Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung condition. IPF causes scar tissue to build up in the lungs, leading to shortness of breath and a persistent cough.
According to the National Heart, Lung, and Blood Institute (NHLBI), IPF is a progressive condition with no known cure. However, treatment plans can help people manage the condition and improve their quality of life.
In this article, we look at the causes, symptoms, and diagnosis of IPF, as well as how people can manage the condition.
We also look at potential complications and when to see a doctor.
Pulmonary fibrosis is a scarring of the lungs.
The NHLBI state that IPF occurs when the lung tissue that surrounds the air sacs thickens and becomes stiff, leading to permanent scarring of the lungs.
This scarring affects the function of the lungs, making it difficult to breathe.
In the past, other names for IPF included cryptogenic fibrosing alveolitis, idiopathic diffuse interstitial pulmonary fibrosis, and idiopathic fibrosing alveolitis, chronic form. Nowadays, doctors may refer to IPF as usual interstitial pneumonia.
According to the NHLBI, the symptoms of IPF develop gradually over time for some people but appear more quickly for others.
The symptoms of IPF include:
- feeling short of breath, which tends to occur when exercising but may worsen over time and cause people to feel out of breath when resting
- a dry cough that worsens and can involve bouts of uncontrollable coughing
- aching muscles and joints
- clubbing, which is where the tips of the fingers or toes become wider and rounder
- unintentional weight loss over time
- feeling unwell in general
- loss of appetite
- pain or tightness in the chest
The authors of a 2014 article state that there are no official stages of IPF but that doctors have traditionally loosely staged IPF using terms such as early or advanced, or mild, moderate, or severe.
At this stage of IPF, people may show mild symptoms of IPF and receive an initial diagnosis.
In some cases, though, people may not have any symptoms until the moderate stage of IPF.
At this stage, there will be a large amount of scarring in the lungs, and people may need oxygen therapy.
Symptoms are severe at this stage, and a lung transplant may be necessary.
The National Organization for Rare Disorders (NORD) suggest that most cases of IPF occur as a result of damage to the epithelial cells that line the alveoli and airways.
The NORD suggest that the body attempts to repair the damaged cells, but the response is abnormal, resulting in progressive damage and scarring to the lung tissue and alveoli.
However, researchers do not know why the initial damage that triggers the healing response occurs.
Although there is no known cause of IPF, according to the National Library of Medicine, researchers think that a mix of environmental, lifestyle, and genetic factors play a part in the disease.
The most significant risk factor appears to be a variation in the MUC5B gene, which results in high mucus production in the respiratory bronchioles.
Other risk factors for IPF include:
- Genetics: People with a family history of IPF have a higher risk of IPF, particularly if a first degree relative, such as a parent or sibling, has IPF. However, IPF can also occur sporadically.
- Smoking cigarettes: About 75% of people with IPF are smokers or have previously been smokers.
- Gastroesophageal reflux disease (GERD): Roughly 75% of people with IPF have acid reflux or heartburn symptoms.
- Being male: Approximately 75% of people with IPF are male.
- Age: Most people with IPF are over the age of 50 years.
According to the NHLBI, although there is currently no cure for IPF, treatments may help slow the progression of IPF and improve quality of life.
The treatment options depend on the stage of IPF but may include:
- Kinase inhibitors: This type of medication can help slow down a loss of lung function and may prevent a sudden worsening of IPF.
- Antacids: These help treat GERD if people also have this condition.
- Oxygen therapy: Initially, a person may need this therapy after exertion. In the later stages of IPF, they may need it continually.
- Ventilator support: A person may need this support if their breathing problems become severe.
- Lung transplant: This surgery may be necessary for people with advanced IPF.
Other forms of treatment depend on the person’s symptoms. For example, if a person has developed a lung infection, they may require antibiotics. For a chronic cough, they may take oral codeine.
Pulmonary rehabilitation may also be a part of their treatment plan.
Pulmonary rehabilitation is a program that may involve:
- breathing exercises
- physical activity to strengthen the body
- nutritional advice
- education on IPF and how to manage the condition
Keeping to a treatment plan can help people with IPF manage the condition and improve their quality of life.
Steps that people can take to manage IPF include:
- keeping track of symptoms and letting a healthcare professional know if they worsen
- attending regular checkups
- refraining from smoking cigarettes and avoiding secondhand smoke
- avoiding substances that can irritate the lungs, such as chemicals and dust
- staying physically active and keeping up regular, moderate exercise, such as walking or using an exercise bike
- maintaining a moderate weight
- eating to support heart and lung health, such as eating smaller meals to reduce fullness, which may ease breathing
- joining support groups, having counseling, and talking with family and friends can help people take care of their mental health
- avoiding places or situations that may make breathing more difficult, such as being at high altitudes
The Canadian Lung Association state that to diagnose IPF, a doctor will assess any symptoms, take a full medical history, and carry out a physical examination.
During the physical examination, they may check whether a person has:
- blue hands and feet
- clubbed toes or fingers
- high pitched crackles coming from the lungs
A combination of tests may help a doctor confirm a diagnosis of IPF. These tests may include:
- Breathing test: To check how well the lungs breathe air in and out and how well they oxygenate the blood.
- Chest X-ray: To look for signs of disease and progression.
- Blood tests: To identify other reasons for lung scarring.
- CT scan: To look for scarring in the lungs.
- Lung biopsy: To confirm a diagnosis if this is not possible based on a CT scan.
According to a 2018 article in the journal Medical Sciences, possible complications of IPF can include:
According to the American Lung Foundation, there are more than 200 types of pulmonary fibrosis (PF), and the most common is IPF.
The Pulmonary Fibrosis Foundation state that there are five main categories for PF based on their causes:
- Drug-induced PF: Due to the previous or current use of drugs that affect the lungs, such as chemotherapy, amiodarone (Pacerone), or nitrofurantoin (Macrobid).
- Radiation-induced PF: Due to radiation treatment of the chest.
- Environmental PF: Due to exposure from lung irritants, such as mold.
- Autoimmune PF: Due to autoimmune diseases, such as rheumatoid arthritis or Sjogren’s syndrome.
- Occupational PF: Due to exposure to dust or fumes, such as asbestos or silica.
IPF differs from the other form of PF because it has no known cause.
People should see a doctor if they have any symptoms of IPF. A doctor may refer people to a specialist called a pulmonologist.
People with IPF may work with a team of healthcare professionals who can make sure that they receive an accurate diagnosis, as well as the most current treatment plan.
IPF is a type of pulmonary fibrosis with no known cause. Environmental, lifestyle, and genetic factors are all among the risk factors.
If people have any symptoms of IPF, they should see their doctor for a checkup and diagnosis.
Although there is no cure for IPF, people can work with a team of healthcare professionals to manage the condition and improve their quality of life.