Figure 1
Conventional chest radiography.
Cryptogenic organizing “non resolving” pneumonia: a recurrent disease.
SectionChest imaging
Case TypeClinical Cases
AuthorsParra-Fariñas C, Salcedo MT, Dyer Hartnett S, Comet R.
Connected authors
71 years, female
Clinical History
A healthy patient presented with a 2-week history of fever, shortness of breath and a non-productive cough. Physical examination revealed crackles over the lower right hemithorax.
Serology for viral agents, blood and sputum cultures, immunological and tumour markers were all within normal range.
The initial treatment was antibiotics for the clinical suspicion of pneumonia, but symptoms persisted.
Serology for viral agents, blood and sputum cultures, immunological and tumour markers were all within normal range.
The initial treatment was antibiotics for the clinical suspicion of pneumonia, but symptoms persisted.
Imaging Findings
Chest x-ray followed by high resolution computed tomography (HRCT) were performed. The HRCT abnormalities mirror the radiographic appearances showing bilateral patchy consolidations with groundglass opacities, air bronchogram and bronchial dilatations in a peripheral distribution mainly in the right lung (Fig. 1, 2).
Bronchoalveolar lavage specimens were smear and culture negative. Lung biopsy was undertaken showing distal airways filled by fibromyxoid plugs of granulation tissue (Fig. 3).
High doses of oral corticosteroid were commenced with dramatic clinical improvement after 72-hours and near complete resolution of consolidations by the 10th day (Fig. 4).
On follow-up, steroid dose was decreased, but 1 month later, fever recurred and consolidations reappeared (Fig. 5). Steroid dose was increased and complaints disappeared.
Oral corticosteroids were gradually tapered down and the patient was asymptomatic for 4 months. Last HRCT showed an almost complete disappearance of lesions, although ground glass densities still persisted, mainly in the left upper lobe (Fig. 6).
Bronchoalveolar lavage specimens were smear and culture negative. Lung biopsy was undertaken showing distal airways filled by fibromyxoid plugs of granulation tissue (Fig. 3).
High doses of oral corticosteroid were commenced with dramatic clinical improvement after 72-hours and near complete resolution of consolidations by the 10th day (Fig. 4).
On follow-up, steroid dose was decreased, but 1 month later, fever recurred and consolidations reappeared (Fig. 5). Steroid dose was increased and complaints disappeared.
Oral corticosteroids were gradually tapered down and the patient was asymptomatic for 4 months. Last HRCT showed an almost complete disappearance of lesions, although ground glass densities still persisted, mainly in the left upper lobe (Fig. 6).
Discussion
Organizing pneumonia or its idiopathic form, cryptogenic organizing pneumonia (COP) is a clinic-pathologic entity described by Davison and colleagues in 1983 [1]. Organizing pneumonia was formerly known as bronchiolitis obliterans organizing pneumonia (BOOP), a term coined by Epler and co-workers in 1985 [2].
The term COP is preferred because it conveys the essential features of the syndrome [polypoid granulation tissue within alveolar ducts and alveoli with or without organization within bronchioles (“polypoid bronchiolitis obliterans”)] and avoids confusion with airway diseases such as constrictive bronchiolitis obliterans [2-4].
COP is a type of interstitial pulmonary disease included in the classification of the American Thoracic Society and European Respiratory Society statement on the classification of idiopathic interstitial pneumonias (IIP) in the group of major IIP [5].
The true incidence of COP is unknown (1-7/100.000) [6]. Similarly affects men and women in fifth and sixth decade of life [7], mostly in non-smokers.
A short-history (median: <3 months) of dyspnoea, cough and fever is the most common form of presentation [5, 7, 8]. Haemoptysis and pleurisy could be seen too. Because of these symptoms a chest x-ray followed by HRCT are usually the first imaging methods to try to establish the diagnosis. The relatively non-specific radiological findings and the absence of response to traditional treatment lead to additional studies such as bronchoalveolar lavage and open biopsy.
The most important radiological findings are bilateral and asymmetric consolidations commonly associated with ground-glass densities with a peripheral and peribronchovascular distribution, predominating in middle and lower lobes, which may change their location [8-12].
Less frequent manifestations are solitary or multiple nodules or masses [11, 12], reverse (atoll) halo sign [10, 15], thick bands of consolidation extending towards the pleura and crazy-paving pattern [13].
The diagnosis of COP is established by combining clinical, radiological and histological criteria.
Initial therapy depends on severity of symptoms, pulmonary function tests and radiological extension. The majority of patients recover completely with corticosteroid treatment but also relapse. Fibrosis is common with similar findings to those seen in non-specific interstitial pneumonias [5, 13, 14, 16].
COP is a form of IIP that can be easily mistaken for both clinical and radiological lung diseases.
