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Pulmonary Fibrosis

Pulmonary fibrosis is a group of lung conditions in which the lung tissue becomes scarred and stiffened, making it harder for the lungs to work properly. Idiopathic pulmonary fibrosis (IPF) is the most common form.[1]

Common symptoms

  • Progressive shortness of breath, initially with exertion, eventually at rest
  • Dry, persistent cough
  • Fatigue and weakness
  • Unintentional weight loss
  • Fingertip clubbing (widening and rounding of fingertips)
  • "Velcro-like" crackles heard by a clinician on lung examination[1]

Types and causes

Idiopathic pulmonary fibrosis (IPF) occurs without an identifiable cause, typically in older adults. It is the most common and most studied form.[1]

Other causes of lung fibrosis include autoimmune diseases (e.g., rheumatoid arthritis, scleroderma), environmental or occupational exposures (e.g., asbestos, silica, mold), certain medications, and radiation therapy.

Progressive pulmonary fibrosis (PPF) refers to any non-IPF interstitial lung disease that worsens over time despite treatment.[1]

Evaluation

How is pulmonary fibrosis diagnosed?

Diagnosis involves clinical history, physical examination, lung function tests, imaging, and sometimes lung biopsy.[1]

Imaging
  • High-resolution CT (HRCT) of the chest: The most important imaging test. Can identify specific patterns of fibrosis, such as usual interstitial pneumonia (UIP), which is the hallmark pattern of IPF.[1]
  • In many cases, a confident diagnosis of IPF can be made from HRCT alone without the need for a lung biopsy.
  • Chest X-ray: May show lung scarring but is less sensitive and specific than HRCT.
Lung function and other tests
  • Spirometry: Typically shows a restrictive pattern (reduced FVC with a preserved or elevated FEV1/FVC ratio).[2]
  • Diffusing capacity (DLCO): Often reduced, indicating impaired gas exchange across the scarred lung tissue. A key marker of disease severity.
  • 6-minute walk test: Assesses exercise capacity and oxygen desaturation with walking. Used to monitor progression over time.
  • Blood tests: To evaluate for autoimmune causes (ANA, RF, anti-CCP, myositis panel) and other treatable etiologies.
  • Lung biopsy: Transbronchial lung cryobiopsy or surgical lung biopsy may be needed when imaging is indeterminate. The 2022 guideline recognizes cryobiopsy as an acceptable alternative to surgical biopsy in centers with appropriate expertise.[1]
Treatment

How is pulmonary fibrosis treated?

Treatment depends on the type and cause of fibrosis. For IPF specifically, antifibrotic medications can slow progression.[1]

Antifibrotic therapy

  • Pirfenidone: An antifibrotic medication that has been shown to slow the decline in lung function in IPF.[1]
  • Nintedanib: Another antifibrotic medication with demonstrated benefit in slowing FVC decline in both IPF and progressive pulmonary fibrosis.[1]
  • These medications do not cure fibrosis or reverse existing scarring, but they can slow the rate of progression.
  • Side effects may include gastrointestinal symptoms (nausea, diarrhea) and liver enzyme changes.

Supportive and other treatments

  • Supplemental oxygen: For patients with low oxygen levels at rest or with activity.
  • Pulmonary rehabilitation: Improves exercise tolerance, reduces dyspnea, and supports quality of life. Learn more →
  • Immunosuppressive therapy: May be appropriate for fibrosis caused by autoimmune disease, but is not recommended for IPF.
  • Lung transplant evaluation: For eligible patients with advanced disease and declining function despite treatment.
  • Cough management: Chronic cough in fibrosis is common and often difficult to treat. Options include medication trials and speech therapy techniques.
  • Palliative care: Focuses on symptom relief, quality of life, and advance care planning — appropriate at any stage, not only end-of-life.

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Related topics

References

  1. Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022;205(9):e18-e47. doi:10.1164/rccm.202202-0399ST
  2. Graham BL, Steenbruggen I, Miller MR, et al. Standardization of spirometry 2019 update: an official American Thoracic Society and European Respiratory Society technical statement. Am J Respir Crit Care Med. 2019;200(8):e70-e88. doi:10.1164/rccm.201908-1590ST