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PubMed This is a summary of 13 peer-reviewed journal articles Updated
Pulmonology · Combined Pulmonary Fibrosis and Emphysema Syndrome

Complications and Long-Term Monitoring

At a Glance

Because emphysema and fibrosis can offset each other, a stable FVC does not always mean CPFE is stable. Long-term monitoring should also track DLCO, oxygen levels during walking, CT changes, pulmonary hypertension, and lung cancer risk.

Monitoring Combined Pulmonary Fibrosis and Emphysema (CPFE) requires a more detailed approach than monitoring either condition on its own. Because your lungs are experiencing two different types of damage, the typical tests doctors use for other lung diseases can sometimes be misleading. Your care team must look at several layers of information to catch complications early and adjust your treatment plan [1].

Why FVC is Not Enough

In many lung diseases, doctors rely heavily on FVC (Forced Vital Capacity), which measures the total amount of air you can breathe out. However, in CPFE, the FVC can stay relatively stable even while your disease is progressing. This happens because the emphysema (which makes lungs larger) and the fibrosis (which makes lungs smaller) can balance each other out on the test [1][2].

To truly understand how your lungs are doing, your monitoring plan should focus on these more sensitive markers:

  • Serial DLCO Tests: This test uses a safe amount of carbon monoxide to estimate how well your lungs transfer gas into your blood. A decline in DLCO over time is an important sign that CPFE may be progressing, even if your lung volumes look stable, though it can also be affected by other factors like anemia [3][4].
  • 6-Minute Walk Test (6MWT): This test tracks how far you can walk in six minutes and, more importantly, how much your oxygen levels drop during that walk (exertional desaturation). A significant drop in oxygen or a decrease in the distance you can walk is a key indicator of your lung health [3][5].
  • High-Resolution CT (HRCT): Periodic imaging allows your doctor to see if the physical amount of scarring or emphysema is increasing, which provides a visual “map” of progression that breathing tests might miss [3][6].

Complication 1: Pulmonary Hypertension (PH)

One of the most common and serious complications of CPFE is Pulmonary Hypertension, or high blood pressure in the arteries of your lungs [1]. Reported rates vary widely depending on the study population, but it is an important and relatively frequent complication [7][8].

  • What it is: PH happens when the lung arteries undergo vascular remodeling and hypoxic vasoconstriction, making it harder for the right side of your heart to pump blood through your lungs [9].
  • How it is screened: Doctors use an echocardiogram (an ultrasound of the heart) to look for signs of heart strain and estimate lung pressures [10]. A normal echocardiogram does not entirely rule out PH.
  • How it is confirmed: If your screening tests suggest PH, your specialist may recommend a right-heart catheterization to directly and accurately measure the pressure in your lung arteries [9].

Complication 2: Increased Lung Cancer Risk

People with CPFE face a significantly higher risk of developing lung cancer than the general population—and even a higher risk than those with just fibrosis or just emphysema [11][12].

Because of this, regular screening is vital, but it is not automatic just because you have CPFE. Most specialists recommend Low-Dose CT (LDCT) scans, which are specialized scans designed to find cancer early. Your doctor will determine how often you need these scans based on established local guidelines regarding your age and smoking history.

Topics to Discuss at Your Visits

There is no universal, fixed schedule for testing in CPFE. The frequency of your tests will be individualized based on your symptoms, prior results, and treatments. Use this list to ensure you are receiving the comprehensive care required:

  • Comprehensive PFTs: Discuss if your latest tests included DLCO, or just basic spirometry [3].
  • Oxygen Check: Ask if your oxygen level was measured during a 6-minute walk test, rather than just while you were sitting still [1].
  • Heart Health: Discuss if you need an echocardiogram to check for signs of pulmonary hypertension [10].
  • Cancer Screening: Check if you are eligible and up to date on your Low-Dose CT lung cancer screening based on your age and smoking history [13].
  • Imaging Review: Ask a specialist to compare your latest HRCT scan to your previous one to look for physical changes in scarring or emphysema [3].

