Pulmonary fibrosis and emphysema are two lung conditions that have some symptoms in common. These include a cough, shortness of breath, tiredness, and swelling in the legs.
However, the atypical physiology underlying the conditions and their causes are different. While the primary drug treatments are also different, interventions for both conditions may include oxygen therapy, pulmonary rehabilitation, and a lung transplant.
In addition to having either condition separately, it is possible to have both at the same time. Doctors refer to this as combined pulmonary fibrosis and emphysema (CPFE).
This article discusses pulmonary fibrosis versus emphysema, including similarities and differences. It also examines symptoms, treatment, and the outlook for people when the conditions co-occur.
Although they are two distinct lung conditions, pulmonary fibrosis and emphysema share similar features. There are also many differences between them.
Pathophysiology
Pathophysiology refers to the effects on the body that a condition can cause.
Pulmonary fibrosis produces scarring and inflammation around the alveoli, which are tiny air sacs in the lungs. The lungs and blood exchange oxygen and carbon dioxide at the alveoli during breathing. The scarring causes lung tissue to stiffen and become thicker, making it more difficult for the blood to absorb oxygen.
In contrast, emphysema destroys the alveoli. Over time, they rupture, allowing one big air pocket to form instead of many smaller ones. Consequently, the air becomes trapped in the damaged alveoli space, preventing oxygen from moving through the bloodstream.
Symptoms
Both conditions make breathing difficult, resulting in shortness of breath. Other symptoms they have in common include leg swelling and tiredness.
They both also produce a cough. The cough tends to be dry with pulmonary fibrosis and wet with emphysema. According to a
Causes
Over 200 different types of pulmonary fibrosis exist. The most common type, idiopathic pulmonary fibrosis, seems to have no known cause.
However, the cause of other types may involve:
- genetics
- autoimmune conditions such as rheumatoid arthritis
- environmental exposure to substances such as asbestos
Smoking is also a risk factor for all types of pulmonary fibrosis.
While smoking is the leading cause of emphysema, other factors may play a role. These can include:
- alpha-1 antitrypsin deficiency, a genetic condition that leads to a lack of a specific protective protein
- infections of the respiratory tract
- air pollutants
Treatment
Neither pulmonary fibrosis nor emphysema currently has a cure. However, treatments can help manage symptoms. Options for both conditions include oxygen therapy and, in severe cases, a lung transplant. Pulmonary rehabilitation, a program involving exercise training, nutritional counseling, and education, can also help with both.
Doctors use glucocorticoids, which are anti-inflammatory drugs, to treat both conditions. Other than this, the medications differ.
The primary drug treatment for pulmonary fibrosis involves antifibrotic agents, such as nintedanib (Ofev), which slow lung scarring or fibrosis. On the other hand, the primary drug treatment for emphysema involves bronchodilators, including albuterol (Proventil HFA) and others, which relax muscles surrounding the airways and help make breathing easier.
When the two conditions co-occur, doctors call it CPFE.
While the prevalence of CPFE is unknown, estimates suggest it may occur more often in males and people who smoke. However, CPFE also may affect those who do not smoke. The mean age of onset is 65–70 years old.
Symptoms
Symptoms of CPFE include labored or difficult breathing upon exertion and coughing.
Doctors often see two co-occurring conditions with CPFE: pulmonary hypertension and lung cancer. Pulmonary hypertension refers to high blood pressure of the arteries in the lungs and the right side of the heart.
Treatment
Existing research offers little data and guidelines concerning CPFE treatment. However, current practices include general measures, such as:
- pulmonary rehabilitation
- vaccinations against flu, pneumonia, and COVID-19
- quitting smoking, if applicable
- possible lung transplant
- oxygen therapy for people with low levels or oxygen saturation at rest or during exertion
Treatment also includes antifibrotic medications for pulmonary fibrosis, along with inhaled bronchodilators and inhaled corticosteroids for emphysema.
Outlook
According to a 2015 study, the outlook for people with CPFE is poor, with a 5-year survival rate of
The outlook for CPFE is less favorable than for emphysema alone. However, the outlook for people with CPFE compared with pulmonary fibrosis alone is unclear.
Pulmonary fibrosis and emphysema show similarities and differences. One similarity is that both can cause shortness of breath, a cough, tiredness, and swelling in the legs.
There are also several differences between the conditions, including their pathophysiology. Fibrosis and scarring underlie pulmonary fibrosis, while the destruction of alveoli underlies emphysema.
Also, there is no known cause associated with most cases of pulmonary fibrosis, while smoking is the leading cause of emphysema. Although treatment strategies for both conditions include medications, these involve different drug classes.
When the two conditions co-occur, doctors call this CPFE. Symptoms of CPFE include coughing and difficulty breathing on exertion.