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PubMed This is a summary of 21 peer-reviewed journal articles Updated
Pulmonology

Treatment Pathways: Medications, Lifestyle, and Transplant

At a Glance

Interstitial Lung Disease (ILD) treatment focuses on slowing disease progression and improving quality of life, rather than curing existing scarring. Key treatments include antifibrotic medications to slow scarring, immunosuppressants to reduce inflammation, oxygen therapy, and pulmonary rehab.

Treating Interstitial Lung Disease (ILD) is not about “curing” the disease in the traditional sense, as existing scar tissue cannot currently be reversed. Instead, the goal of treatment is to stabilize the lungs, slow the progression of further damage, and improve your daily quality of life [1][2]. Your specific treatment path will depend entirely on whether your disease is driven primarily by inflammation or fibrosis (scarring) [3].

The Two Pillars of Medication

Most patients will be prescribed medications from one or both of these categories:

  • Antifibrotics (e.g., Nintedanib, Pirfenidone): These drugs act like a “brake” on the scarring process. They interfere with the chemical signals that tell your body to build more scar tissue [1][4]. While they are proven to slow the annual rate of decline in your lung function, they do not make you feel better immediately [5][6].
    • Side Effects & Logistics: It is important to know that these are heavy specialty drugs. Nintedanib commonly causes severe diarrhea and stomach upset, while Pirfenidone often causes intense sun sensitivity (photosensitivity) and nausea [2]. These side effects are very common but can often be managed through diet changes, supportive medications, or adjusting your dose. Additionally, getting insurance approval for these specialty drugs can take weeks or even months, so patience is required during the initial prescription process.
  • Immunosuppressants (e.g., Mycophenolate Mofetil, Corticosteroids, Rituximab): These are used when an overactive immune system is attacking the lung tissue, which is common in autoimmune-related ILDs (CTD-ILD) [7][8]. By calming the immune system, these drugs reduce the inflammation that eventually leads to permanent scarring [9].

In many cases, especially in Progressive Fibrosing ILD, doctors may use combination therapy, using both an immunosuppressant to stop the “attack” and an antifibrotic to slow down the “scarring” that has already begun [10][11].

Essential Non-Drug Treatments

Medication is only one part of the puzzle. Two other “treatments” are just as critical for your health:

  1. Supplemental Oxygen: If your blood oxygen levels drop (hypoxia), your heart and other organs have to work much harder. Using supplemental oxygen—especially “ambulatory” oxygen used during exercise—can reduce breathlessness, protect your heart, and allow you to stay active [12][13].
  2. Pulmonary Rehabilitation: This is a structured program that combines exercise with breathing techniques and education. It is one of the most effective ways to improve your physical endurance and mental well-being, even if your lung function numbers stay the same [14][15].

Understanding Lung Transplantation

For patients with progressive disease that does not respond to medication, a lung transplant may be an option. It is important to understand that a transplant is a life-changing surgery with its own set of challenges, not a simple “fix” [16].

Transplant centers have rigorous eligibility requirements. Because of the extreme toll of the surgery, patients must generally meet strict age limits (often under 65-70), fall within specific BMI (weight) cutoffs, and have perfectly healthy other organs (like the heart and kidneys) [17][18].

The current international guidelines (ISHLT) recommend early referral for a transplant evaluation [19][20]. This does not mean you are getting a transplant tomorrow; rather, it means the transplant team starts getting to know you while you are still relatively healthy [21].

When is it time to talk about transplant?

  • If your lung function (FVC or DLCO) is declining despite treatment [19].
  • If you require high amounts of supplemental oxygen just to walk or sit [20].
  • Before you experience a “flare-up” or acute exacerbation, as these can make surgery much riskier [19].

