What treatments are available to slow the progression of pulmonary fibrosis?
Pulmonary fibrosis is a chronic condition that causes the lungs to become scarred and stiff over time. While there is currently no way to reverse existing scarring, medical advancements have led to several treatments that can help slow down the rate at which the disease gets worse. In the United Kingdom, the primary focus of healthcare providers is to maintain lung function, manage symptoms, and improve the overall quality of life for patients. These treatments are usually managed by specialist respiratory teams within the NHS, ensuring that care is tailored to the specific type of fibrosis and the individual needs of the patient.
What We’ll Discuss in This Article
- The use of antifibrotic medications to reduce the speed of lung scarring.
- The role of pulmonary rehabilitation in building physical stamina.
- How lifestyle adjustments, such as smoking cessation, protect lung health.
- The application of oxygen therapy for managing blood oxygen levels.
- The criteria for lung transplantation in the UK healthcare system.
- Future developments in medication and upcoming clinical guidance.
Antifibrotic medications for lung scarring
The most significant pharmacological intervention for slowing the progression of pulmonary fibrosis involves a class of drugs known as antifibrotics. There are two main medicines available in the UK to help slow down the progression of idiopathic pulmonary fibrosis: pirfenidone and nintedanib. These medications work by interfering with the chemical signals that tell the body to produce more scar tissue. Pirfenidone reduces the activity of the immune system and the production of collagen, while nintedanib blocks specific enzymes that are involved in the scarring process. It is important to note that these drugs do not fix existing damage, but they have been shown in clinical trials to help preserve lung function for a longer period of time compared to those not taking the medication.
These medications are typically prescribed and monitored by specialists in interstitial lung disease (ILD). Because both drugs can affect liver function, patients must undergo regular blood tests, often monthly for the first six months and then every three months thereafter. Side effects are relatively common and can vary between the two drugs. Nintedanib is often associated with gastrointestinal issues, such as diarrhoea or nausea, while pirfenidone can make the skin highly sensitive to sunlight, a condition known as photosensitivity. If side effects become difficult to manage, specialists may adjust the dosage or switch the patient to a different medication to ensure they can continue treatment safely.
NICE criteria and monitoring treatment
The National Institute for Health and Care Excellence (NICE) provides clear standards for when these life-altering medications should be started and stopped. NICE recommends that antifibrotic medications should be used if breathing tests show a lung capacity between 50% and 80% of what would be expected. This measurement is known as the Forced Vital Capacity (FVC). By targeting this range, the NHS ensures that the medication is provided to individuals who are most likely to benefit from the slowing effect of the drugs. If a patient’s lung function remains stable or declines only slightly, they can usually continue the treatment for as long as it remains effective.
However, there are specific “stopping rules” implemented to ensure the medication is providing a clinical benefit. If a patient’s lung capacity falls by 10% or more within a single year despite being on the medication, the treatment is often reviewed and may be stopped. This is because a significant drop suggests the disease is progressing at a rate the current medication cannot sufficiently control. At this stage, the multidisciplinary team (MDT) will discuss alternative supportive care options or different clinical trials that might be available. This structured approach to monitoring ensures that patients receive the most appropriate care at every stage of their condition.
Pulmonary rehabilitation and physical support
Pulmonary rehabilitation is a vital non-pharmacological treatment that helps individuals manage the symptoms of lung scarring and stay active. Pulmonary rehabilitation is a structured programme of exercise and education designed for people with long-term lung conditions. These courses are usually held locally and are led by a team of physiotherapists, nurses, and occupational therapists. The programme typically lasts between six and eight weeks, with two sessions per week. Each session involves a combination of supervised physical exercise, such as walking or using a stationary bike, and educational talks on topics like managing breathlessness, nutrition, and psychological well-being.
The goal of pulmonary rehabilitation is not to heal the lungs, but to help the body use the available oxygen more efficiently. By strengthening the muscles in the arms and legs, the heart and lungs do not have to work quite as hard during daily activities. This can significantly reduce the sensation of breathlessness and increase a person’s stamina. Many patients find that the social aspect of the programme is also highly beneficial, as it allows them to connect with others who are facing similar challenges. In the UK, a referral for pulmonary rehabilitation can be made by a GP or a hospital specialist, and it is considered a cornerstone of chronic lung disease management.
