Is ipf terminal?
It's not clear what causes it, but it usually affects people around 70-75 years of age 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.
The symptoms of IPF tend to develop gradually and get slowly worse over time.
Symptoms can include:
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.
See your GP if you've struggled with your breathing for a while or have had a cough for more than three weeks.
These symptoms aren't normal and shouldn't be ignored.
If your GP thinks you could have a lung condition such as IPF, they can refer you to a hospital specialist for tests such as:
Read more about the tests for 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 means it's difficult for oxygen to get into the blood.
The reason this happens isn't clear. Idiopathic means the cause is unknown.
IPF has been linked to:
But it's not known whether some of these factors directly cause IPF.
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:
Read more about how idiopathic pulmonary fibrosis is treated.
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.
Chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF) are chronic, disabling lung diseases that cause many of the same symptoms. But IPF and COPD are not the same and cause different types of damage to the lungs. COPD is associated with pulmonary inflammation and IPF with lung scarring.
Please continue reading to learn more about the clinical features of chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis. Find out in what ways these progressive diseases are similar and how they differ. Also learn why early treatment is essential for both COPD and IPF.
Chronic obstructive pulmonary disease (COPD) is an inflammatory condition of the lungs that causes airflow obstruction, making it difficult to breathe. Other symptoms of COPD include wheezing, cough, and mucus. COPD is frequently caused by long-term exposure to cigarette smoke or environmental toxins. People with chronic obstructive lung disease are at increased risk of developing lung cancer, cardiovascular diseases, and other serious health conditions.
There are two lung conditions that commonly contribute to COPD - emphysema and chronic bronchitis. Emphysema involves the alveoli (air sacs) in the lungs, which are destroyed by prolonged exposure to cigarette smoke, particulate matter, or irritating gases. Chronic bronchitis is an inflammation of the lining of the air passages. Both emphysema and chronic bronchitis can be present in people with COPD.
Chronic obstructive pulmonary disease is a progressive disease that gets worse over time. However, it is treatable. Proper management can allow most people with this lung disease to achieve control over their symptoms and have a good quality of life.
Idiopathic pulmonary fibrosis is a condition that involves lung scarring, which makes breathing difficult. Other symptoms of IPF can include persistent cough, fatigue, loss of appetite, weight loss, and clubbed fingers (swollen and rounded tips).
Idiopathic pulmonary fibrosis sometimes goes undetected because people put down their shortness of breath to old age or being out of shape. However, as the condition progresses, even light activities such as getting dressed can cause shortness of breath.
The exact cause of IPF is unclear, but it mostly affects people over the age of 70. IPF is rare in people under 50 years old. There are no treatments to stop or reverse the lung scarring. However, proper management of idiopathic pulmonary fibrosis can reduce how fast the disease gets worse.
Idiopathic pulmonary fibrosis and chronic obstructive pulmonary diseases are similar in some ways, but they are not the same. The similarities between IPF and COPD are that they are both chronic progressive lung diseases that worsen over time. Both conditions can cause similar symptoms, such as breathing difficulties. The key differences between the two are described in the paragraphs below.
Idiopathic pulmonary fibrosis
IPF is classified as interstitial lung disease, affecting the lung tissue between other structures like blood vessels, air sacs, air passages, etc. In people with pulmonary fibrosis and other interstitial lung diseases, the cells between lung structures are damaged.
Chronic obstructive pulmonary disease
COPD is a more common type of lung damage. It can consist of emphysema, chronic bronchitis, or both. In people with COPD, the alveoli (air sacs) are damaged or destroyed. Also, there is pulmonary inflammation (the airways may be irritated and swollen).
IPF is a rare disease affecting an estimated 100,000 people in the United States. Around 30,000 to 40,000 new cases are diagnosed each year. Unfortunately, the condition is often not diagnosed until permanent scar tissue has formed in the lungs.
COPD is much more common. It affects approximately 16 million Americans, which could be an underestimation. The American Lung Association believes up to 24 million adults in the U.S. could be living with impaired lung function due to COPD. Many people with COPD have a mild form of the disease and are not diagnosed until the condition has progressed. The CDC lists COPD as a leading cause of death in the United States.
