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PubMed This is a summary of 17 peer-reviewed journal articles Updated
Pulmonology · Combined Pulmonary Fibrosis and Emphysema

Treatment Strategy and Daily Management

At a Glance

CPFE treatment uses a two-pronged plan: inhalers and smoking cessation for emphysema, antifibrotic medicines for qualifying or worsening fibrosis, and oxygen, rehabilitation, and specialist care to manage low oxygen and pulmonary hypertension.

Managing Combined Pulmonary Fibrosis and Emphysema (CPFE) requires a “two-pronged” approach. Because there is no single pill that treats both conditions, your care team will build a strategy that manages the emphysema and the fibrosis separately while monitoring for complications like low oxygen and heart strain [1].

Managing the Emphysema Component

The goal of treating the emphysema part of CPFE is to keep your airways as open as possible and prevent sudden flare-ups (exacerbations).

  • Smoking Cessation: This is the most critical step you can take. Stopping smoking is the only intervention proven to slow the damage in emphysema [2].
  • Inhalers: If you have demonstrable airflow limitation or asthma, your doctor may prescribe bronchodilators [1]. These include long-acting muscarinic antagonists (LAMA) or long-acting beta-agonists (LABA) that help relax the muscles around your airways [3][4].
  • Inhaled Steroids: In some cases of specific COPD phenotypes, combined inhalers (ICS/LABA) may be used to reduce inflammation, though they are not used for everyone with CPFE as they carry a potential increased risk of pneumonia [5].

Managing the Fibrosis Component

Treatment for the scarring (fibrosis) in your lungs depends on the specific pattern and cause of the damage.

  • Antifibrotic Medications: If your fibrosis is identified as Idiopathic Pulmonary Fibrosis (IPF) or is showing signs of worsening (called Progressive Pulmonary Fibrosis), your doctor may prescribe medications like nintedanib or pirfenidone, depending on local approvals [6][7].
  • How They Work: These drugs are not a cure and do not reverse existing scars. Instead, they work like a “brake” to slow down the rate at which physiological decline occurs [8][9].
  • Monitoring: These medications require regular blood tests to check your liver function and may cause side effects like nausea or diarrhea [10].

Oxygen, Activity, and Safety

Because CPFE severely affects your lungs’ ability to move oxygen into your blood, managing your oxygen levels is a cornerstone of daily care.

  • The Exertion Gap: Many people with CPFE have normal oxygen levels while sitting but experience significant “dips” (hypoxemia) the moment they start walking [11][12].
  • Supplemental Oxygen: Oxygen is prescribed to correct documented hypoxemia. Using oxygen during activity can help improve your exercise tolerance and allow you to stay active longer [3]. Safety is paramount: Never smoke or permit open flames near oxygen. Keep equipment away from heat, secure tanks properly, and never change your prescribed flow rate without medical instruction.
  • Pulmonary Rehabilitation: This is a supervised exercise and education program tailored for lung patients. It helps improve your physical strength and teaches you techniques to manage breathlessness and pacing [13][14].

Medications to Approach with Caution

A common complication of CPFE is pulmonary hypertension (PH), which is high blood pressure in the lung arteries. While there are many drugs used to treat PH in other patients, standard oral vasodilators are not routinely used by general practitioners in CPFE [15].

  • The Risk: Standard PH medications can sometimes worsen oxygen levels in people with lung scarring by sending blood to areas of the lung that cannot exchange gas effectively [16].
  • Expert Care: If you have severe PH, you should be evaluated at a specialized ILD/PH center. These experts can perform a right-heart catheterization and determine if specific therapies (such as inhaled treprostinil, which is approved in some jurisdictions for PH associated with interstitial lung disease) are appropriate for your specific case [15][17].

Common questions in this guide

How is combined pulmonary fibrosis and emphysema treated?
There is no single medicine that treats both parts of CPFE. Clinicians usually manage emphysema with smoking cessation and, when appropriate, inhalers, while treating qualifying fibrosis with antifibrotic medicine and monitoring oxygen levels, lung function, and pulmonary hypertension.
Can inhalers help if I have CPFE?
Bronchodilator inhalers such as LAMA or LABA may help when testing shows narrowed airways or asthma. Inhaled steroid combinations are used only for selected COPD patterns because they can increase the risk of pneumonia.
When are antifibrotic medicines used for CPFE?
Nintedanib or pirfenidone may be considered when the fibrosis is idiopathic pulmonary fibrosis or is worsening as progressive pulmonary fibrosis, depending on local approval. They slow further decline but do not remove existing scars, and regular liver blood tests are needed.
Do I need oxygen if my oxygen level is normal at rest?
Possibly. People with CPFE can have normal oxygen levels while sitting but develop low oxygen during walking, so a clinician may use a walk test to document whether oxygen is needed during activity. Never change the prescribed flow rate, smoke, or use open flames near oxygen.
Can pulmonary rehabilitation help with CPFE?
Pulmonary rehabilitation is a supervised exercise and education program for people with lung disease. It can improve strength and exercise tolerance and teach pacing and breathlessness-management techniques.
What should I know about pulmonary hypertension treatment in CPFE?
Pulmonary hypertension in CPFE should be assessed by an experienced ILD/PH team, especially when it is severe. Standard oral pulmonary vasodilators are not routinely used because they can sometimes worsen oxygen levels; a specialist may consider tests such as right-heart catheterization and treatments such as inhaled treprostinil when appropriate.