Oxygen therapy and mobility
As pulmonary fibrosis progresses, the lungs may become less efficient at moving oxygen into the bloodstream, leading to low blood oxygen levels (hypoxia). If this happens, oxygen therapy may be prescribed to ensure the body’s vital organs receive enough fuel to function correctly. Oxygen can be delivered through a mask or small tubes that sit just inside the nostrils, connected to a machine called an oxygen concentrator. This therapy can be used only when you are moving around (ambulatory oxygen) or for 15 hours or more a day if oxygen levels are consistently low even at rest (long-term oxygen therapy).
Using oxygen can help reduce the strain on the heart and may make it easier for individuals to remain mobile and independent. However, oxygen is a medical treatment and must be prescribed by a specialist after a formal assessment, which involves checking oxygen levels both at rest and while walking. In the UK, portable oxygen cylinders or lightweight concentrators are available for those who wish to remain active outside their home. While oxygen therapy does not slow the scarring process itself, it is a crucial tool for protecting the heart and improving the ability to perform daily tasks without severe distress.
Lifestyle adjustments and preventative care
Maintaining a healthy lifestyle is one of the most effective ways to support lung health and potentially slow the decline in function. Quitting smoking is the single most important step a person can take if they have been diagnosed with pulmonary fibrosis. Smoking causes further inflammation and damage to the air sacs, which can accelerate the scarring process. The NHS provides extensive support for smoking cessation, including nicotine replacement therapy and behavioural support, which can be accessed through local GP surgeries.
Preventing lung infections is another critical part of managing the condition. Chest infections or the flu can cause a sudden and severe worsening of symptoms, known as an acute exacerbation, which can lead to permanent damage. Patients are strongly encouraged to stay up to date with their vaccinations, including the annual flu jab and the one-off pneumococcal vaccine. Maintaining a healthy weight is also important, as being overweight can put extra pressure on the diaphragm, making breathing more difficult, while being underweight can lead to muscle weakness and reduced stamina.
Future treatments and clinical research
The field of respiratory medicine is constantly evolving, and new treatments for pulmonary fibrosis are currently being developed and tested. Clinical trials are an essential part of the UK healthcare landscape, allowing researchers to evaluate new drugs that may be more effective or have fewer side effects than current options. For example, a new medication called nerandomilast has recently shown promise in international trials for its ability to slow lung function decline. In the UK, NICE is currently appraising this medication, with a decision regarding its availability on the NHS expected by late 2026.
Patients who are interested in participating in research are often encouraged to discuss this with their hospital specialist. Being part of a clinical trial can provide access to emerging therapies and helps the wider medical community understand how to treat the condition better in the future. While these new drugs represent a hopeful future, the current focus remains on the effective use of pirfenidone, nintedanib, and supportive therapies to protect the lung health of patients today.
Conclusion
Treatments for pulmonary fibrosis focus on slowing the rate of scarring and managing the symptoms that affect daily life. Antifibrotic medications like pirfenidone and nintedanib are the primary tools used in the UK to reduce disease progression, alongside supportive care like pulmonary rehabilitation and oxygen therapy. While the condition is progressive, a proactive approach involving early diagnosis, lifestyle changes, and specialist monitoring offers the best chance of maintaining independence and quality of life. If you experience severe, sudden, or worsening symptoms, call 999 immediately.
Can I take both pirfenidone and nintedanib together?
Current UK guidelines generally recommend taking only one of these medications at a time, as the safety and benefit of combining them are still being researched.
Will the medication make me feel better immediately?
No, these drugs are designed to slow down future damage rather than reduce current symptoms like cough or breathlessness.
What happens if I miss a dose of my medication?
You should follow the specific advice in your patient information leaflet, but generally, you should take the next dose as scheduled and not double up.
Is a lung transplant a common treatment?
No, lung transplants are reserved for a small number of people who are otherwise fit and meet strict clinical criteria, as donor lungs are rare.
How often will my lung function be tested?
In the UK, patients are typically reviewed every three to six months to track their breathing capacity and adjust their treatment plan.
Are there natural remedies that slow lung scarring?
There is currently no scientific evidence that herbal supplements or natural remedies can slow the progression of pulmonary fibrosis.
What is an ILD specialist nurse?
They are senior nurses who specialise in interstitial lung diseases and provide essential support, information, and coordination for your care.
Authority Snapshot (E-E-A-T Block)
This article provides an evidence-based overview of the treatments used to manage pulmonary fibrosis in the United Kingdom, strictly adhering to NHS and NICE protocols. The information is designed to help patients understand the clinical options available to slow disease progression and manage their symptoms effectively. This content was produced by a medical content team and reviewed by Dr. Rebecca Fernandez, a UK-trained physician, to ensure clinical accuracy and safety.
