Идиопатический легочный фиброз
Идиопатический легочный фиброз (ИЛФ) — это состояние, при котором легкие рубцуются, и дыхание становится все более затрудненным.
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- Symptoms of idiopathic pulmonary fibrosis
- When to get medical advice
- Causes of idiopathic pulmonary fibrosis
- Treatments for idiopathic pulmonary fibrosis
- Outlook for idiopathic pulmonary fibrosis
- National Congenital Anomaly and Rare Disease Registration Service
- Diagnosis Idiopathic pulmonary fibrosis
- Medical history and examination
- Breathing and blood tests
- Chest X-ray and CT scan
- Bronchoscopy
- Lung biopsy
- Treatment Idiopathic pulmonary fibrosis
- Self care
- Medicines
- Oxygen support
- Pulmonary rehabilitation
- Lung transplant
- Palliative care
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Форма выпуска и дозировка: КАПСУЛА, 4 мгДействующее вещество: ПомалидомидПроизводитель лекарства: Krka D.D. Novo MestoОтпускается по рецептуФорма выпуска и дозировка: КАПСУЛА, 2 мгДействующее вещество: ПомалидомидПроизводитель лекарства: Sandoz Farmaceutica S.A.Отпускается по рецептуФорма выпуска и дозировка: КАПСУЛА, 4 мгДействующее вещество: ПомалидомидПроизводитель лекарства: Laboratorios Basi Industria Farmaceutica S.A.Отпускается по рецепту
Idiopathic pulmonary fibrosis (IPF) is a condition in which the lungs become scarred and breathing becomes increasingly difficult.
It's not clear what causes it, but it usually affects people who are around 70 to 75 years old, and is rare in people under 50.
Several treatments can help reduce the rate at which IPF gets worse, but there's currently no treatment that can stop or reverse the scarring of the lungs.
Symptoms of idiopathic pulmonary fibrosis
The symptoms of IPF tend to develop gradually and get slowly worse over time.
Symptoms can include:
- shortness of breath
- a persistent dry cough
- tiredness
- loss of appetite and weight loss
- rounded and swollen fingertips (clubbed fingers)
Many people ignore their breathlessness at first and blame it on getting old or being out of shape.
But eventually even light activity such as getting dressed can cause shortness of breath.
When to get medical advice
See a GP if you have struggled with your breathing for a while or have had a cough for more than 3 weeks.
These symptoms are not normal and should not be ignored.
If a GP thinks you could have a lung condition such as IPF, they can refer you to a hospital specialist for tests such as:
- breathing (lung function) tests
- blood tests
- a chest X-ray and CT scan
- a lung biopsy, where a small piece of lung tissue is removed during keyhole surgery so it can be analysed
Causes of idiopathic pulmonary fibrosis
In people with IPF, the tiny air sacs in the lungs (alveoli) become damaged and increasingly scarred.
This causes the lungs to become stiff and makes it difficult for oxygen to get into the blood.
The reason this happens is not clear. Idiopathic means the cause is unknown.
IPF has been linked to:
- exposure to certain types of dust, such as metal or wood dust
- viral infections
- a family history of IPF – around 1 in 20 people with IPF has another family member with the condition
- acid reflux
- smoking
But it's not known whether some of these factors directly cause IPF.
Treatments for idiopathic pulmonary fibrosis
There's currently no cure for IPF, but there are several treatments that can help relieve the symptoms and slow down its progression.
Treatments include:
- self care measures, such as stopping smoking, eating healthily and exercising regularly
- medicines to reduce the rate at which scarring worsens, such as pirfenidone and nintedanib
- breathing oxygen through a mask – you can do this while you're at home or while you're out and about
- exercises and advice to help you breathe more easily (pulmonary rehabilitation)
- a lung transplant – this is suitable in a few cases, although donor lungs are rare
Outlook for idiopathic pulmonary fibrosis
IPF gets worse over time, although the speed at which this happens is highly variable.
Some people respond well to treatment and remain relatively free of symptoms for many years, while others may get rapidly worse or find the breathlessness debilitating.
Other problems can also sometimes develop, including chest infections, pulmonary hypertension and heart failure.
It's very difficult to predict how long someone with IPF will survive at the time of diagnosis.
Regular monitoring over time can indicate whether it's getting worse quickly or slowly.
National Congenital Anomaly and Rare Disease Registration Service
The NCARDRS help scientists look for better ways to prevent and treat IPF. You can opt out of the register at any time.
Diagnosis Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis (IPF) can be hard to diagnose because its symptoms are similar to other lung conditions, such as chronic obstructive pulmonary disease (COPD).
A GP can refer you to hospital specialists for a number of tests to help rule out other conditions and confirm the diagnosis.
Medical history and examination
Your doctor will ask about your medical history and whether there are other factors that could be causing a problem with your lungs, such as whether you:
- smoke or have smoked in the past
- have been exposed to harmful substances at work, such as asbestos
- have other medical conditions
They may also:
- check the sound of your breathing through a stethoscope – a crackling sound can suggest lung scarring (fibrosis)
- look at your fingers to see if the ends are swollen (finger clubbing)
- ask you to walk around for a few minutes to see if you become breathless
Breathing and blood tests
Lung function tests (also called pulmonary function tests) assess how well your lungs work and can help indicate what the problem may be.
These tests measure:
- how quickly you can move air in and out of your lungs
- how much air your lungs can hold
- how well your lungs transfer oxygen into your blood and remove carbon dioxide from it (this can be checked using a blood test)
A commonly used lung function test is spirometry. During the test, you breathe into a mouthpiece that's attached to a monitor.