Questions For You

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Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What is my underlying fibrosis type (like IPF or CTD-ILD), and does it meet the criteria for starting an antifibrotic medication in our region?
  2. 2.Based on my breathing tests and symptoms, which inhaler class (like LAMA or LABA) is most likely to help my specific airflow issues?
  3. 3.What was my lowest oxygen level during my walk test, and at what level should I start using supplemental oxygen?
  4. 4.Do I have any signs of pulmonary hypertension on my echocardiogram, and if so, should I be seen at a specialized ILD/PH center?
  5. 5.Are there specific side effects of nintedanib or pirfenidone I should watch for, and how will we monitor my liver function?

References

References (17)
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    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
  2. 2

    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
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    Combined pulmonary fibrosis and emphysema: a retrospective analysis of clinical characteristics, treatment and prognosis.

    Zhang L, Zhang C, Dong F, et al.

    BMC pulmonary medicine 2016; (16(1)):137 doi:10.1186/s12890-016-0300-7.

    PMID: 27809901
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    HRCT diagnosis of combined pulmonary fibrosis and emphysema in a patient of chronic obstructive pulmonary disease with pulmonary hypertension and clinical or radiograph suspicion of pulmonary fibrosis.

    Manjunath KS, Udnur H

    BJR case reports 2016; (2(4)):20150070 doi:10.1259/bjrcr.20150070.

    PMID: 30460002
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    Clinical efficacy and safety of ICS/LABA in patients with combined idiopathic pulmonary fibrosis and emphysema.

    Dong F, Zhang Y, Chi F, et al.

    International journal of clinical and experimental medicine 2015; (8(6)):8617-25.

    PMID: 26309513
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    Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.

    Raghu G, Remy-Jardin M, Richeldi L, et al.

    American journal of respiratory and critical care medicine 2022; (205(9)):e18-e47 doi:10.1164/rccm.202202-0399ST.

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    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.

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    Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis.

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    European respiratory review : an official journal of the European Respiratory Society 2019; (28(153)) doi:10.1183/16000617.0022-2019.

    PMID: 31578210
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    The Efficacy of Antifibrotics in Combined Pulmonary Fibrosis and Emphysema: A Nationwide Multicentre Retrospective Cohort Study.

    Gwon HR, Lee J, Chung MP, et al.

    Archivos de bronconeumologia 2026; doi:10.1016/j.arbres.2026.08.001.

    PMID: 42760209
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    Pirfenidone as a Cornerstone in the Management of Fibrotic Interstitial Lung Diseases and Its Emerging Applications: A Comprehensive Review.

    Babariya H, Gaidhane SA, Acharya S, Kumar S

    Cureus 2024; (16(9)):e70497 doi:10.7759/cureus.70497.

    PMID: 39479105
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    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
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    Combined Pulmonary Fibrosis and Emphysema (CPFE): A "New" Smoking-Related Interstitial Lung Disease (ILD).

    Afloarei CA, Birladeanu T, Pintilie AL, et al.

    Biomedicines 2025; (13(11)) doi:10.3390/biomedicines13112703.

    PMID: 41301796
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    Chronic Obstructive Pulmonary Disease Combined with Interstitial Lung Disease.

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    Tuberculosis and respiratory diseases 2022; (85(2)):122-136 doi:10.4046/trd.2021.0141.

    PMID: 35385639
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    Combined pulmonary fibrosis and emphysema: effect of pulmonary rehabilitation in comparison with chronic obstructive pulmonary disease.

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    Pulmonary Hypertension With Interstitial Pneumonia: Initial Treatment Effectiveness and Severity in a Japan Registry.

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    Impact of lung morphology on clinical outcomes with riociguat in patients with pulmonary hypertension and idiopathic interstitial pneumonia: A post hoc subgroup analysis of the RISE-IIP study.

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This page is for informational purposes only and does not replace professional medical advice. Your pulmonologist or ILD/PH specialist should guide inhalers, oxygen, antifibrotic medicines, and pulmonary hypertension care.