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PubMed This is a summary of 63 peer-reviewed journal articles Updated
Pulmonology · Combined Pulmonary Fibrosis and Emphysema

Combined Pulmonary Fibrosis and Emphysema (CPFE): A Patient Guide

At a Glance

CPFE combines emphysema and lung scarring. Their opposing effects can make basic lung tests look preserved even when oxygen transfer is impaired. Care focuses on stopping smoking, measuring oxygen during activity, and screening for high blood pressure in the lungs and lung cancer.

Combined Pulmonary Fibrosis and Emphysema (CPFE) is a unique medical syndrome where two distinct lung conditions—emphysema (damaged, enlarged air sacs) and pulmonary fibrosis (stiff, scarred tissue)—exist in the same person at the same time. Typically, emphysema is more prominent in the upper parts of the lungs, while fibrosis affects the lower parts, though the patterns can vary widely [1]. While most commonly linked to a history of heavy smoking, CPFE can also be triggered by certain autoimmune diseases or long-term exposure to industrial dust and fumes [2][3].

Living with CPFE often involves a confusing paradox: you may feel significantly short of breath during daily activities, yet your basic breathing volume tests (like spirometry) may come back as “relatively preserved” or less abnormal than expected. This happens because the two conditions have opposing effects on lung size. The emphysema makes your lungs extra stretchy, while the fibrosis makes them stiff. These effects can balance each other out, making your total lung volume appear somewhat average on paper [4][1]. However, both conditions damage the delicate surface area where oxygen enters your blood, meaning your actual ability to transfer oxygen is often much more impaired than your breathing volume suggests [5].

Because of this complex interaction, CPFE is a syndrome, not a single uniform disease. Successfully managing it requires a specialized, individualized approach:

  • Smoking Cessation: This is the most critical step to prevent further damage [6].
  • Identifying the Cause: Securing a precise diagnosis of the underlying cause of the fibrosis determines your treatment path [7].
  • Targeted Monitoring: Monitoring must go beyond basic volume tests to include more sensitive measures, such as diffusion capacity (DLCO) and walk tests that track how your oxygen levels behave during physical activity [8].
  • Complication Vigilance: Your care team will actively screen for two major complications—pulmonary hypertension (high blood pressure in the lung arteries) and lung cancer. Your eligibility for cancer screening scans will depend on your age and smoking history [9][10].

By working with a multidisciplinary team of specialists, you can ensure that every aspect of this syndrome is addressed with a personalized plan focused on protecting your lung function, managing symptoms, and maintaining your quality of life [11].

In this guide

6 chapters

01

Understanding Your Diagnosis of CPFE

Learn what combined pulmonary fibrosis and emphysema (CPFE) means, how CT and breathing tests show both conditions, and why DLCO, oxygen, and monitoring matter.

02

Symptoms and Warning Signs of CPFE

Learn about combined pulmonary fibrosis and emphysema symptoms, including activity-related breathlessness, low oxygen, cough, and emergency warning signs.

03

Biology and Diagnosis of CPFE

Learn how CPFE develops and is diagnosed, including smoking risks, HRCT patterns, spirometry, DLCO testing, fibrosis types, and treatment implications.

04

Treatment Strategy and Daily Management

Learn how combined pulmonary fibrosis and emphysema (CPFE) is managed with inhalers, antifibrotic medicines, oxygen, rehabilitation, and safety monitoring.

05

Complications and Long-Term Monitoring

Learn how CPFE is monitored over time, including DLCO, 6-minute walk tests, CT scans, pulmonary hypertension checks, and lung cancer screening to guide care.

06

Prognosis and Advanced Care Options

Learn how combined pulmonary fibrosis and emphysema (CPFE) prognosis is assessed, including lung transplant, palliative care, and advanced disease planning.

Common questions in this guide

What is combined pulmonary fibrosis and emphysema, or CPFE?
CPFE is a syndrome in which emphysema and pulmonary fibrosis occur in the same person, often in different parts of the lungs. It can cause significant breathlessness and impaired oxygen transfer even when basic breathing volumes look relatively preserved.
Why can spirometry look relatively normal if CPFE causes shortness of breath?
Emphysema can make the lungs more stretchy, while pulmonary fibrosis makes them stiff. These opposing effects may partly balance each other on breathing-volume tests, even though both conditions damage the lung surface that transfers oxygen into the blood.
Which tests are used to monitor CPFE?
Monitoring CPFE usually needs more than spirometry. Doctors may use diffusion capacity testing, called DLCO, and walking tests that measure how oxygen levels change during activity; imaging and other tests may be added based on the fibrosis pattern and possible complications.
What can cause or increase the risk of CPFE?
CPFE is most commonly linked with a history of heavy smoking. It can also occur with certain autoimmune diseases or long-term exposure to industrial dust and fumes.
How is CPFE treated?
Treatment is individualized because CPFE is a syndrome rather than one uniform disease. Stopping smoking is a critical step, and identifying the cause and pattern of the fibrosis helps the care team choose an appropriate management plan.
Which complications should people with CPFE watch for?
Care teams commonly monitor for pulmonary hypertension, which is high blood pressure in the lung arteries, and lung cancer. Whether cancer screening scans are appropriate and how often they are needed depends in part on age and smoking history.

