Treatments for Interstitial Lung Disease

Medications for interstitial lung disease are limited, and can carry a high burden of side effects, which patients in primary care may need support to manage.

They can be divided into two broad categories:

• Antifibrotic medications to slow development of scarring (fibrosis) in the lungs.
• Immunosuppressant medications to target inflammatory disease (which is potentially reversible)

Antifibrotics

Antifibrotic medications are used to manage pulmonary fibrosis by slowing the progression of lung fibrosis.

These therapies can reduce the rate of fibrosis progression, but they do not stop the scarring process entirely, or reverse existing scar tissue in the lungs.

Antifibrotics are prescribed by your patient's specialist team and are usually delivered directly to the patient at home.

There are two antifibrotics currently available to patients. 

Nintedanib

Nintedanib is licensed for:

• Idiopathic pulmonary fibrosis
• Progressive pulmonary fibrosis

The specialist team will determine eligibility according to local prescribing criteria.

Patients can stay on Nintedanib for as long as the benefits continue to outweigh any side effects.

Nintedanib is usually taken twice daily, approximately 12 hours apart, with or immediately after food.  The usual dose is 150 mg twice daily, although this may be reduced to 100 mg twice daily if side effects are difficult to tolerate.

The most common side effect is diarrhoea. Other frequently reported side effects include nausea, vomiting, reduced appetite and abdominal pain. Less commonly, patients may develop liver dysfunction or experience bleeding, such as epistaxis.

Nintedanib can interact with other medications. Some medicines may affect how Nintedanib works, while others can increase the risk of side effects. In particular, anticoagulants such as warfarin, apixaban, rivaroxaban and dabigatran may increase the risk of bleeding.

Pirfenidone

Pirfenidone is licensed for

• Idiopathic pulmonary fibrosis

The specialist team will determine eligibility according to local prescribing criteria.

Patients can continue taking pirfenidone for as long as the benefits outweigh any side effects. Treatment is reviewed regularly by the specialist ILD team using lung function tests, symptoms and tolerability. NICE recommends stopping treatment if there is evidence of disease progression, defined as a decline in predicted FVC of 10% or more within a 12 month period.

Pirfenidone is taken three times daily with or immediately after food. The target dose is 801 mg three times daily. If side effects occur, a dose reduction may be considered by the medical team.

Side effects include gastrointestinal symptoms and photosensitivity. Patients should be advised to avoid direct sunlight and apply SPF 50 sunscreen year-round. Some patients use UV protective clothing.

Smoking reduces Pirfenidone’s effectiveness, so smoking cessation is vital. Grapefruit and grapefruit juice should be avoided.

Medicines that can reduce the effectiveness of or interact with Pirfenidone include omeprazole and rifampicin.

Action for Pulmonary Fibrosis has information leaflets for patients who are taking antifibrotic medications.

Routine monitoring for patients taking antifibrotics

Regular monitoring is essential while a patient is taking antifibrotic medication. This may include:

• Regular assessment of lung function, including spirometry and gas transfer testing
• Monitoring and management of side effects
• Blood tests, including liver function monitoring
• Review of symptoms, exercise tolerance and overall wellbeing

CT scans are not usually performed routinely but may be requested by the specialist team if there is concern about disease progression or a change in symptoms.

Immunosuppressants

Immunosuppressive medications are used to treat ILDs where inflammation plays a significant role, such as hypersensitivity pneumonitis, sarcoidosis and autoimmune ILDs.  

These medications work by reducing inflammation and suppressing the immune response. In some patients, this can help improve symptoms, stabilise lung function and slow disease progression.

Immunosuppressant medications include:

• Corticosteroids: Prednisolone
• Immunomodulators: Mycophenolate, Cyclophosphamide, Azathioprine, Methotrexate
• Biologic agents: Rituximab, Tocilizumab

Your patient's specialist team will decide which medication and dose is best for your patient and how and where this should be prescribed.  They will advise what monitoring is required.

