Idiopathic pulmonary fibrosis (IPF): breathlessness, diagnosis and treatment in later life

Asthma + Lung UK explains that pulmonary fibrosis is scarring in the lungs, part of a larger group of lung diseases called interstitial lung diseases. It affects the tissue in and around the tiny air sacs, making it harder for the lungs to expand and for oxygen to pass into the blood, which can lead to low blood oxygen levels. Idiopathic means there is no known cause, and IPF is a progressive type; other fibrotic lung diseases, such as hypersensitivity pneumonitis or sarcoidosis, can behave differently.

The NHS says it is not known what causes IPF, though it has been linked in some cases to long-term exposure to certain dusts such as metal, wood or asbestos, and risk may be higher if a close family member has had it. Asthma + Lung UK adds that smoking raises risk, and that some drug side effects (including some antibiotics, anti-inflammatory medicines and chemotherapy) and autoimmune conditions such as rheumatoid arthritis can cause fibrosis, so a medicines and occupational history is part of the work-up.

The NHS lists shortness of breath, a dry cough that does not go away, feeling tired and finger clubbing, with symptoms usually starting slowly and getting worse. Asthma + Lung UK adds breathlessness that may be noticed only on exertion such as climbing stairs, though some people are breathless at rest, a cough not relieved by over-the-counter remedies, constant tiredness and unintentional weight loss. A GP may notice clubbing, blue or grey lips, or crackling sounds on listening to the chest.

The charity points out that breathlessness may be caused by other conditions such as COPD, heart disease, being overweight or becoming less fit as you get older. That overlap is why IPF is often diagnosed late in older people. The NHS says to see a GP if breathlessness gets worse when doing normal activities or when lying down, or if you have had a cough for 3 weeks or more, and that you are not wasting anyone's time by getting it checked.

According to the NHS, a doctor who suspects IPF refers you to a hospital specialist for tests, including spirometry to check how well your lungs work and a chest X-ray followed by a chest CT scan to look for scarring. If it is still unclear, you may be offered a bronchoscopy (a thin flexible tube passed into the lungs) or a lung biopsy (small samples of lung tissue taken during surgery).

Asthma + Lung UK says that at first diagnosis specialists may not be able to tell whether the condition will be progressive or how quickly it may change, and that regular reviews and lung function tests help show how it is changing over time.

The NHS says two medicines, pirfenidone (Esbriet) and nintedanib (Ofev), taken as tablets or capsules several times a day, may be prescribed to some people to help slow scarring, with regular blood tests to check how well they are working. Whether they are suitable depends on assessment by the specialist team, and the sources read here do not set out side effects or eligibility, so discuss these with your specialist.

Other support described by the NHS includes oxygen therapy (home oxygen, a portable device or both) to improve breathlessness and help people stay active, and local pulmonary rehabilitation, a course of exercise and education. If treatment does not work, a lung transplant may rarely be recommended, and if doctors think nothing more can slow progression you are offered supportive end of life care. The NHS advice for easing breathlessness includes stopping smoking, regular exercise, a balanced diet, the annual flu vaccine and pneumococcal and RSV vaccines if eligible, and keeping away from people with chest infections and colds.

The NHS says IPF gets worse over time but how quickly varies, and that if treatment works well you may not have many symptoms for years. It can eventually lead to chest infections, pulmonary hypertension and heart failure. Asthma + Lung UK says pulmonary fibrosis that has been steady can sometimes get worse suddenly, which is called an exacerbation or flare-up, and advises seeking urgent advice via the GP or NHS 111 if you feel more breathless than usual, are coughing more or your cough changes, find usual activities harder, feel more tired than normal, or have blue or grey lips.

On life expectancy, Asthma + Lung UK stresses that it depends on factors including how quickly the condition was diagnosed, whether you are on antifibrotic treatment, how you respond, lung function results, age, general health and fitness, and smoking. It notes that figures of 3 to 5 years quoted online are old, and that your specialist team can talk about it only to the extent you want. Planning ahead, including your wishes for care, is something the team can support.

The NHS points to Action for Pulmonary Fibrosis, and Asthma + Lung UK to the Pulmonary Fibrosis Trust and its own helpline staffed by respiratory nurse specialists. Action for Pulmonary Fibrosis offers a support line and a befriending service. Carers and families can use the same services, which can help older couples cope with the practical and emotional load.