Idiopathic pulmonary fibrosis
The lung works like a sponge made of hundreds of millions of tiny air sacs called alveoli.
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Medicines commonly prescribed for Idiopathic pulmonary fibrosis
For informational purposes only. Always consult a doctor before using any medicine.
Dosage form: CAPSULE, 3 mgActive substance: pomalidomideManufacturer: Krka D.D. Novo MestoPrescription requiredDosage form: CAPSULE, 4 mgActive substance: pomalidomideManufacturer: Krka D.D. Novo MestoPrescription requiredDosage form: CAPSULE, 4 mgActive substance: pomalidomideManufacturer: Laboratorio Reig Jofre, S.A.Prescription required
The lung works like a sponge made of hundreds of millions of tiny air sacs called alveoli. Their walls separate air from blood by no more than a couple of layers of cells, so oxygen crosses them almost without resistance. In pulmonary fibrosis dense scar tissue is laid down in that wall: the partition thickens, the sponge stops expanding, every breath takes effort, and less and less oxygen reaches the blood. The word "idiopathic" means that no cause for the scarring has been found, and the diagnosis is reached last of all, once every known cause has been checked and ruled out. It appears mainly in the second half of life, usually after sixty; it is uncommon in younger people and affects more men than women.
What people notice first
The onset is almost always quiet, and a year or two often passes between the first symptoms and the diagnosis. That is wasted time: the earlier follow-up begins, the more options remain open.
- Breathlessness on exertion. At first only on hills and stairs; then the threshold drops and getting dressed, walking to the shop or taking a shower becomes hard work.
- A dry cough. Stubborn, in bouts, for months, bringing nothing up. Ordinary cough remedies barely touch it, and it is one of the most wearing parts of the illness.
- Tiredness that rest does not fix.
- Loss of weight and appetite without dieting and without trying.
- Changed fingers. The tips become rounded and swollen and the nails curve like a watch glass. This happens in roughly half of those affected.
There is one sign a doctor can find before anything shows on a plain chest X-ray: listening over the lower parts of the lungs picks up a fine dry crackling on breathing in, very like the sound of Velcro being pulled apart. It does not clear with coughing and is there day after day. That alone is reason enough to arrange a CT scan.
Breathlessness is far too easily blamed on age, extra weight or being out of practice. But if it has been building for months and has nothing to do with a cold, "just unfit" is not an adequate explanation.
When waiting is not an option
Fibrosis does not always progress smoothly. Some people have flare-ups: breathing deteriorates sharply over days or weeks with no obvious trigger. This is a dangerous situation and is treated in hospital.
Call an ambulance (in Spain, Italy, Portugal, Poland and Ukraine the single European number 112 answers) if:
- breathlessness has worsened sharply within days, or has appeared at rest;
- there is not enough breath to finish a sentence;
- the lips, fingertips or face have turned blue;
- confusion, heavy drowsiness or loss of consciousness set in;
- sudden chest pain comes on together with breathlessness — this is how a torn lung leaking air into the chest and a clot in the lung artery announce themselves, and both are commoner than usual in fibrosis;
- a noticeable amount of blood is coughed up.
Contact a doctor the same day if a temperature develops, the sputum turns purulent, the cough worsens, or everyday activity has suddenly become distinctly harder than it was a week ago. On scarred lungs an ordinary infection runs a heavier course, and it is not something to sit on.
If breathlessness or a cough have lasted more than three weeks with no explanation, book an appointment rather than waiting for things to get worse.
Why this diagnosis is left until last
Lung scarring is not one disease but the end point of many different processes, and some of them can be treated. From the outside they are indistinguishable from idiopathic fibrosis, which is why the questions about home and work are not a formality but the most useful part of the consultation.
- Breathing in organic dust. Pet birds, pigeon lofts, feather pillows and duvets, mould in a damp home, humidifiers and hot tubs with standing water, hay and grain. All of these cause hypersensitivity pneumonitis, which in time also leaves scarring — but which stops once the source is removed. This is a particularly painful thing to get wrong.
- Occupational dust. Asbestos, silica, metal and wood dust. The whole working life counts, including a job from thirty years ago.
- Rheumatic disease. Rheumatoid arthritis, scleroderma, muscle inflammation, Sjögren's syndrome. The lungs are sometimes affected before joint pain or skin changes appear, which is why blood is tested for the relevant markers.
- Medicines and radiation. Some drugs for irregular heart rhythm, certain cancer and antimicrobial drugs and several medicines for rheumatic disease can damage the lungs; radiotherapy to the chest also leaves its mark. Bring a complete list of what you take and have taken.
- Family predisposition. Around one in twenty of those affected also has a blood relative with fibrosis.
- Smoking and reflux of stomach contents. Both clearly raise the risk, although neither on its own explains the disease.
How the diagnosis is worked out
The main test is a high-resolution CT scan: it shows fine net-like markings and characteristic honeycomb spaces in the lower and outer parts of the lungs. When the picture is typical, the diagnosis is made from it and no surgery is needed.
