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Pulmonary fibrosis

Last revised by Yaïr Glick ◉ on 8 Oct 2024

Citation, DOI, disclosures and article data

Citation:

Weerakkody Y, Glick Y, Silverstone L, et al. Pulmonary fibrosis. Reference article, Radiopaedia.org (Accessed on 19 Jan 2025) https://doi.org/10.53347/rID-22678

DOI:

https://doi.org/10.53347/rID-22678

Permalink:

https://radiopaedia.org/articles/22678

rID:

22678

Article created:

15 Apr 2013, Yuranga Weerakkody ◉

Disclosures:

At the time the article was created Yuranga Weerakkody had no recorded disclosures.

View Yuranga Weerakkody's current disclosures

Last revised:

8 Oct 2024, Yaïr Glick ◉

Disclosures:

At the time the article was last revised Yaïr Glick had no financial relationships to ineligible companies to disclose.

View Yaïr Glick's current disclosures

Revisions:

37 times, by 14 contributors - see full revision history and disclosures

Systems:

Chest

Sections:

Gamuts

Synonyms:

Lung fibrosis
Fibrosis of the lung
Fibrosis of lungs
Fibrosis of lung
Fibrosis of the lungs
Scarring of the lung
Scarring of the lungs
Scarring of lung
Interstitial fibrosis - lung
Interstitial fibrosis
Fibrosing lung disease

Pulmonary fibrosis is the preferred general term for the permanent replacement of lung parenchyma by connective tissue and is typically associated with functional impairment. A variety of insults cause focal or diffuse lung injury (mechanical, infectious, inflammatory, and iatrogenic). Lung repair culminates in fibrosis with volume loss and architectural distortion.

significant acute insult to the lungs
- adult respiratory distress syndrome ⁴
- from a significant pulmonary infection
  - Post COVID fibrosis, especially in patients admitted to ICU and needing intubation/ventilation
- diffuse alveolar damage from any source
inhaled substances
- coal/silica: progressive massive fibrosis
- asbestos: asbestos-related pulmonary fibrosis
chronic microaspiration ⁹
radiation: radiation-induced pulmonary fibrosis
congenital conditions
- cystic fibrosis
- Hermansky-Pudlak syndrome
autoimmune conditions
connective tissue disorders
granulomatous conditions
- sarcoidosis: pulmonary manifestations of sarcoidosis
- tuberculosis: pulmonary manifestations of tuberculosis
- granulomatosis with polyangiitis: pulmonary manifestations of granulomatosis with polyangiitis
others
- airway-centered interstitial fibrosis
- chronic conditions
  - chronic hypersensitivity pneumonitis
  - chronic eosinophilic pneumonia
- polymyalgia rheumatica (occasional case reports ⁶)
- medications: drug-induced lung disease
- combined pulmonary fibrosis and emphysema'
- aging
  - some studies report thin-section CT findings associated with interstitial lung disease to some degree are frequently seen in "asymptomatic" elderly individuals ^7,8
- idiopathic pulmonary fibrosis

Radiographic features

CT

Early pulmonary fibrosis ¹²:

irregular interlobular septal thickening
irregular pleural thickening
intralobular reticular opacity

This can progress with increasing reticulation and

traction bronchiectasis and bronchiolectasis
honeycombing
architectural distortion

Treatment and prognosis

Antifibrotic medication provides benefit for patients with progressive pulmonary fibrosis ¹².

Quiz questions

References

1. Noble PW, Barkauskas CE, Jiang D. Pulmonary fibrosis: patterns and perpetrators. J. Clin. Invest. 2012;122 (8): 2756-62. J. Clin. Invest. (abstract) - doi:10.1172/JCI60323 - Free text at pubmed - Pubmed citation
2. Collins J, Stern EJ. Chest Radiology. Lippincott Williams & Wilkins. (2008) ISBN:0781763142. Read it at Google Books - Find it at Amazon
3. Schwartz MI, King TE. Interstitial Lung Disease, 5e. pmph usa. (2010) ISBN:1607950243. Read it at Google Books - Find it at Amazon
4. Marshall R, Bellingan G, Laurent G. The acute respiratory distress syndrome: fibrosis in the fast lane. Thorax. 1999;53 (10): 815-7. Free text at pubmed - Pubmed citation
5. Elicker BM, Webb WR. Fundamentals of High-Resolution Lung Ct. Lippincott Williams & Wilkins. (2013) ISBN:1451184085. Read it at Google Books - Find it at Amazon
6. Shimada T, Matsumura K, Higashi K et-al. Pulmonary fibrosis associated with polymyalgia rheumatica. Intern. Med. 1997;36 (11): 837-40. Pubmed citation
7. Susan J. Copley, Athol U. Wells, Katherine E. Hawtin, Daren J. Gibson, James M. Hodson, Audrey E. T. Jacques, David M. Hansell. Lung Morphology in the Elderly: Comparative CT Study of Subjects over 75 Years Old versus Those under 55 Years Old1. (2009) Radiology. 251 (2): 566-73. doi:10.1148/radiol.2512081242 - Pubmed
8. Winter DH, Manzini M, Salge JM, Busse A, Jaluul O, Jacob Filho W, Mathias W, Terra-Filho M. Aging of the lungs in asymptomatic lifelong nonsmokers: findings on HRCT. (2015) Lung. 193 (2): 283-90. doi:10.1007/s00408-015-9700-3 - Pubmed
9. Cardasis J, MacMahon H, Husain A. The Spectrum of Lung Disease Due to Chronic Occult Aspiration. Annals ATS. 2014;11(6):865-73. doi:10.1513/annalsats.201310-360oc - Pubmed
10. Shah R, Kolansky A, Kligerman S. Thin-Section CT in the Categorization and Management of Pulmonary Fibrosis Including Recently Defined Progressive Pulmonary Fibrosis. Radiology: Cardiothoracic Imaging. 2024;6(1):e230135. doi:10.1148/ryct.230135 - Pubmed
11. Bankier A, MacMahon H, Colby T et al. Fleischner Society: Glossary of Terms for Thoracic Imaging. Radiology. 2024;310(2):e232558. doi:10.1148/radiol.232558 - Pubmed
12. Chae K, Hwang H, Duarte Achcar R et al. Central Role of CT in Management of Pulmonary Fibrosis. Radiographics. 2024;44(6):e230165. doi:10.1148/rg.230165 - Pubmed

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Pulmonary fibrosis

Citation, DOI, disclosures and article data

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Terminology

Pathology

Radiographic features

CT

Treatment and prognosis

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