Chest X-ray and CT scan
A chest X-ray does not show the lungs in much detail, but can help doctors spot some more obvious problems that could be causing your symptoms, such as cancer or a build-up of fluid.
If IPF is suspected, the chest X-ray will be followed by a CT scan.
A CT scan is similar to an X-ray, but lots more images are taken and these are put together by a computer to create a more detailed image of your lungs.
This can help your doctor spot signs of scarring in your lungs.
Bronchoscopy
If doctors are still not sure what the problem is after these tests, they may suggest having a bronchoscopy.
This is a test where a narrow, flexible tube with a camera (bronchoscope) is passed down into your airways.
Your doctor will look for anything abnormal and may take small tissue samples for testing.
You'll usually be awake during a bronchoscopy and it may cause coughing.
Local anaesthetic will be used to numb your throat so it does not hurt, and you may also be given a sedative injection that will make you feel sleepy during the procedure.
Lung biopsy
If other tests are not conclusive, a lung biopsy may need to be carried out.
This involves keyhole surgery to remove a small sample of lung tissue so it can be analysed for signs of scarring.
This is performed under a general anaesthetic, where you're asleep.
Your surgeon makes several small cuts in your side and an endoscope, a thin tube with a camera and a light at the end, is inserted into the area between the lungs and the chest wall.
The surgeon can see the lung tissue through the endoscope and is able to obtain a small sample.
Treatment Idiopathic pulmonary fibrosis
There's currently no cure for idiopathic pulmonary fibrosis (IPF). The main aim of treatment is to relieve the symptoms as much as possible and slow down its progression.
As the condition becomes more advanced, end of life (palliative) care will be offered.
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Self care
There are several things you can do to stay as healthy as possible if you have IPF.
These include:
- stopping smoking if you smoke
- exercising regularly and staying as fit as you can
- eating a healthy, balanced diet
- making sure you get the annual flu vaccine and one-off pneumococcal vaccine – these infections can be more serious if you have a lung condition
- try to keep away from people with chest infections and colds whenever possible
Medicines
There are 2 medicines that can help slow down the progression of IPF in some people: pirfenidone and nintedanib.
Pirfenidone
Pirfenidone has been shown to help slow down the process of scarring in the lungs by reducing the activity of the immune system.
It's normally taken as capsules 3 times a day.
It's recommended if breathing tests have shown your lung capacity is 50% to 80% of what would normally be expected.
If your condition gets worse despite taking pirfenidone and your lung capacity falls by 10% or more within a year, the medicine will usually be stopped.
Side effects of pirfenidone can include:
- feeling sick
- tiredness
- diarrhoea
- indigestion
- a rash caused by exposure to sunlight
There have been reports of pirfenidone causing serious liver damage. While this risk is thought to be low, serious liver damage can be fatal.
If you’re prescribed pirfenidone, you’ll have blood tests to check your liver function before starting treatment and at regular intervals during treatment.
Symptoms of liver damage include:
- yellowing of the eyes or skin
- loss of appetite
- itchy skin
- your pee turns a darker colour
- feeling tired
- pain in the upper right side of your stomach
If you have any of these symptoms, then stop taking your medicine and talk to your doctor immediately. If this is not possible call 112 for advice.
Nintedanib
Nintedanib is a newer medicine that can also help slow down scarring of the lungs in some people with IPF.
It's normally taken as capsules twice a day.
Like pirfenidone, it may be used if you have a lung capacity 50% to 80% of what would normally be expected, and should be stopped if your lung capacity falls by 10% or more in a year while taking it.
Side effects of nintedanib can include:
- diarrhoea
- feeling and being sick
- tummy (abdominal) pain
- loss of appetite and weight loss
Oxygen support
IPF can cause levels of oxygen in your blood to fall, which can make you feel more breathless.
If this happens, oxygen treatment can help with your breathing and allow you to be more active.
Oxygen is taken through nasal tubes or a mask attached to a small machine. This device provides a much higher level of oxygen than the air.
The tubes from the machine are long, so you'll be able to move around your home while connected.
Portable oxygen devices that you can use while you're out and about are also available.
Pulmonary rehabilitation
Pulmonary rehabilitation is used for many long-term lung conditions.
It aims to help people come to terms with their condition, learn the best ways to deal with it and improve their ability to function on a daily basis without severe breathlessness.
Courses in pulmonary rehabilitation are usually held locally and may involve:
- education about pulmonary fibrosis
- physical activity
- breathing exercises
- advice on nutrition
- psychological support
- a social support network
Lung transplant
If your condition continues to get worse despite treatment, your specialist may recommend a lung transplant.
The decision to have a transplant will be based on:
- how bad your condition is
- how quickly your condition is getting worse
- your age and general health
- how much your condition is likely to improve after a transplant
- whether a donor lung is available
A lung transplant can significantly improve life expectancy in people with IPF, although it's a major procedure that puts a great strain on the body.
Few people with idiopathic pulmonary fibrosis are suitable candidates for a transplant, and donor lungs are scarce.
Palliative care
If you're told there's nothing more that can be done to treat you, or you decide not to have treatment, your GP or care team will give you support and treatment to relieve your symptoms.
This is called palliative care.
You can choose to have palliative care:
- at home
- in a care home
- in hospital
- in a hospice
Your doctor or care team should work with you to establish a clear plan based on your wishes.
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