Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis is a serious lung disease where tissue surrounding the air sacs becomes thick, stiff, and scarred for reasons that remain unknown. This progressive condition makes breathing increasingly difficult over time, affecting mostly people in their 60s and 70s.
Table of contents
- What Is Idiopathic Pulmonary Fibrosis?
- Symptoms and Disease Progression
- Causes and Risk Factors
- Diagnosis
- Treatment Options
- Complications
- Living With Idiopathic Pulmonary Fibrosis
- Outlook and Prognosis
What Is Idiopathic Pulmonary Fibrosis?
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease that affects the tissue surrounding the air sacs, or alveoli (the tiny sacs in your lungs where oxygen enters your blood), in the lungs[1]. The condition develops when that lung tissue becomes thick and stiff for unknown reasons[1]. Over time, these changes cause permanent scarring in the lungs, called fibrosis (the buildup of thick, stiff scar tissue), that makes it progressively more difficult to breathe[1].
IPF is the most common type of idiopathic interstitial lung disease (a group of lung conditions affecting the tissue between the air sacs)[2]. The word “idiopathic” means the cause is unknown[2]. There are more than 200 types of interstitial lung diseases, which are characterized by varied amounts of inflammation, scarring, or both, that damage the ability of the lung to absorb oxygen from the air[2].
When lung tissue becomes scarred and thickened, the hard, stiff tissues do not expand as well as they should[6]. This makes it harder for the lungs to work properly and causes people to become short of breath during daily tasks that never seemed tiring before[3].
- Lungs
- Alveoli (air sacs)
- Lung tissue (interstitium)
Symptoms and Disease Progression
The symptoms of IPF tend to develop gradually and get slowly worse over time[4]. Many people ignore their breathlessness at first and blame it on getting old or being out of shape[4]. However, eventually even light activity such as getting dressed can cause shortness of breath[4].
The most common symptoms of IPF include[1][3]:
- Shortness of breath, especially during or soon after physical activity
- A persistent dry cough that does not go away
- Extreme tiredness and fatigue
- Weight loss that is not intended
- Aching muscles and joints
- Clubbing (widening and rounding of the tips of the fingers or toes)
Some people may not have symptoms at first, but symptoms can develop and get worse as the disease progresses[1]. The way that IPF progresses varies from person to person, and scarring may happen slowly or quickly[1]. In some people, the disease stays the same for years. In other people, the condition quickly gets worse[1].
Many people with IPF also experience what are known as acute exacerbations (sudden, severe worsening of symptoms), where symptoms suddenly become much more serious[1]. These episodes can be life threatening and are more common in advanced stages of IPF[19].
Causes and Risk Factors
In people with IPF, the tiny air sacs in the lungs become damaged and increasingly scarred[4]. The reason this happens is not clear. Idiopathic means the cause is unknown[4]. Experts think pulmonary fibrosis happens when lungs do not heal properly from damage or inflammation[6].
IPF has been linked to several factors[4][6]:
- 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 (gastroesophageal reflux disease)
- Smoking
- Environmental exposures to substances like asbestos, silica, and beryllium
- Certain medications or treatments, including some chemotherapy drugs and radiation therapy
However, it is not known whether some of these factors directly cause IPF[4].
Your risk for IPF is higher if you smoke or have a family history of IPF, and the risk increases with age[1]. IPF usually develops in people aged 70 and older, and it is more common in men[7]. It is rare in people under 50[4]. Those in their 60s and 70s are most commonly affected[8].
You might also be at a higher risk for pulmonary fibrosis if you are 65 or older, are male, or have certain conditions caused by changes in your DNA[6].
Diagnosis
It might take a number of tests and imaging procedures to diagnose pulmonary fibrosis[11]. If you have struggled with your breathing for a while or have had a cough for more than 3 weeks, you should see a doctor[4]. These symptoms are not normal and should not be ignored[4].
Your doctor will review your medical and family history and do a physical exam[11]. During the physical exam, your healthcare professional listens carefully to your lungs while you breathe[11]. Pulmonary fibrosis often occurs along with a crackling sound at the base of the lungs[11].
Tests and procedures that may be used to diagnose IPF include[4][11]:
- Breathing tests (also called lung function tests or pulmonary function tests) to understand how your lungs are working
- Blood tests, including an arterial blood gas test
- Chest X-ray to look for scar tissue
- High-resolution CT scan (a detailed imaging test that combines X-ray images) to diagnose pulmonary fibrosis and find out how much lung damage has occurred
- Lung biopsy, where a small piece of lung tissue is removed during keyhole surgery so it can be examined
- Bronchoscopy (a procedure using a thin tube with a camera to view inside the airways)
- Echocardiogram (an ultrasound of the heart) to diagnose or rule out issues with your heart
Sometimes a chest X-ray may not show any changes, and more tests may be needed to find out why you are short of breath[11]. Your provider will check for and rule out other conditions that affect your heart and lungs[6].
Treatment Options
There is currently no cure for IPF[1][4]. However, certain treatments may slow the progression of IPF and help your lungs work better[1]. This may extend the lifespan and improve the quality of life of people who have the disease[1]. Treatment focuses on relieving symptoms as much as possible and slowing down disease progression[10].