It is unknown why some people develop pulmonary fibrosis. The disease can have an unpredictable course.
In contrast, the cause of COPD is well known. Cigarette smoking is implicated in about 90% of cases. In addition, the course of COPD has been well studied.
Risk factors for idiopathic pulmonary fibrosis include age over 60 years; gender (men are diagnosed more often with IPF than women); family history of IPF; occupational exposure to dust, fumes, or chemicals; smoking; radiation therapy to the chest, for example, to treat lung cancer; and certain chemotherapy medications, antibiotics, and heart medications.
Risk factors for chronic obstructive lung disease include smoking, including exposure to secondhand smoke; age over 40; occupational exposure to dust, fumes, and chemicals, gender (women who don’t smoke are more likely to develop COPD than men who don’t smoke); and a family history of COPD.
As you can see, many risk factors are common for these two lung diseases.
Idiopathic pulmonary fibrosis is underdiagnosed. It is also frequently misdiagnosed because it shares many features with other lung diseases, including chronic obstructive pulmonary disease (COPD) and asthma. Many of the signs and symptoms of COPD and IPF are similar, including progressively worsening shortness of breath, chronic cough, tiredness, clubbed fingers, and weight loss. Both conditions are made worse by respiratory infections.
One difference between the two is the type of cough they cause. The cough in COPD is produced by sputum (mucus), while IPF produces a dry cough. People with COPD can develop additional symptoms such as cyanosis (blue fingertips and lips) due to low oxygen levels, chest tightness, and swollen ankles and feet.
In people with combined pulmonary fibrosis and emphysema syndrome, either COPD or IPF typically predominates. As a result, it is rare for an individual patient to be diagnosed with both diseases simultaneously. However, smokers can display changes in lung tissue consistent with both interstitial lung disease (IPF) and pulmonary emphysema (COPD).
Pulmonary function tests (measures of lung volumes) in such individuals are different from pure fibrosis or pure emphysema. However, traditional diagnostic tests are limited in identifying the simultaneous occurrence of both idiopathic pulmonary fibrosis and chronic obstructive pulmonary disease.
High-resolution computed tomography (CT) scan may provide enhanced recognition of the simultaneous occurrence of these lung diseases. The presence of concurrent chronic obstructive lung disease and idiopathic pulmonary fibrosis may also be discovered on radiographic examination, which might show interstitial lung abnormalities as well as upper lobe emphysema, for example. This can be correlated with clinical features such as significant smoking history, severe pulmonary hypertension, and shortness of breath.
There is currently no cure for IPF. Instead, it gets progressively worse over time and eventually leads to death. For this reason, healthcare providers consider idiopathic pulmonary fibrosis a terminal illness.
In IPF, scar tissue or fibrosis builds up around the air sacs(alveoli) in the lungs and reduces their ability to transfer oxygen that is breathed in to the blood.
We do not know what causes IPF. Research suggests that fibrosis occurs in response to damage of the cells that line the air sacs (alveoli). It is likely due to a combination of genetic and environmental factors, such as smoking, dusts from metal, wood and agricultural work.
IPF is the commonest form of pulmonary fibrosis and it is estimated that there are 6,000 new cases of this condition each year in the UK. It usually develops in people aged 70 and older, and is more common in men. It can occur in younger individuals particularly if there is a family history of idiopathic pulmonary fibrosis.
IPF may run in families: it has been estimated that 5-20% of patients have a relative with the disease. This is termed familial pulmonary fibrosis.
People with idiopathic pulmonary fibrosis are usually breathless and may have a cough. They may also describe feeling tired or fatigue and weight loss. Pulmonary hypertension (high blood pressure in the arteries of the lungs) can occur as a consequence of low blood oxygen levels associated with IPF.
Find more about pulmonary fibrosis and its symptoms.
Anti-fibrotic drugs are often prescribed for IPF to slow down the scarring process in the lungs and improve survival.
There are a number of treatments to help with symptom control. These include:
Your doctor may suggest assessment by palliative and supportive care specialists for helping with symptom control and psychological support.
For people with chronic lung disease, you may be advised to have a pneumonia vaccine and the seasonal flu vaccine.