Common questions in this guide

Do ILD medications cure the disease?
No, current treatments cannot reverse existing lung scarring. The goal of ILD medications like antifibrotics is to stabilize your lungs, slow down further damage, and improve your daily quality of life.
What is the difference between antifibrotics and immunosuppressants?
Antifibrotics slow down the buildup of lung scar tissue, while immunosuppressants calm an overactive immune system to reduce lung inflammation. Doctors prescribe them based on whether your ILD is driven primarily by scarring or inflammation.
When should I be evaluated for a lung transplant for ILD?
Guidelines recommend an early referral for a lung transplant evaluation so the transplant team can get to know you while you are still relatively healthy. You should discuss this with your doctor if your lung function is declining or if you need high amounts of supplemental oxygen.
How can I manage the side effects of antifibrotic medications?
Common side effects like stomach upset from nintedanib or sun sensitivity from pirfenidone can often be managed through diet changes, protective clothing, and supportive medications. Always communicate with your doctor to adjust doses or find relief strategies.
Why is pulmonary rehabilitation important for ILD?
Pulmonary rehabilitation is a structured program combining exercise with breathing techniques and education. It is one of the most effective ways to improve your physical endurance and mental well-being, even if your actual lung function numbers do not change.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Based on my specific diagnosis, should we be prioritizing an immunosuppressant, an antifibrotic, or a combination of both?
  2. 2.What is the target for my oxygen saturation during exercise, and do I need a prescription for portable oxygen now?
  3. 3.Is there a local pulmonary rehabilitation program you recommend, and can you provide a referral?
  4. 4.At what point in my disease progression (e.g., a specific drop in FVC or DLCO) will you refer me for a lung transplant evaluation?
  5. 5.What are the most common side effects of the medications you are recommending, and how can we manage them (e.g., diet changes for nintedanib or sun protection for pirfenidone)?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (21)
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    Clinical Pharmacokinetics and Pharmacodynamics of Nintedanib.

    Wind S, Schmid U, Freiwald M, et al.

    Clinical pharmacokinetics 2019; (58(9)):1131-1147 doi:10.1007/s40262-019-00766-0.

    PMID: 31016670
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    Interstitial Lung Disease: A Review.

    Maher TM

    JAMA 2024; (331(19)):1655-1665 doi:10.1001/jama.2024.3669.

    PMID: 38648021
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    Current perspective of progressive-fibrosing interstitial lung disease.

    Kishaba T

    Respiratory investigation 2022; (60(4)):503-509 doi:10.1016/j.resinv.2022.03.004.

    PMID: 35431170
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    Consensus document for the diagnosis and treatment of idiopathic pulmonary fibrosis: Joint Consensus of Sociedade Portuguesa de Pneumologia, Sociedade Portuguesa de Radiologia e Medicina Nuclear e Sociedade Portuguesa de Anatomia Patológica.

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    Comparison of the Effects of Nintedanib and Pirfenidone on Pulmonary Function Test Parameters and Radiological Findings in Patients with Idiopathic Pulmonary Fibrosis: A Real-Life Study.

    Aycicek O, Keskin S, Haciosmanoglu M, et al.

    Medicina (Kaunas, Lithuania) 2025; (61(2)) doi:10.3390/medicina61020283.

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    Idiopathic Pulmonary Fibrosis: An Update on Pathogenesis.

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    Frontiers in pharmacology 2021; (12()):797292 doi:10.3389/fphar.2021.797292.

    PMID: 35126134
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    Immunopathogenic Mechanisms in Connective Tissue Disease-Associated Interstitial Lung Disease: Incessant Loop of Immunity to Fibrosis.

    Lee JH, Jang JH, Lee S, Her M

    International journal of molecular sciences 2025; (26(24)) doi:10.3390/ijms262412126.

    PMID: 41465549
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    Treatment of the Connective Tissue Disease-Related Interstitial Lung Diseases: A Narrative Review.

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    Efficacy and safety of pharmacological treatments for autoimmune disease-associated interstitial lung disease: A systematic review and network meta-analysis.

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    Treatment Response and Safety Profile of Nintedanib in Connective Tissue Disease-Associated Interstitial Lung Disease: A Retrospective Observational Study.

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    Mediterranean journal of rheumatology 2025; (36(3)):370-379 doi:10.31138/mjr.080825.lfh.

    PMID: 41122197
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    Efficacy and Safety of Nintedanib in Patients with Connective Tissue Disease-Interstitial Lung Disease (CTD-ILD): A Real-World Single Center Experience.

    Boutel M, Boutou A, Pitsiou G, et al.

    Diagnostics (Basel, Switzerland) 2023; (13(7)) doi:10.3390/diagnostics13071221.

    PMID: 37046439
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    Ambulatory Oxygen for Pulmonary Fibrosis (OxyPuF): a randomised controlled trial and acceptability study.

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    Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial.

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    Indications for Lung Transplantation and Patient Selection.

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    Journal of chest surgery 2022; (55(4)):255-264 doi:10.5090/jcs.22.057.

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    Lung transplantation for interstitial lung disease: evolution over three decades.

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    Airway complications in lung transplant recipients with telomere-related interstitial lung disease.

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This page provides educational information about ILD treatments and lung transplants. Always consult your pulmonologist or transplant team to determine the safest treatment plan for your specific condition.

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