- Pulmonary fibrosis causes lung scarring that leads to shortness of breath, dry cough, and fatigue.
- It can be linked to smoking, environmental exposure, autoimmune diseases, or certain medications.
- Treatment helps manage symptoms and slow progression but does not cure the disease.
Pulmonary fibrosis is a chronic lung disease caused by scarring in the lungs. This scarring makes the lungs stiffer and less able to expand, which can lead to trouble breathing, shortness of breath, a dry cough, and extreme tiredness.
This lung scarring, known as fibrosis, usually develops after long-term exposure to chemicals or toxins. It can also be linked to smoking, autoimmune diseases, or side effects from medications used for a long time.
If pulmonary fibrosis is suspected, it is important to see a pulmonologist to confirm the diagnosis and begin the right treatment. Depending on the severity of the disease, treatment may include medications, respiratory therapy, or a lung transplant.
Main symptoms
The main symptoms of pulmonary fibrosis are:
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Dry cough or cough with a small amount of mucus
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A feeling of noise or wheezing in the chest while breathing
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Fast, labored breathing
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A feeling of tightness in the chest
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Muscle or joint pain
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Excessive tiredness
The fingers may also appear bluish or purplish. Over time, the fingertips can become more rounded and the nails may widen, a change known as digital clubbing. According to the American Thoracic Society (ATS), clubbing is associated with chronic lung diseases such as pulmonary fibrosis and long-term low oxygen levels in the blood.
At first, pulmonary fibrosis may not cause symptoms. As the disease progresses and the lungs become less able to expand, symptoms usually begin to emerge.
The severity of symptoms and how quickly they appear can vary depending on the cause. In some cases, the disease may worsen over a period of months to years.
Confirming a diagnosis
Pulmonary fibrosis is diagnosed by a pulmonologist (lung specialist), who reviews a person’s symptoms, medical history, and lifestyle habits. The doctor will also perform a physical exam and listen to the lungs while the person breathes to check for abnormal sounds.
The doctor may also perform pulse oximetry by placing a small device on one of the fingers to measure the amount of oxygen in the blood.
In addition, the doctor may order blood tests, such as arterial blood gas (ABG) testing, electrolyte levels, a complete blood count (CBC), urea and creatinine tests, and liver function tests (LFTs).
To evaluate lung damage, imaging tests such as a chest X-ray or computed tomography (CT) scan may be ordered. Spirometry may also be done to assess breathing function.
Other tests that may be used to confirm the diagnosis of pulmonary fibrosis include bronchoscopy or a lung biopsy.
Main causes
Some factors that may contribute to the development of pulmonary fibrosis include:
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Family history of pulmonary fibrosis
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Smoking
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A history of lung diseases, such as tuberculosis, pneumonia, or severe complications from COVID-19
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Autoimmune diseases, such as lupus, rheumatoid arthritis, and polymyositis
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Working in or being exposed to environments with dust, gases, chemicals, toxins, and heavy metals
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Frequent contact with bird droppings or feathers
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Radiation therapy or chemotherapy for the treatment of lung or breast cancer
In addition, the use of some medications for heart rhythm problems, antibiotics, and anti-inflammatory drugs, such as amiodarone, propranolol, nitrofurantoin, and sulfasalazine, may contribute to the development of pulmonary fibrosis.
Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis is a type of pulmonary fibrosis in which scarring develops in the lungs without a known cause. It is the most common form of the disease and usually affects adults over age 65.
Although the exact cause is unknown, some factors are often associated with it, such as smoking, gastroesophageal reflux, and genetic predisposition.
Pulmonary fibrosis vs cystic fibrosis
Pulmonary fibrosis is caused by scarring in the lungs related to environmental factors or certain diseases.
Cystic fibrosis, on the other hand, is a hereditary disease that appears in childhood. In this condition, some glands produce abnormal secretions that mainly affect the digestive tract and the lungs.
Treatment options
Treatment for pulmonary fibrosis should be guided by a pulmonologist. The goal is to relieve symptoms and help slow the progression of the disease, and treatment may include:
1. Use of medications
The medications a pulmonologist may prescribe for pulmonary fibrosis are used to try to slow lung scarring and control inflammation.
According to the American Thoracic Society (ATS), antifibrotic medications such as nintedanib and pirfenidone help slow the decline in lung function in idiopathic pulmonary fibrosis. Corticosteroids such as prednisone may also be used to reduce inflammation.
It may also be necessary to treat other conditions, such as autoimmune diseases or reflux, because they can make lung scarring worse.
2. Respiratory therapy
Respiratory therapy helps strengthen the lungs, making it easier to breathe and helping relieve symptoms such as cough and fatigue. This can improve quality of life for a person with pulmonary fibrosis.
Exercises recommended by the physical therapist may include deep breathing techniques, chest expansion exercises, and light physical activities such as walking and stretching, which can help improve lung endurance.
3. Oxygen therapy
Oxygen therapy is a treatment that helps increase oxygen levels in the blood, which may drop because of lung damage caused by pulmonary fibrosis.
The doctor may recommend using it for a few hours a day or continuously, depending on each person’s needs.
There are different forms of oxygen therapy, such as oxygen masks, nasal cannulas, portable oxygen concentrators, or liquid oxygen systems. The pulmonologist chooses the most appropriate option based on the person’s condition.
4. Lung transplant
A lung transplant may be recommended by the doctor in cases of severe pulmonary fibrosis with progressive symptoms, as it can improve breathing ability and quality of life.
However, a lung transplant carries several risks, especially organ rejection. In addition, the person may need to take immunosuppressive medications for the rest of their life.
Is pulmonary fibrosis curable?
Pulmonary fibrosis has no cure. Because of this, treatment focuses on relieving symptoms, slowing disease progression, and improving quality of life.