Common questions in this guide

Why can my FVC stay stable if CPFE is getting worse?
Emphysema can make the lungs larger while fibrosis makes them smaller and stiffer, so the two effects may partly offset each other on an FVC test. A stable FVC therefore does not always rule out progression. Doctors may also follow DLCO, oxygen levels during walking, and CT changes.
What tests are used to monitor CPFE over time?
Monitoring commonly includes full pulmonary function tests with DLCO, a six-minute walk test that measures oxygen levels during activity, and periodic high-resolution CT scans. These tests assess gas transfer, exertional oxygen drops, and changes in scarring or emphysema. The schedule is individualized to your symptoms and previous results.
How is pulmonary hypertension checked in someone with CPFE?
An echocardiogram can look for right-heart strain and estimate pressure in the lung arteries, but a normal result does not completely exclude pulmonary hypertension. If there is enough concern, right-heart catheterization can directly measure the pressure and confirm the diagnosis.
Does everyone with CPFE need low-dose CT lung cancer screening?
CPFE is associated with a higher risk of lung cancer, but the diagnosis alone does not automatically determine screening. Your doctor will assess your eligibility and scan frequency using local guidelines, your age, and your smoking history.
What does oxygen desaturation during a six-minute walk test mean?
Oxygen desaturation means your blood oxygen level falls during activity, suggesting that your lungs are having more difficulty transferring oxygen when they are working harder. A significant drop may lead your care team to evaluate whether home oxygen could help. Your clinician should interpret the result in the context of your other tests.
How often should CPFE monitoring tests be repeated?
There is no single testing schedule for everyone with CPFE. Doctors usually tailor the timing to your symptoms, earlier test results, imaging findings, and treatments. Ask whether your follow-up includes DLCO, walking oxygen measurements, heart evaluation, and imaging when appropriate.

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Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.How much emphysema versus fibrosis is visible on my scans, and how does that ratio affect my personal risk for pulmonary hypertension?
  2. 2.Given that my FVC (lung volume) can look stable, what specific DLCO and 6-minute walk test trends are you looking for to measure my progression?
  3. 3.Do I meet the local guidelines for Low-Dose CT (LDCT) lung cancer screening based on my age and smoking history?
  4. 4.What were the findings on my last echocardiogram regarding my heart's right-ventricular function and estimated pulmonary pressures?
  5. 5.Is my 6-minute walk test showing enough 'desaturation' (oxygen drop) that we should evaluate a home oxygen prescription?

References

References (13)
  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: 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
  3. 3

    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
  4. 4

    Mortality surrogates in combined pulmonary fibrosis and emphysema.

    Zhao A, Gudmundsson E, Mogulkoc N, et al.

    The European respiratory journal 2024; (63(4)) doi:10.1183/13993003.00127-2023.

    PMID: 37973176
  5. 5

    Clinical implications of six-minute walk test in patients with idiopathic pulmonary fibrosis: a retrospective cohort study.

    Kim MJ, Nathan SD, Kim HH, Kim HC

    Therapeutic advances in respiratory disease 2024; (18()):17534666241275329 doi:10.1177/17534666241275329.

    PMID: 39175212
  6. 6

    Emphysema or fibrosis progression in patients with combined pulmonary fibrosis and emphysema.

    Zhai L, Wang F, Liu H, et al.

    The American journal of the medical sciences 2025; (369(5)):562-567 doi:10.1016/j.amjms.2025.01.004.

    PMID: 39788421
  7. 7

    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
  8. 8

    Combined Pulmonary Fibrosis and Emphysema (CPFE) Clinical Features and Management.

    Hage R, Gautschi F, Steinack C, Schuurmans MM

    International journal of chronic obstructive pulmonary disease 2021; (16()):167-177 doi:10.2147/COPD.S286360.

    PMID: 33536752
  9. 9

    Severe pulmonary hypertension due to combined pulmonary fibrosis and emphysema: another cause of death among smokers.

    Hirano ACG, Targueta EP, Ferraz de Campos FP, et al.

    Autopsy & case reports 2017; (7(2)):15-26 doi:10.4322/acr.2017.022.

    PMID: 28740835
  10. 10

    Likelihood of pulmonary hypertension in patients with idiopathic pulmonary fibrosis and emphysema.

    Jacob J, Bartholmai BJ, Rajagopalan S, et al.

    Respirology (Carlton, Vic.) 2018; (23(6)):593-599 doi:10.1111/resp.13231.

    PMID: 29237236
  11. 11

    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
  12. 12

    Incidence of and predictive factors for lung cancer in patients with rheumatoid arthritis: A retrospective long-term follow-up study.

    Mori S, Hasegawa M, Sakai F, et al.

    Modern rheumatology 2025; (35(2)):240-248 doi:10.1093/mr/roae084.

    PMID: 39223693
  13. 13

    Lung cancer in combined pulmonary fibrosis and emphysema: a large retrospective cohort analysis.

    Nasim F, Moua T

    ERJ open research 2020; (6(4)) doi:10.1183/23120541.00521-2020.

    PMID: 33344625

This page is for informational purposes only and does not constitute medical advice. Ask your pulmonologist about the monitoring schedule, oxygen evaluation, heart testing, and lung cancer screening appropriate for your situation.