Pulmonary fibrosis (PF) is characterized by the formation of scar tissue in the lungs, which impairs their normal function and makes it harder for oxygen to reach the bloodstream.
Despite varying from person to person, PF symptoms tend to be progressive, meaning they worsen gradually over time. At times, patients also may experience acute exacerbations — periods when symptoms suddenly worsen, which usually require prompt treatment.
Shortness of breath, also known as dyspnea, is one of the most common symptoms of PF. People with PF usually notice dyspnea first when they are exercising or being physically active. At later stages, however, dyspnea can occur during less-demanding activities, or even when patients are at rest.
Low blood oxygen levels can cause tachypnea, or fast, shallow breathing. In adults, tachypnea is generally defined as taking more than 20 breaths per minute. (The average healthy adult takes about 12–20 breaths per minute.)
Another characteristic respiratory symptom of PF is a persistent, dry, or hacking cough.
Many people with PF experience substantial fatigue — a feeling of overwhelming tiredness or physical malaise, that usually is not alleviated with sleep. Notably, many patients also experience sleep issues, such as sleep apnea — a condition in which a person stops breathing repeatedly during sleep. These breathing issues are thought to be driven in part by the body’s lack of oxygen, in combination with other factors.
Aches in joints and muscles also are common in people with PF. These are thought to develop in large part because the tissues are not receiving enough oxygen.
Another common feature of PF, particularly idiopathic pulmonary fibrosis — meaning the cause is unknown — is digital clubbing, or swelling at the tips of the fingers and/or toes. The exact causes of this symptom are not fully understood, though low blood oxygen levels and abnormalities in some signaling molecules that control swelling are thought to play a role.
People with PF may experience a loss of appetite and lose weight without trying. This can arise due to a number of interconnected factors, including treatment side effects and mental health problems, such as depression.
PF can cause patients to develop other respiratory complications, such as pneumothorax — a condition in which a lung is partially or completely collapsed. Lung scarring also can lead to pneumomediastinum, also called mediastinal emphysema, a condition in which air leaks out into the space between the lungs in the chest.
People with PF may develop pulmonary edema, a condition in which fluid builds up in the lungs in the absence of an infection. They may be at high risk of developing severe lung infections, which can lead to pneumonia.
The risk of pulmonary embolism — when a clot blocks blood flow in the lungs — is higher than normal in people with PF. The risk of other circulatory-related conditions, such as acute coronary syndrome — a condition in which blood supply to the heart is reduced, potentially leading to a heart attack — also is greater in PF.
Scar tissue in the lungs can put strain on the organs’ blood vessels and cause pulmonary hypertension, a condition in which blood pressure in the lungs’ blood vessels is excessively high. This also puts strain on the heart, particularly on its the right ventricle — one of the organ’s lower chambers that is responsible for pumping blood to the lungs. Over time, this may lead to heart failure.
Last updated: Sept. 21, 2021
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