If you smoke, there is help to stop smoking
For some people, referral for lung transplantation may be discussed.
There are a number of novel therapies being evaluated in clinical trials for IPF. Please discuss with your doctor if you are interested in finding out about clinical trials.
Your medical team will monitor you regularly to understand how your IPF is progressing, and whether you require different treatment.
Idiopathic pulmonary fibrosis is a progressive and ultimately a terminal disease. Most patients may live three to five years after diagnosis. However, anti-fibrotic therapies may improve life expectancy.
IPF usually progresses steadily. But sometimes it can progress very rapidly, which is called an acute exacerbation. This can occur spontaneously or be triggered by a respiratory tract infection. If this happens, you must contact your ILD specialist nurse or GP urgently.
It’s important to remember that you are not alone. There are now over 75 pulmonary fibrosis support groups around the country.
Support groups meet informally to share experiences, provide expert information and raise funds and awareness.
APF runs an email and telephone support line offering information and advice about living with PF.
Idiopathic pulmonary fibrosis is a progressive and ultimately a terminal disease. Most patients may live three to five years after diagnosis. However, anti-fibrotic therapies may improve life expectancy. IPF usually progresses steadily.
Pulmonary fibrosis is a group of serious lung diseases that affect the respiratory system. Pulmonary fibrosis scars and thickens lung tissue. It impacts the connecting tissue in the lung and the alveoli (air sacs inside the lungs).
The lung damage gradually gets worse over time. Hard, stiff lung tissues don’t expand as well as they should, making it harder to breathe. Pulmonary fibrosis may cause shortness of breath when you do routine tasks that never seemed tiring before.
Alveoli are tiny, delicate air sacs in your lungs. They help get oxygen into the bloodstream when you inhale.
In pulmonary fibrosis, the thin walls of these air sacs start to scar and thicken. When that happens, it’s harder for the air sacs to do their job and get oxygen to the rest of the body.
Yes, healthcare providers typically consider pulmonary fibrosis a terminal illness. Pulmonary fibrosis is a progressive disease (gets worse over time). There is no cure, and it eventually leads to death.
Many things factor into how long and well people can live with pulmonary fibrosis. The disease may get worse quickly (over months) or very slowly (over years). Newer medications may help slow the disease progression. Research continues to focus on improving therapies.
No, pulmonary fibrosis and chronic obstructive pulmonary disease (COPD) are not the same. However, they are similar in some ways. Pulmonary fibrosis and COPD are both lung diseases that get worse over time. Both conditions can make breathing difficult.
But these conditions affect your lungs differently:
Idiopathic is a term providers use when they can’t determine what caused a condition. Idiopathic pulmonary fibrosis is the most common type of interstitial lung disease.
Pulmonary fibrosis is a group of serious lung diseases that affect the respiratory system. Pulmonary fibrosis scars and thickens lung tissue. It impacts the connecting tissue in the lung and the alveoli (air sacs inside the lungs).
The lung damage gradually gets worse over time. Hard, stiff lung tissues don’t expand as well as they should, making it harder to breathe. Pulmonary fibrosis may cause shortness of breath when you do routine tasks that never seemed tiring before.
Alveoli are tiny, delicate air sacs in your lungs. They help get oxygen into the bloodstream when you inhale.
In pulmonary fibrosis, the thin walls of these air sacs start to scar and thicken. When that happens, it’s harder for the air sacs to do their job and get oxygen to the rest of the body.
Yes, healthcare providers typically consider pulmonary fibrosis a terminal illness. Pulmonary fibrosis is a progressive disease (gets worse over time). There is no cure, and it eventually leads to death.
Many things factor into how long and well people can live with pulmonary fibrosis. The disease may get worse quickly (over months) or very slowly (over years). Newer medications may help slow the disease progression. Research continues to focus on improving therapies.
No, pulmonary fibrosis and chronic obstructive pulmonary disease (COPD) are not the same. However, they are similar in some ways. Pulmonary fibrosis and COPD are both lung diseases that get worse over time. Both conditions can make breathing difficult.
But these conditions affect your lungs differently:
Idiopathic is a term providers use when they can’t determine what caused a condition. Idiopathic pulmonary fibrosis is the most common type of interstitial lung disease.