Questions For You

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Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Given my combination of emphysema and fibrosis, which specific tests are you using to track my disease since my basic breathing tests might look reassuring?
  2. 2.Based on my imaging, what is the pattern of my fibrosis, and how does that affect the treatment options available to me?
  3. 3.How often should we be checking for complications like pulmonary hypertension or scheduling lung cancer screenings?
  4. 4.What are my specific oxygen goals during exercise versus when I am resting?

References

References (11)
  1. 1

    Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement.

    Cottin V, Selman M, Inoue Y, et al.

    American journal of respiratory and critical care medicine 2022; (206(4)):e7-e41 doi:10.1164/rccm.202206-1041ST.

    PMID: 35969190
  2. 2

    Combined pulmonary fibrosis and emphysema.

    Caceres JD, Venkata A

    Current opinion in pulmonary medicine 2024; (30(2)):167-173 doi:10.1097/MCP.0000000000001044.

    PMID: 38164807
  3. 3

    Combined Pulmonary Fibrosis and Emphysema in a Patient With Chronic Occupational Exposure to Trichloroethylene.

    Asif H, Braman SS

    Military medicine 2024; (189(3-4)):e907-e910 doi:10.1093/milmed/usad359.

    PMID: 37769213
  4. 4

    Combined Pulmonary Fibrosis and Emphysema: Pulmonary Function Testing and a Pathophysiology Perspective.

    Amariei DE, Dodia N, Deepak J, et al.

    Medicina (Kaunas, Lithuania) 2019; (55(9)) doi:10.3390/medicina55090580.

    PMID: 31509942
  5. 5

    Combined pulmonary fibrosis and emphysema characteristics in a Greek cohort.

    Malli F, Papakosta D, Antoniou K, et al.

    ERJ open research 2019; (5(1)) doi:10.1183/23120541.00014-2018.

    PMID: 30895186
  6. 6

    Combined Pulmonary Fibrosis and Emphysema: Comparative Evidence on a Complex Condition.

    Calaras D, Mathioudakis AG, Lazar Z, Corlateanu A

    Biomedicines 2023; (11(6)) doi:10.3390/biomedicines11061636.

    PMID: 37371731
  7. 7

    Combined pulmonary fibrosis and emphysema as a clinicoradiologic entity: Characterization of presenting lung fibrosis and implications for survival.

    Alsumrain M, De Giacomi F, Nasim F, et al.

    Respiratory medicine 2019; (146()):106-112 doi:10.1016/j.rmed.2018.12.003.

    PMID: 30665507
  8. 8

    Long-term follow-up results of patients with combined pulmonary fibrosis and emphysema: A single-center experience.

    Calis AG, Uzer F, Gurbuz AF, et al.

    Respiratory medicine 2026; (255()):108780 doi:10.1016/j.rmed.2026.108780.

    PMID: 41856387
  9. 9

    An increased risk of pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema: a meta-analysis.

    Ni H, Wei Y, Yang L, Wang Q

    BMC pulmonary medicine 2023; (23(1)):221 doi:10.1186/s12890-023-02425-4.

    PMID: 37344866
  10. 10

    An increased risk of lung cancer in combined pulmonary fibrosis and emphysema patients with usual interstitial pneumonia compared with patients with idiopathic pulmonary fibrosis alone: a systematic review and meta-analysis.

    Chen Q, Liu P, Zhou H, et al.

    Therapeutic advances in respiratory disease 2021; (15()):17534666211017050 doi:10.1177/17534666211017050.

    PMID: 34011211
  11. 11

    Diagnosis and Management of Idiopathic Pulmonary Fibrosis.

    Munchel JK, Shea BS

    Rhode Island medical journal (2013) 2021; (104(7)):26-29.

    PMID: 34437662

This page explains CPFE, its testing, and monitoring for educational purposes only and is not medical advice. Your healthcare team can interpret your results and recommend care for your situation.