Pulmonary fibrosis (PF), as its name suggests, is a disease marked by scarring — or fibrosis — of the lungs, leading to shortness of breath. PF is part of a larger group of more than 200 lung conditions collectively known as interstitial lung diseases (ILDs), many of which are known to cause progressive fibrosis.
Types and causes of PF
In most cases, the exact cause of lung scarring is never determined, and the disease is classified as idiopathic pulmonary fibrosis (IPF) — or “of unknown cause.” More rarely, PF can run in families and have a genetic basis. This specific type of PF, which typically requires two or more members of the same family to have the disease, is known as familial pulmonary fibrosis.
PF also may develop in association with other diseases. These include autoimmune disorders like rheumatoid arthritis and scleroderma. Lung scarring also can be caused by certain infections, or exposure to toxic substances in the air.
Symptoms of PF
Scarring in the lungs can cause respiratory symptoms, such as shortness of breath, known as dyspnea, and a dry, hacking cough. Some patients also may experience tachypnea, or fast, shallow breathing. Fatigue and sleep problems are common among people with PF, as is unexplained weight loss and loss of appetite. Other symptoms may include aching muscles and joints, or digital clubbing, which is swelling at the tips of the fingers and toes.
Symptoms of PF tend to be progressive, meaning they start gradually and then slowly worsen over time, though patients may sometimes experience exacerbations — episodes in which their symptoms suddenly worsen, often in response to a trigger like an infection. PF also can cause serious health complications, including collapsed lung (pneumothorax), pneumonia, and heart failure.
Diagnosis of PF
There is no single test to confirm that a person has PF. Instead, a diagnosis typically involves several tests and a team of specialists working together. Imaging the lungs with X-rays or CT scans can help physicians look for signs of scarring. Lung function tests can be used to assess how well lungs are working, and blood tests can determine whether enough oxygen is being delivered to the bloodstream.
A biopsy of the lungs also may be taken during surgery or while patients undergo a bronchoscopy to look for direct evidence of scarring.
There is no cure for PF. Treatment, therefore, focuses on managing its symptoms, slowing its progression, and treating its underlying cause when it is known.
Some medications like Ofev (nintedanib) and Esbriet (pirfenidone) can slow PF’s progression, ease its symptoms, and improve patients’ quality of life. Oxygen therapy also may be beneficial for some patients.
Certain self-care measures, such as exercising regularly, maintaining a balanced and healthy diet, and quitting smoking also can help ease symptoms and possibly slow disease progression. Pulmonary rehabilitation has been found useful by some patients for maintaining and improving their lung health.
Palliative care, ranging from breathing assistance to pain relief, may be recommended as the disease advances and symptoms worsen. In cases of severe and extensive lung damage, a lung transplant may be necessary.
Last updated: Sept. 24, 2021
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