Pulmonary fibrosis: symptoms, causes, treatment and outlook

Pulmonary means to do with the lungs and fibrosis means scarring. Asthma + Lung UK explains that pulmonary fibrosis affects the tissue in and around the tiny air sacs in the lungs (interstitial tissue). That tissue gives the lungs structure and helps oxygen and carbon dioxide move in and out.

Scarring makes it harder for the lungs to expand fully when you breathe in, and harder for oxygen to move from the lungs into the blood. People with pulmonary fibrosis can therefore have low blood oxygen levels.

Pulmonary fibrosis is a long-term condition and part of a larger group of lung diseases called interstitial lung diseases. There are many types of ILD. Some can lead to pulmonary fibrosis, but not all do.

Some types of pulmonary fibrosis stay the same or change very slowly, and some are progressive and get worse over time. Idiopathic pulmonary fibrosis (IPF) is a progressive type. Idiopathic means there is no known cause.

Asthma + Lung UK says treatment aims to slow the rate of scarring, keep the condition as stable as possible, help manage symptoms, help you stay as active as possible and support emotional wellbeing. The choice depends on the type you have, what has caused it and whether it is progressing.

The two main antifibrotic medicines are pirfenidone, mainly used for IPF, and nintedanib, which is used for IPF and can also be used for other progressive fibrotic ILDs. They can help slow down scarring, but cannot treat scarring that has already developed. The NHS says they are tablets or capsules taken several times a day, with regular blood tests to check how well they are working. Asthma + Lung UK lists very common side effects: for nintedanib feeling sick, vomiting, rash, diarrhoea and abdominal pain, and for pirfenidone diarrhoea, loss of appetite, headache, indigestion, nausea and a rash from sun sensitivity.

If fibrosis is caused by an inflammatory or autoimmune ILD, a specialist team may prescribe immunosuppressant medicines such as prednisolone to calm the immune response and reduce inflammation.

Other support is just as important. The NHS says you may be offered oxygen therapy to improve breathlessness and help you be more active, and a local pulmonary rehabilitation course. In rare cases where the condition continues to get worse with treatment, a specialist may discuss a lung transplant. If there is nothing more that can slow progression, the NHS says you will be given supportive treatment known as end of life care.

Asthma + Lung UK says most people with pulmonary fibrosis have breathlessness. You may only notice it when you are active, such as climbing stairs, but some people feel breathless at rest. Other common symptoms are a dry cough that does not go away and is not relieved by over-the-counter remedies, feeling tired a lot or all the time, and losing weight without trying to.

The NHS says IPF symptoms usually start slowly but continue to get worse over time. A doctor may notice finger clubbing, blueish or grey lips from low blood oxygen, or crackling sounds when listening to your chest with a stethoscope.

Breathlessness has many causes. Asthma + Lung UK lists chronic obstructive pulmonary disease (COPD), heart disease, being overweight or becoming less fit as you get older. That is why it needs proper assessment.

Asthma + Lung UK lists several causes: breathing in dust from metal, stone and wood, breathing in certain types of dust or organic matter such as bird droppings, side effects of drugs or treatments including some antibiotics, anti-inflammatory medicines and chemotherapy, and autoimmune conditions such as rheumatoid arthritis.

Risk is higher if you are older (IPF mainly affects people over 50), if there is a family history of ILDs, particularly IPF, or if you smoke. For IPF the NHS says it is not known what causes it, but in some cases it has been linked to long-term exposure to dust such as metal, wood or asbestos, and you may be more at risk if someone in your close family has had it.

Asthma + Lung UK notes that often the cause is not known, which can be upsetting, and that more is being found out about causes all the time.

The NHS says that if a doctor thinks you could have IPF they will refer you to a specialist in hospital for tests. These include a spirometry test (a breathing test that checks how well the lungs work) and a chest X-ray, followed by a chest CT scan to look for scarring. If it is still unclear what is causing your symptoms you may be offered a bronchoscopy, where a thin flexible tube is passed into the lungs, or a lung biopsy, which is a type of surgery to remove small samples of lung tissue.

Asthma + Lung UK says that when you are first diagnosed, specialists may not be able to tell whether your condition is likely to be progressive or how quickly it might change. Regular reviews and lung function tests show how it is behaving over time, and working out the cause makes this easier to predict.

The terms are often confused. The table sets out the main distinctions made by the NHS and Asthma + Lung UK.

The NHS says there are things you can do to ease breathlessness. These are listed below, and Asthma + Lung UK also recommends breathing techniques and pulmonary rehabilitation.

• Stop smoking if you smoke.

• Exercise regularly and stay as fit as you can.

• Eat a healthy, balanced diet.

• Get the annual flu vaccine, pneumococcal vaccine and RSV vaccine if you are eligible, because these infections can be more serious if you have a lung condition.

• Try to keep away from people with chest infections and colds.

• Tell your specialist team about heartburn, post-nasal drip or chest infections, which can make a cough worse, and ask about managing a persistent cough.

• Charities such as Action for Pulmonary Fibrosis and the Pulmonary Fibrosis Trust offer information and support, as the NHS notes.

The NHS says IPF gets worse over time, but how quickly this happens varies from person to person. If treatment works well you may not have many symptoms for years. It can eventually lead to chest infections, pulmonary hypertension and heart failure, which can make you very tired and reduce your life expectancy.

Asthma + Lung UK says the outlook depends on what has caused the condition and whether it is progressive. Some types respond well to treatment and remain stable for many years, and even progressive types can have periods when little changes. A sudden worsening is called an exacerbation or flare up.

Asthma + Lung UK notes that figures of 3 to 5 years found online come from old research into IPF, and that newer treatments are improving outcomes for many people. Your specialist team can discuss how much you want to know and plan supportive care alongside treatment.

See a GP if you think you might have IPF, if breathlessness gets worse when doing normal activities or when you lie down, or if you have had a cough for 3 weeks or more, as the NHS advises.

If you already have pulmonary fibrosis, Asthma + Lung UK says to contact your GP or call 111 for urgent advice if you feel more breathless than usual, are coughing more or your cough has changed or got worse, find your usual activities more difficult, feel more tired than is normal for you, have symptoms such as blue or grey lips that are not normal for you, or notice different symptoms that are getting worse.

On the NHS a GP usually arranges initial tests and refers you to a hospital specialist, usually in respiratory medicine. Antifibrotic medicines, oxygen assessment and pulmonary rehabilitation are organised through the specialist team, and Asthma + Lung UK notes that a pulmonary fibrosis patient passport can help you check the basic care you should receive.

Some people choose to see a private respiratory specialist for an opinion or tests such as a CT scan. In general terms the same steps apply, and ongoing medicines, monitoring blood tests and review are usually coordinated with an NHS ILD service, so it is worth asking how follow-up will be shared.