Next comes how much work the lungs can still do. Spirometry records volumes and the speed of the breath out, and a separate test measures how well oxygen is transferred into the blood. The six-minute walk is done with a probe on the finger: the distance covered is noted along with any fall in oxygen saturation. These measurements are repeated every few months, and their trend shows whether the disease is moving fast or slowly.
Blood tests are there so that rheumatic disease is not missed. Sometimes a bronchoscopy is done: a flexible tube with a camera is passed into the airways and a small area is washed out so the fluid can be examined, which helps separate out other processes. If doubt remains, a biopsy is discussed — a small piece of tissue is taken through a small cut in the chest wall under general anaesthetic, or through the bronchoscope. The conclusion is not signed off by one doctor but by a joint meeting of respiratory physician, radiologist and pathologist, and in borderline cases that changes the diagnosis.
What treatment can and cannot do
Start with the honest part: scar tissue that has already formed does not dissolve, and the lungs cannot be returned to their earlier state. The aim is to slow the process down, ease symptoms and preserve strength.
- Antifibrotic medicines. There are two, and both are taken long term. They roughly halve the rate at which breathing measurements decline, but they do not make anyone feel better straight away: these are drugs for the future, not for today. Side effects are common — nausea, loose stools, loss of weight and appetite. One of them makes the skin sensitive to sunlight (covering clothes and sun cream are needed) and calls for regular liver blood tests. The other causes more diarrhoea and raises the risk of bleeding, so tell the doctor if you take anything that thins the blood.
- A warning sign while on these drugs: yellowing of the skin or the whites of the eyes, dark urine, itching, pain under the right ribs, a sudden loss of appetite. Stop the medicine and contact a doctor the same day.
- Oxygen. When saturation falls, it is prescribed at home through nasal prongs from a concentrator; portable units are available for going out. Oxygen does not treat the scarring, but it gives back the ability to move about and takes strain off the heart.
- Pulmonary rehabilitation. A course of graded exercise with breathing work, education and support. Of everything listed here, this is what improves exercise tolerance soonest.
- Lung transplant. The only thing capable of extending life substantially. It suits a minority and depends on age, other illnesses and donor availability, and the wait is long — which is why the question is raised early, not once someone is already very unwell.
- Everything else that gets in the way of breathing. Reflux of stomach contents is treated, remedies are found for an exhausting cough, the heart is checked, and pauses in breathing during sleep and raised pressure in the lung artery are looked for.
It is worth saying what should not be done. Steroids combined with drugs that damp down the immune system used to be prescribed for this condition. Trials showed that in idiopathic fibrosis this combination does not help and can cause harm; if a long course of steroids is proposed for the fibrosis itself, ask what the decision rests on.
What is in your own hands
- Stopping smoking. It is the one measure that acts on the disease and on the risk of lung cancer at the same time.
- Vaccination. Against flu every year, and against pneumococcus and other respiratory infections according to the schedule used in your country. On scarred lungs, a routine infection turns serious.
- Removing the source if one has been found. Rehoming the birds, replacing feather pillows, dealing with damp and mould. This works completely or not at all.
- Keeping moving. Regular exercise within your limits preserves muscle, and how far you can walk depends on it.
- Watching your weight. In this illness weight comes off by itself, and losing it makes things worse. Meals need enough calories and protein; if appetite has gone, say so.
- Asking about travel. The air is thin on aircraft and in the mountains, and some people need oxygen in flight. That has to be arranged in advance.
How the illness goes on
The course varies enormously and is nearly impossible to predict on the day of diagnosis. In some people the picture changes slowly over years, in others it worsens within months, and in others still a sudden flare interrupts a stable spell. The answer comes not from a prognosis but from follow-up: repeating the breathing tests every few months shows the pace far more reliably than any average figure.
Over time, raised pressure in the lung artery and strain on the right side of the heart may join in, and infections come round more often. There is also something that rarely gets said: pulmonary fibrosis raises the risk of lung cancer, particularly in those who have smoked — one more reason not to skip reviews and to report any new symptom.
Palliative care is not the same as giving up on treatment, and it is not the last week of life. It means working on breathlessness, cough, anxiety, weakness and the practical difficulties of daily life, and it makes sense alongside the main treatment. That is also the time to talk calmly with family and doctor about what you would and would not want if things deteriorate, while the conversation does not have to be rushed.
Online consultation
In an online consultation the doctor will go through in detail how your breathing has changed: what level of effort first brought on the breathlessness, what you can manage now compared with a year ago, how long the dry cough has been there, whether weight is falling. They will ask separately about the things usually left out — birds and damp at home, work with dust or asbestos, every medicine you take, joint and skin disease in you and your relatives. They will help you make sense of a scan and breathing tests you have already had, say what is missing from the work-up, and explain which specialist to see and how urgently. If the diagnosis is already made, they will review how well you tolerate the drugs, whether you need oxygen, rehabilitation, and the signs that mean contacting a doctor at once.
This material is for information only and does not replace medical advice.
Consult with a doctor about Idiopathic pulmonary fibrosis
Discuss your symptoms and possible next steps with a doctor online.
Online doctors for Idiopathic pulmonary fibrosis
Discuss your symptoms and possible next steps for Idiopathic pulmonary fibrosis with a doctor online.
