Cryptogenic organising pneumonia (COP) is a disease of unknown aetiology characterised on imaging by multifocal ground glass opacifications and/or consolidation. A wide variety of infectious as well as noninfectious causes may result in a similar histologic pattern.
Organising pneumonia (OP) is a histological pattern of alveolar inflammation with varied aetiology (including pulmonary infection). The idiopathic form of OP is called cryptogenic organising pneumonia (COP) and it belongs to the idiopathic interstitial pneumonias (IIPs).
COP was previously termed bronchiolitis obliterans organising pneumonia (BOOP), not to be confused with bronchiolitis obliterans 8.
The presentation is commonest in the 55-60 age group.
Patients present with a short history (i.e. less than ~2 months) of breathlessness, non-productive cough, weight loss, malaise and fever. There is no association with smoking.
In addition to the alveolar inflammatory changes found with regular pneumonia, there is also the involvement of the bronchioles.
Histologically, it is characterised by mild chronic patchy interstitial inflammation without fibrosis and the presence of buds of granulation tissue made of mononuclear cells, foamy macrophages, and fibrous tissue (Masson bodies) in the distal airspaces which may cause secondary bronchiolar occlusion due to extension of the inflammatory process. Hence, the reason for being previously termed bronchiolitis obliterans organising pneumonia (BOOP).
- unilateral or bilateral patchy areas (commonest finding 3): often migratory
- can affect all lung zones
- usually peripheral, subpleural, peribronchovascular 2
- foci of granulation tissue up to 1 cm
- may mimic neoplasm if >5 cm in size
- may be numerous in immunocompromised patients
The most common HRCT features include 6:
- patchy consolidation with a predominantly subpleural and/or peribronchial distribution
- small, ill-defined peribronchial or peribronchiolar nodules
- large nodules or masses
- bronchial wall thickening or dilatation in the abnormal lung regions
- a perilobular pattern with ill-defined linear opacities that are thicker than the thickened interlobular septa and have an arcade or polygonal appearance
- ground glass opacity or crazy paving
Treatment and prognosis
Corticosteroids have been widely used and most patients recover completely 3-4.
History and etymology
It was first described by Davison and colleagues in 1983.
On a radiograph consider:
- differential for peripheral consolidation: reverse bat wing opacities
- differential for bilateral airspace opacities
On CT consider:
- 1. McQueen AS, Grant LA, Grant L et-al. Grainger & Allison's Diagnostic Radiology, Single Best Answer Mcqs. Churchill Livingstone. (2009) ISBN:0702031496. Read it at Google Books - Find it at Amazon
- 2. Lee KS, Kullnig P, Hartman TE et-al. Cryptogenic organizing pneumonia: CT findings in 43 patients. AJR Am J Roentgenol. 1994;162 (3): 543-6. AJR Am J Roentgenol (abstract) - Pubmed citation
- 3. Montesinos JJ, Laguna MA. Case 1: Cryptogenic organizing pneumonia. AJR Am J Roentgenol. 1998;171 (3): 835, 838-9. AJR Am J Roentgenol (citation) - Pubmed citation
- 4. Wittram C, Mark EJ, McLoud TC. CT-histologic correlation of the ATS/ERS 2002 classification of idiopathic interstitial pneumonias. Radiographics. 23 (5): 1057-71. doi:10.1148/rg.235035702 - Pubmed citation
- 5. Kim SJ, Lee KS, Ryu YH et-al. Reversed halo sign on high-resolution CT of cryptogenic organizing pneumonia: diagnostic implications. AJR Am J Roentgenol. 2003;180 (5): 1251-4. AJR Am J Roentgenol (full text) - Pubmed citation
- 6. Webb WR, Műller NL, Naidich DP. High-resolution CT of the lung. Lippincott Williams & Wilkins. (2008) ISBN:0781769094. Read it at Google Books - Find it at Amazon
- 7. Cordier JF. Cryptogenic organising pneumonia. Eur. Respir. J. 2006;28 (2): 422-46. doi:10.1183/09031936.06.00013505 - Pubmed citation
- 8. Demedts M, Costabel U. ATS/ERS international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Eur. Respir. J. 2002;19 (5): 794-6. Eur. Respir. J. (link) - Pubmed citation
- 9. Mueller-Mang C, Grosse C, Schmid K et-al. What every radiologist should know about idiopathic interstitial pneumonias. Radiographics. 2007;27 (3): 595-615. Radiographics (full text) - doi:10.1148/rg.273065130 - Pubmed citation
- 10. Lynch DA, Travis WD, Müller NL et-al. Idiopathic interstitial pneumonias: CT features. Radiology. 2005;236 (1): 10-21. Radiology (full text) - doi:10.1148/radiol.2361031674 - Pubmed citation
- 11. Greenberg-Wolff I, Konen E, Ben Dov I et-al. Cryptogenic organizing pneumonia: variety of radiologic findings. Isr. Med. Assoc. J. 2005;7 (9): 568-70. Pubmed citation
- 12. Al-Ghanem S, Al-Jahdali H, Bamefleh H et-al. Bronchiolitis obliterans organizing pneumonia: pathogenesis, clinical features, imaging and therapy review. Ann Thorac Med. 2008;3 (2): 67-75. doi:10.4103/1817-1737.39641 - Free text at pubmed - Pubmed citation
Interstitial lung disease
interstitial lung disease
- drug-induced interstitial lung disease
- hypersensitivity pneumonitis
idiopathic interstitial pneumonia (mnemonic)
- acute interstitial pneumonia (AIP)
- cryptogenic organising pneumonia (COP)
- desquamative interstitial pneumonia (DIP)
- idiopathic non-specific interstitial pneumonia (NSIP)
- idiopathic pleuroparenchymal fibroelastosis
- lymphoid interstitial pneumonia (LIP)
- respiratory bronchiolitis–associated interstitial lung disease (RB-ILD)
- usual interstitial pneumonia / idiopathic pulmonary fibrosis (UIP/IPF)