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What is it about ?
The lungs, thanks to the alveoli, take oxygen from the air to supply it to the body. Pulmonary fibrosis is a rare disease in which scar tissue (also called connective tissue or fibrosis) develops in the alveoli. Scar tissue usually appears after an inflammatory reaction. Scar tissue is less flexible and contains fewer blood vessels than the tissue it replaces. As a result, the alveoli function less well and take less oxygen.
The known causes of pulmonary fibrosis are the inhalation of toxic substances, the use of certain drugs, certain diseases. When it is not known why connective tissue has formed, it is called idiopathic pulmonary fibrosis or pulmonary fibrosis of unknown origin. This form of pulmonary fibrosis is the most common.
Idiopathic pulmonary fibrosis is a chronic disease. The average survival after diagnosis is 3 to 5 years depending on the studies. The course of the disease can vary greatly from person to person. In some people, it may progress slowly or remain stable for several years. In others, flare-ups can occur suddenly and lead to respiratory problems and rapid degradation.
What is its frequency?
It is estimated that 7 to 20 people in 100,000 worldwide suffer from idiopathic pulmonary fibrosis.
How to recognize it?
Initially, the symptoms of pulmonary fibrosis are vague, like a dry cough. Then fromshortness of breath and an tired appear quickly. As the disease progresses and connective tissue in the lungs increases, daily activities become more difficult due to shortness of breath (dyspnea). Sometimes the fingertips get thicker (fingers in “drum sticks”) and the nails become shiny and convex (“watch glass” nails).
Flare-ups of pulmonary fibrosis are manifested by sudden deterioration over a period of 30 days, with a gradual increase in shortness of breath.
How is the diagnosis made?
At first, the clinical examination and additional examinations may be normal. Depending on the progress of the disease, the doctor hears dry crackling rales on auscultation of the lungs. With advanced disease, the level of oxygen in the blood decreases. This rate is measured using an oximeter placed at the fingertip.
If pulmonary fibrosis is suspected, several examinations are carried out. The most important are a CT scan and a lung function test. A regular x-ray of the lungs may be normal at first. At a later stage, we can see thick lines. A scan of the lungs gives a detailed picture of the lungs and allows you to see typical abnormalities. THE’lung function test allows to measure the contents of the lungs and to distinguish different lung diseases.
Idiopathic pulmonary fibrosis does not cause abnormalities in blood tests. Some cases require a lung biopsy (the doctor takes a piece of the lung). A specialist doctor (pathologist) examines the biopsy sample under a microscope.
Before making the diagnosis of idiopathic pulmonary fibrosis, all other causes of scar tissue must have been ruled out. The diagnosis of pulmonary fibrosis is usually made in a specialized center where pulmonologists, radiologists and pathologists work together.
The doctor will also look at whether there is a heart problem or other lung problems (such as infection) that could explain the symptoms.
What can you do ?
- Stop smoking immediately and completely.
- Avoid dusty environments and strenuous efforts.
- When it is very hot, it is best to stay indoors.
- Maintain some physical activity, such as walking, possibly with a physiotherapist. It is beneficial for your lung capacity.
What can the doctor do?
Once the lung tissue is replaced with connective tissue, it is final. This is why respiratory rehabilitation is important. Patients then learn to treat the symptoms of pulmonary fibrosis through training and breathing exercises for better functioning in everyday life. Ultimately, home oxygen therapy may be necessary.
Specific drugs are available to slow the formation of connective tissue in the lungs and to control symptoms. But these drugs are very expensive, and sometimes they have serious side effects. The reimbursement conditions for these drugs are therefore very strict.
It is not possible to predict who will have flare-ups in pulmonary fibrosis and who will not. That is why, in anyone with pulmonary fibrosis, it is important to find out whether a lung transplant will be possible in the future.
Flare-ups during pulmonary fibrosis are treated with cortisone.
Want to know more?
- Stop smoking. It’s possible – Tabacstop
- Physical activity – Eat Move – Health question
- Move at any age… After 50 too! – Eat Move – Health Question
Are you looking for more specialized help?
- Smoking Assistance Center (CAF®) – FARES – Respiratory Affections Fund
- Tobacco specialists – FARES – Respiratory Affections Fund
Source
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