Combined pulmonary fibrosis and emphysema (CPFE) is a condition that causes symptoms of both pulmonary fibrosis and emphysema.
People with CPFE have both air sac destruction (seen in people with emphysema) and also scarred lung tissue of what holds the air sacs together (as seen with pulmonary fibrosis). CPFE is considered rare and — until recently — has usually been treated as two separate conditions.
CPFE is heavily linked to smoking, although environmental and genetic factors might also increase the risk. The only cure for CPFE is a lung transplant, but treatments such as inhaled corticosteroids and bronchodilators can improve symptoms in selected patients can help control chronic symptoms.
In most cases, these conditions are seen separately.
Pulmonary fibrosis occurs when lung tissue is scarred. The scarred tissue makes it difficult for your lungs to work correctly and makes it difficult to breathe.
Likewise, emphysema happens when the air sacs in your lungs (alveoli) are also scarred. The sacs weaken and rupture, reducing the surface area of your lungs and causing less oxygen to reach your bloodstream.
Although rare, it’s possible to have both pulmonary fibrosis and emphysema together.
Both conditions cause symptoms that can impact your daily life.
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Pulmonary fibrosis symptoms
The common symptoms of pulmonary fibrosis include:
Symptoms can be mild or severe. They can progress slowly or very quickly. Some people might have flare-ups or exacerbations of pulmonary fibrosis when their symptoms are especially bad for a short time period.
You may experience the following with emphysema:
This combined condition was
CPFE shares many symptoms with pulmonary fibrosis and emphysema, but some symptoms are more likely in people with CPFE. These include:
- shortness of breath that gets worse over time
- dry crackles or breath sounds
- widening at the tips of fingers and toes
- blue tint to the skin
There is still limited research into CPFE, and the conditions are still frequently treated separately.
However, increasing research is exploring the causes of and possible treatments for CPFE.
One of the primary causes for CPFE is thought to be smoking.
However, smoking isn’t the only risk factor. Not all smokers, even heavy smokers who develop other lung health conditions, develop CPFE. Researchers are still looking into what else might cause CPFE. It’s been suggested that environmental factors as well as workplace exposures including vapors, gases, dusts, and fumes may contribute to both fibrosis and emphysema. Genetics might also be involved, and it’s possible that a combination of these risk factors is behind the development of CPFE.
Currently, there are no treatments that are specific to CPFE.
One of the first treatments in almost all cases will likely be assistance to quit smoking. This is also the first-line treatment for both pulmonary fibrosis and emphysema. Stopping smoking can stop the progression of CPFE. Other lifestyle changes, such as avoiding secondhand smoke or industrial fumes, might also be recommended.
Other treatments will depend on symptoms and their severity.
- In rare cases, the use of corticosteroids or immunosuppressants can bring down inflammation and reduce symptoms.
- More commonly, the use of inhaled bronchodilators might be helpful to open airways and improve breathing. Other people with CPFE might use supplemental oxygen to improve the oxygen levels in their bodies.
- For people with advanced CPFE, a lung transplant might be the best and only treatment option.
The overall treatment course will depend on the individual.
The only curative treatment for CPFE is a lung transplant.
The damage caused by the condition is too severe to repair any other way. Treatments such as corticosteroids and inhalers can minimize symptoms, but they can’t restore lung function.
A transplant is the only way to restore full lung function.
If you have CPFE, your doctor can discuss your options with you and can let you know if you’re a good candidate for a transplant.
CPFE is thought to have a higher mortality rate than either pulmonary fibrosis or emphysema alone.
However, CPFE is rare and understudied. Different studies indicate that the reported 5-year survival rates ranged between
Additionally, new research may result in new treatments and better outcomes for people with CPFE. The current outlook for people with CPFE depends on the person, their symptoms, the severity of their case, and their overall health.
If you still have questions about pulmonary fibrosis and emphysema, you can read more about them by checking out the answers to some common questions below.
Can you have emphysema and pulmonary fibrosis together?
Yes. Having emphysema and pulmonary fibrosis together is known as combined pulmonary fibrosis and emphysema (CPFE).
What is the life expectancy for pulmonary fibrosis and emphysema?
The life expectancy for CPFE varies. CPFE is rare, and there has not been a lot of data collected. In studies, averages have ranged between
What’s the ICD-10 code for combined pulmonary fibrosis and emphysema?
The ICD-10 code for CPFE is J84. 10.
CPFE is a rare condition that combines the features of both pulmonary fibrosis and emphysema. CPFE is rare, and it is often treated as two conditions even when it occurs. However, since 2005, researchers and medical professionals have started to treat CPFE as a singular condition. By treating CPFE as one condition, researchers are hopeful that a cause can be found, and that treatment options can improve. Currently, the only curative treatment is a lung transplant. Doctors may consider prescribing corticosteroids, immunosuppressants, inhalers, as well as smoking cessation.