Immunosuppressant drugs can increase susceptibility to infections, including viruses like chickenpox and measles. Live vaccines are contraindicated during treatment with these medications. Vaccination status should ideally be reviewed before immunosuppressive treatment starts. Household and close contacts may need to be fully vaccinated according to the national schedule to reduce exposure risk.

Oxygen

Breathlessness is a common and often distressing symptom of ILD. As the disease progresses, patients with ILDs often require oxygen therapy due to chronic hypoxemia and to avoid complications such as pulmonary hypertension, right-sided heart failure (cor pulmonale), and polycythemia.

In ILD, ambulatory oxygen may be required even in the early stages of disease because of the exertional desaturation which is a feature of the condition.  This group of patients generally have higher requirements for oxygen than people with COPD.  

Patients with resting oxygen saturations of 92% or below, or significant exertional desaturation, should be referred for specialist oxygen assessment. The specialist oxygen team will determine the most suitable oxygen delivery system, flow rate and monitoring requirements.

Patients using oxygen at home may benefit from additional support and education. See our patient guide for living with home oxygen.  Action for Pulmonary Fibrosis also has information for patients on home oxygen, including advice on coping with the cost of using an oxygen concentrator.

Cough 

Cough is a common and often distressing symptom of ILD, particularly pulmonary fibrosis. It is typically dry, persistent and difficult to control, and can have a significant impact on quality of life.  More frequent and severe cough has been associated with disease progression, reduced exercise tolerance and poorer quality of life.

Many people with ILD experience cough hypersensitivity, where the cough reflex becomes overly sensitive. Common triggers include cold air, strong smells, smoke, vaping, cleaning products, perfumes and dust.

Although cough is often caused by the underlying lung disease, it may be worsened by other treatable conditions, including gastro-oesophageal reflux disease (GORD), postnasal drip, respiratory infections, airway inflammation, medication side effects and other respiratory conditions.

There are currently no licensed treatments specifically for cough associated with ILD. However, this does not mean that nothing can be done. Specialist ILD teams can assess for treatable causes of cough, offer symptom management strategies and, where appropriate, prescribe medications to help reduce cough severity.

Respiratory physiotherapists and speech and language therapists can provide cough suppression techniques and other non pharmacological approaches that may help some patients.

Action for Pulmonary Fibrosis' factsheet on cough management outlines coping strategies for ILD cough.

Pulmonary rehabilitation 

Breathlessness can make patients with ILD reluctant to exercise, but avoiding activity can lead to muscle weakness, reduced fitness and worsening symptoms. Encouraging patients to remain as active as possible is therefore an important part of ILD management.

Pulmonary rehabilitation is recommended for people with ILD who are limited by breathlessness. Courses include:

• Structured education
• Lifestyle support  
• Personalised exercises so that people with ILD can be confident to live well with their condition.

There are significant waiting times in some regions.  To bridge this gap, signpost your patient to:

The A+LUK Keep Active Programme

The Keep Active Programme provides advice on how to move safely with a lung condition, breathing techniques to use during activity, gentle warm up and cool down exercises, step by step exercise programmes designed to improve strength and energy levels, and inspiring stories from people living with lung conditions who have successfully become more active.

Keep Active has been created by specialist respiratory physiotherapists for anyone living with a lung condition and includes three levels for your patient to choose from.  

The videos are supported by the My Keep Active Handbook.

Lung transplantation

For a small number of patients, lung transplantation may be considered if pulmonary fibrosis progresses despite treatment and the patient is otherwise suitable for transplantation.

Lung transplantation is relatively uncommon. In 2023 to 2024, 135 adult lung transplants were performed in the UK, not all for pulmonary fibrosis. Patients who may be suitable for transplantation are referred by their specialist team to a transplant centre for a comprehensive assessment. If considered suitable, they may be placed on the transplant waiting list.

Lung transplantation can improve quality of life and survival in selected patients, but it carries significant risks and requires lifelong follow up and immunosuppressive treatment. 

Action for Pulmonary Fibrosis has comprehensive information for patients about lung transplantation.