What is Pulmonary Fibrosis?
Pulmonary fibrosis is part of a family of conditions known as Interstitial Lung Diseases (ILDs) because they affect the ‘interstitium’ – the network of tissues that support the air sacs in the lungs. The disease affects around 70,000 people in the UK.
In normal lungs oxygen is breathed in and is transferred into the blood by delicate gas-exchanging air sacs called alveoli. The oxygenated blood is then pumped around the body by the heart.
Pulmonary fibrosis occurs when collagen – scar tissue which is also known as fibrosis - accumulates within these air sacs and impairs how well they can transfer oxygen into the blood. This eventually leads to low oxygen levels. PF is a progressive disease, which means that as the amount of scar tissue in the lungs increases, they become smaller and stiffer and the amount of oxygen they can hold also decreases. The rate at which this deterioration happens is unpredictable and varies from person to person.
Action for Pulmonary Fibrosis is the leading charity in Europe supporting people with pulmonary fibrosis.
Their website has a wealth of detailed and medically-approved information about the disease.
The charity also offers a wide variety of support for patients and carers including a Support Line, a carer support group and videos and webinars by the UK’s leading PF experts.
We would highly recommend that you take a good look at the website but here is some brief information about the symptoms, causes and diagnosis of PF.
Symptoms
Shortness of breath, which may occur only when you exercise initially but worsens over time.
Breathlessness can be an early or late PF symptom. People often write off shortness of breath, putting it down to lack of fitness and ageing. In the early stages of PF, you may be breathless when you do strenuous exercise or when walking up hills or stairs. As the disease progresses, you may notice breathlessness with milder activities such as showering or dressing.
Rounded and swollen finger tips.
Finger clubbing – the widening and rounding of the fingernails or toenails – is a less common pulmonary fibrosis symptom. At the moment we don’t know why some patients develop this and others don’t.
Persistent cough and ‘crackles’ in your lungs.
A persistent dry and tickly cough is a common symptom of pulmonary fibrosis. Sometimes the cough can be produce sticky sputum. Often patients visit their GP with a cough. On listening to the lungs, the GP hears dry or “Velcro”-type crackles that are a strong indicator of PF.
Other symptoms may include:
Tiredness or unintentional weight loss. Some people develop pulmonary hypertension, where there is increased blood pressure within the arteries of the lungs. You may have chest pains, a racing heartbeat and swelling of your ankles or legs. Your medical team is likely to carry out tests for pulmonary hypertension.
Causes
Idiopathic Pulmonary Fibrosis.
The most common form of pulmonary fibrosis is Idiopathic Pulmonary Fibrosis (IPF) which currently has no known cause – ‘idiopathic’ means unknown.
Known causes.
Doctors do understand that pulmonary fibrosis can be caused by hypersensitivity pneumonitis, asbestosis and by autoimmune diseases, such as rheumatoid arthritis and systemic sclerosis (scleroderma).
Occasionally, some medications can cause drug-induced pulmonary fibrosis. But it is not yet clear why some people develop these conditions, while others don't.
Diagnosis
Before making a diagnosis of lung fibrosis, your medical team will ask you questions about your breathing problems, examine you and arrange specific tests.
The tests and the clinical information taken together are reviewed in a multi-disciplinary team meeting to help the medical team understand your pulmonary fibrosis condition and recommend the most appropriate treatment.
What questions will I be asked?
You’ll be asked questions about your symptoms, your general health, jobs you have had - particularly where you may have been exposed to asbestos, dust from wood or stone, metals, birds or animals, your hobbies and pets, smoking and your family history of lung disease.
Will I have to undergo tests?
Pulmonary fibrosis diagnosis involves a number of tests. You may be asked to do some or all of these, over time:
Blood tests to assess for autoimmune diseases
In a lung function test, you will breathe into a tube so that your medical team can see how well your lungs work. The tests will identify your lung capacity (how much air your lungs can hold) and how well your lungs transfer oxygen to and from your blood. This will help to inform your treatment plan.
Six-minute walking test to assess if your blood oxygen level falls when you walk. This is a simple procedure that will give your doctor accurate information about your blood oxygen levels when you exercise. You will have a probe clipped to your finger to measure oxygen levels and be asked to walk for six minutes along a flat area. It takes about half an hour to complete.
What about scans or X-rays?
A chest x-ray can indicate whether you have scarring in your lungs. Pulmonary fibrosis often starts in the lower part of your lungs.
A high-resolution CT scan will give a more detailed picture of the inside of your lungs. Typical features of pulmonary fibrosis include fine lines (called reticulations), a ‘ground glass’ appearance and honeycombing patterns.
An echocardiogram looks at how well your heart is working. Increased pressure on the right side of the heart is a sign of pulmonary hypertension – which is a complication of pulmonary fibrosis.
Are there any other tests or procedures I may need?
A bronchoscopy: this involves a small camera that looks at the airways inside your lungs and takes samples from the lung tissues (bronchial lavage). The camera is passed either through the nose or mouth into your lungs. You will have a local anaesthetic and will not usually have to spend the night at hospital.
You may also have a bronchial lavage as part of the bronchoscopy, in which small amounts of salty water are passed into the lungs and sucked back out, sampling cells from the lungs that can be analysed to inform your diagnosis.
A surgical lung biopsy is sometimes necessary when other tests haven’t provided enough information for a certain diagnosis. This is usually performed by keyhole approach called video-assisted thoracoscopic surgery (VATS). You will have a general anaesthetic and have to spend a few days in hospital.