What medications are used to treat pulmonary fibrosis? 

Medical treatments for pulmonary fibrosis have advanced significantly in recent years, focusing primarily on slowing the rate of lung scarring and preserving respiratory function. In the United Kingdom, the primary medications used for this purpose are a class of drugs known as antifibrotics. These treatments are not a cure and cannot reverse existing damage, but they represent a vital step in managing the long-term outlook for patients with idiopathic pulmonary fibrosis and other progressive lung conditions. Access to these medications is carefully managed by specialist respiratory centres to ensure they are prescribed safely and effectively according to national clinical guidelines. 

What We’ll Discuss in This Article 

• The two main antifibrotic medications available on the NHS. 

• How these drugs work to interfere with the scarring process. 

• The specific clinical criteria required to start treatment. 

• Common side effects and how they are monitored by specialists. 

• The role of other supportive medications for symptom relief. 

Antifibrotic medications: Pirfenidone and Nintedanib 

The most common medications used to treat pulmonary fibrosis in the UK are pirfenidone and nintedanib, which are specifically designed to reduce the speed of lung scarring. Pirfenidone and nintedanib are the two main medicines that can help slow down the progression of idiopathic pulmonary fibrosis in some people. Pirfenidone works by reducing the activity of the immune system and the production of collagen, which is the main component of scar tissue. Nintedanib, often known by the brand name Ofev, targets specific growth factor receptors that are involved in the fibrotic process. Both medications have been shown in large clinical trials to reduce the rate of decline in lung function, often by as much as 50% per year, which can help patients maintain their independence for a longer period. 

How these medications are administered 

These treatments are taken orally in the form of capsules or tablets, but the dosing schedules differ between the two. Pirfenidone is typically taken three times a day with food to help reduce stomach-related side effects. Patients usually start on a lower dose and gradually increase it over three weeks to reach the full therapeutic level. Nintedanib is usually taken twice a day, also with or after food. Because these are specialist medications, they are not usually prescribed by a GP but are instead supplied directly through a hospital-based specialist respiratory service or a dedicated home-delivery provider. This ensures that the specialist multidisciplinary team can closely monitor the patient’s response to the treatment. 

Criteria for receiving medication on the NHS 

To ensure that these medications are used where they provide the most benefit, the National Institute for Health and Care Excellence (NICE) has established specific eligibility criteria. NICE recommends pirfenidone and nintedanib for treating idiopathic pulmonary fibrosis only if the person has a forced vital capacity between 50% and 80% of what is expected. This measurement, known as FVC, is determined through a lung function test. If a person’s lung function is above 80%, they may be monitored until it reaches the eligibility threshold. Furthermore, there is a “stopping rule” in place: if a patient’s lung function falls by 10% or more within any 12 month period despite taking the medication, the treatment is typically reviewed and may be stopped as it is considered to no longer be providing sufficient benefit. 

Managing side effects and monitoring 

While antifibrotic medications are effective at slowing the disease, they are known to have a significant side-effect profile that requires careful management. Common side effects for both drugs include nausea, diarrhoea, and a loss of appetite, which can sometimes lead to unintended weight loss. Pirfenidone is specifically linked to photosensitivity, meaning the skin becomes much more sensitive to sunlight and can rash easily. Nintedanib is more commonly associated with gastrointestinal upset and diarrhoea. To manage these risks, patients must undergo regular blood tests to monitor their liver function, as both medications can occasionally cause liver inflammation. These tests are usually performed monthly for the first few months and then every three months thereafter. 

Other supportive medications 

In addition to antifibrotics, other medications may be prescribed to help manage the symptoms and complications associated with pulmonary fibrosis. For individuals with a persistent dry cough, doctors may suggest specific cough suppressants or low-dose morphine, which can be very effective at settling the cough reflex and reducing the sensation of breathlessness. If a patient also suffers from acid reflux, medications such as proton pump inhibitors (PPIs) are often prescribed, as stomach acid entering the airways can worsen lung irritation. For those whose fibrosis is linked to an autoimmune condition, immunosuppressant drugs like mycophenolate mofetil or steroids might be used to reduce the underlying inflammation before scarring becomes permanent. 

Conclusion 

Medications for pulmonary fibrosis in the UK focus on slowing the progression of lung scarring and managing the symptoms that affect quality of life. Antifibrotics like pirfenidone and nintedanib are the main clinical tools available, provided to patients who meet specific lung function criteria. While these drugs require careful monitoring for side effects, they offer a significant opportunity to preserve lung health for longer. If you experience severe, sudden, or worsening symptoms, call 999 immediately. 

Authority Snapshot (E-E-A-T Block) 

This article provides an evidence-based overview of the medications used to manage pulmonary fibrosis within the United Kingdom, strictly following the guidance of the NHS and NICE. The information is designed to help the general public understand the clinical criteria for treatment and the importance of specialist monitoring. This content has been produced by a medical content team and reviewed by Dr. Rebecca Fernandez, a UK-trained physician, to ensure clinical accuracy and patient safety.