Self-Care and Lifestyle Changes
There are several things you can do to stay as healthy as possible if you have IPF[10][22]:
- Stopping smoking if you smoke
- Exercising regularly and staying as fit as you can
- Eating a healthy, balanced diet
- Getting the annual flu vaccine and one-off pneumococcal vaccine – these infections can be more serious if you have a lung condition
- Trying to keep away from people with chest infections and colds whenever possible
- Staying well hydrated by drinking plenty of water
Medications
There are two main medicines that can help slow down the progression of IPF in some people: pirfenidone and nintedanib[10][15]. These are called anti-fibrotic drugs (medications that slow down scarring in the lungs)[12][14].
Pirfenidone has been shown to help slow down the process of scarring in the lungs by reducing the activity of the immune system[10]. It is normally taken as capsules three times a day[10]. Side effects can include feeling sick, tiredness, diarrhea, indigestion, and a rash caused by exposure to sunlight[10].
Nintedanib is a medicine that can also help slow down scarring of the lungs in some people with IPF[10]. It is normally taken as capsules twice a day[10]. Side effects can include diarrhea, feeling and being sick, stomach pain, and loss of appetite and weight loss[10].
Both medications are approved to treat idiopathic pulmonary fibrosis and have been shown to slow the decline in lung function in clinical trials[12][14][15].
Oxygen Therapy
IPF can cause levels of oxygen in your blood to fall, which can make you feel more breathless[10]. If this happens, oxygen treatment can help with your breathing and allow you to be more active[10]. Oxygen is taken through nasal tubes or a mask attached to a small machine[10]. Portable oxygen devices that you can use while you are out and about are also available[10].
Pulmonary Rehabilitation
Pulmonary rehabilitation is used for many long-term lung conditions[10]. 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[10]. Courses in pulmonary rehabilitation are usually held locally and may involve education about pulmonary fibrosis, physical activity, breathing exercises, advice on nutrition, psychological support, and a social support network[10].
Lung Transplantation
If your condition continues to get worse despite treatment, your specialist may recommend a lung transplant[10][22]. Lung transplantation may be an option for some people[1]. A lung transplant can significantly improve life expectancy in people with IPF, although it is a major procedure that is not suitable for everyone[10]. Unilateral or bilateral lung transplantation is the only treatment for IPF shown to increase life expectancy[13].
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, and whether a donor lung is available[10].
Complications
Scarred lung tissues have a hard time getting oxygen to the rest of your body and can make your heart work harder[6]. Other complications of IPF include[1][6][8]:
- Pulmonary hypertension (high blood pressure in the lungs), which happens when the lungs cannot deliver enough oxygen into the bloodstream
- Respiratory failure, which happens when the lungs cannot deliver enough oxygen into the bloodstream without support
- Heart failure
- Pneumonia and other lung infections
- Pulmonary embolism (blood clots in the lungs)
- Lung cancer
- Collapsed lung
- Low oxygen in your blood and tissues
These complications prevent the brain and other organs from getting the oxygen they need[1].
Living With Idiopathic Pulmonary Fibrosis
If you have been diagnosed with IPF, it is important to continue your treatment plan[19]. Talk to your doctor about how often to schedule follow-up care and how to monitor and understand your condition so you know when to get medical help[19].
Living with chronic lung disease changes a person’s life and requires adjusting to a new way of being in the world[21]. You may have been physically active your entire life, and now that you have IPF, you may find that you cannot do things you once enjoyed[21]. You may feel slowed down and less spontaneous[21].
It is normal to feel angry, afraid, sad, depressed, guilty, stressed, and frustrated with all of the changes[21]. You will be better off if you allow yourself to feel all of these things, even when it is uncomfortable[21]. Using the support of others will help you feel stronger and less alone in dealing with the challenges of IPF[21].
Regular checkups with your doctor may be part of your follow-up and treatment[19]. Tell your doctor if you suddenly experience a worsening of symptoms over a period of days or weeks[19]. People who have IPF can have other conditions as well, so you may need to see several doctors who specialize in different areas of medical care[19].
People often benefit from staying as healthy as possible through lifestyle changes, pulmonary rehabilitation, and supplemental oxygen if needed[1][8]. Managing your condition is best done with the help of a healthcare team that can help improve your quality of life[19].
Outlook and Prognosis
IPF gets worse over time, although the speed at which this happens is highly variable[4]. 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[4].
It is very difficult to predict how long someone with IPF will survive at the time of diagnosis[4]. Regular monitoring over time can indicate whether it is getting worse quickly or slowly[4]. Average life expectancy following diagnosis is about four years[1][8]. Research that suggests an average life expectancy of between 3 to 5 years was carried out before treatments that can slow down the rate of lung scarring were widely available[7].
The progression of IPF is different for each person and cannot be predicted[19]. Pulmonary fibrosis usually gets worse over time, but how quickly it gets worse is different for everyone[6]. There are many factors that will affect life expectancy, such as the cause of pulmonary fibrosis, a person’s age, and any underlying health conditions[7].
Clearly, there is an unmet need for accelerated research into IPF mechanisms so that progress can be made in therapeutics toward the goals of increasing life expectancy, alleviating symptoms, and improving well-being[13].
