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Treatments for pulmonary fibrosis

Medications for pulmonary fibrosis

Antifibrotics

Antifibrotics are medicines for pulmonary fibrosis that are thought to work in 2 ways: by reducing inflammation and decreasing the formation of lung scarring. This can slow down the loss of lung function. Antifibrotics can be effective, so they will likely form a key part of your treatment plan. In many countries, there are 2 antifibrotic medicines available: nintedanib and pirfenidone.1

Nintedanib can be used to treat pulmonary fibrosis related to2:

  • Idiopathic pulmonary fibrosis (IPF)
  • Scleroderma/systemic sclerosis-related interstitial lung disease
  • Other progressive fibrosing interstitial lung diseases, including:

    - Rheumatoid arthritis-related interstitial lung disease 

    - Sarcoidosis

    - Hypersensitivity pneumonitis

    - Primary Sjögren’s syndrome-related interstitial lung disease 

Pirfenidone can be used to treat pulmonary fibrosis related to IPF.1

Medications for pulmonary fibrosis

Immunosuppressants

Pills
Immunosuppressants used to treat conditions like rheumatoid arthritis, sarcoidosis, and others include2:
  • Mycophenolate mofetil
  • Cyclophosphamide
  • Methotrexate
  • Rituximab
  • Azathioprine
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Corticosteroids, such as prednisone, may also be given to help reduce inflammation in the lungs.3
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Important

As with all medications, antifibrotics and immunosuppressants both come with manageable side effects, so it's important to speak with your doctor about what to expect and how to manage these issues.

Future treatments being explored: PDE4B inhibitors

Future treatments being explored: PDE4B inhibitors

Scientists continue to look for new ways to treat pulmonary fibrosis.2 One promising area for development is a class of drug called PDE4B inhibitors.4 These medications, which are still in the research phase, work by blocking PDE4B enzymes that can cause inflammation in your body and lead to lung scarring.4

Oxygen therapy can be helpful for many people with pulmonary fibrosis.

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Abbreviations: ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; PDE4B, phosphodiesterase 4B; PF, pulmonary fibrosis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease.

  1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.

  2. Wijsenbeek M, Cottin V. Spectrum of fibrotic lung diseases. N Engl J Med. 2020;383(10):958-968.

  3. Cottin V, Crestani B, Valeyre D, et al. Diagnosis and management of idiopathic pulmonary fibrosis: French practical guidelines. Eur Respir Rev. 2014;23(132):193-214.

  4. Kolb M, Crestani B, Maher TM. Phosphodiesterase 4B inhibition: a potential novel strategy for treating pulmonary fibrosis. Eur Respir Rev. 2023;32:220206.