them know it is happening. Catching it early often keeps it under control, and your team may adjust the dose if needed rather than stopping the medicine.
Why do I need liver blood tests on these medicines?
Both pirfenidone and nintedanib can affect the liver, sometimes raising liver enzymes without you feeling anything. Regular liver blood tests let your team pick this up early and adjust the dose if needed to keep you safe. Keeping to these tests is an important part of taking either medicine over the long term.
Will these medicines cure my pulmonary fibrosis?
No. They slow the progression of the scarring and the decline in lung function, but they do not reverse the scarring already there or cure the disease. They are still a worthwhile advance, as they help preserve breathing for longer. They work best as part of a wider plan including oxygen, pulmonary rehabilitation and vaccination.
Authoritative sources
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