•Non-specific pattern of reparative response of lung to the injury to a non-identifiable cause.
•Should be considered in the differential diagnosis of patients with short-history of breathlessness, cough and fever.
•Typical radiologic manifestations: patchy consolidations and ground-glass opacities that may change their location, resolve without treatment and reappear.
•Fast corticosteroid response with a good prognosis.
The term COP is preferred because it conveys the essential features of the syndrome [polypoid granulation tissue within alveolar ducts and alveoli with or without organization within bronchioles (“polypoid bronchiolitis obliterans”)] and avoids confusion with airway diseases such as constrictive bronchiolitis obliterans [2-4].
COP is a type of interstitial pulmonary disease included in the classification of the American Thoracic Society and European Respiratory Society statement on the classification of idiopathic interstitial pneumonias (IIP) in the group of major IIP [5].
The true incidence of COP is unknown (1-7/100.000) [6]. Similarly affects men and women in fifth and sixth decade of life [7], mostly in non-smokers.
A short-history (median: <3 months) of dyspnoea, cough and fever is the most common form of presentation [5, 7, 8]. Haemoptysis and pleurisy could be seen too. Because of these symptoms a chest x-ray followed by HRCT are usually the first imaging methods to try to establish the diagnosis. The relatively non-specific radiological findings and the absence of response to traditional treatment lead to additional studies such as bronchoalveolar lavage and open biopsy.
The most important radiological findings are bilateral and asymmetric consolidations commonly associated with ground-glass densities with a peripheral and peribronchovascular distribution, predominating in middle and lower lobes, which may change their location [8-12].
Less frequent manifestations are solitary or multiple nodules or masses [11, 12], reverse (atoll) halo sign [10, 15], thick bands of consolidation extending towards the pleura and crazy-paving pattern [13].
The diagnosis of COP is established by combining clinical, radiological and histological criteria.
Initial therapy depends on severity of symptoms, pulmonary function tests and radiological extension. The majority of patients recover completely with corticosteroid treatment but also relapse. Fibrosis is common with similar findings to those seen in non-specific interstitial pneumonias [5, 13, 14, 16].
COP is a form of IIP that can be easily mistaken for both clinical and radiological lung diseases.
•Non-specific pattern of reparative response of lung to the injury to a non-identifiable cause.
•Should be considered in the differential diagnosis of patients with short-history of breathlessness, cough and fever.
•Typical radiologic manifestations: patchy consolidations and ground-glass opacities that may change their location, resolve without treatment and reappear.
•Fast corticosteroid response with a good prognosis.
Differential Diagnosis List
Cryptogenic organizing pneumonia (COP).
Community acquired pneumonia.
Adenocarcinoma in situ or minimally invasive (bronchoalveolar carcinoma).
Pulmonary lymphoma.
Pulmonary vasculitides.
Sarcoidosis.
Chronic eosinophilic pneumonia.
Other idiopathic interstitial pneumonias.
Final Diagnosis
Cryptogenic organizing pneumonia (COP).
References
[1] Davison AG, Heard BE, McAllister WAC, Turner-Warwick ME (1983) Cryptogenic organizing pneumonitis. Q J Med 207:382–394 (PMID: 6647749)
[2] Epler GR, Colby TV, McLoud TC, Carrington CB, Gaensler EA (1985) Bronchiolitis obliterans organizing pneumonia. N Engl J Med 312:152–158 (PMID: 3965933)
[3] Kitaichi M (1994) Bronchiolitis obliterans organizing pneumonia (BOOP). In: Takishima T, editor. Basic and Clinical Aspects of Pulmonary Fibrosis. Boca Raton, FL: CRC Press 463-488
[4] Kitaichi M (1992) Differential diagnosis of bronchiolitis obliterans organizing pneumonia. Chest 102:44S–49S (PMID: 1623810)
[5] Travis WD, Costabel U, Hansell DM, et al (2013) An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 188:733-48 (PMID: 24032382)
[6] Gudmundsson G, Sveinsson O, Isaksson HJ, Jonsson S, Frodadottir H, Aspelund T (2006) Epidemiology of organising pneumonia in Iceland. Thorax 61:805-8 (PMID: 16809413)
[7] Drakopanagiotakis F, Paschalaki K, Abu-Hijleh M, et al (2011) Cryptogenic and secondary organizing pneumonia. Clinical presentation, radiographic findings, treatment response, and prognosis. Chest 139:893-900 (PMID: 20724743)
[8] Webb WR, Müller NL, Naidich DP (2015) High-resolution CT of the lung. 5th Ed. Wolters Kluver, Philadephia 232-9
[9] Baque-Juston M, Pellegrin A, Leroy S, Marquette CH, Padovani B (2014) Organizing pneumonia: What is it? A conceptual approach and pictorial review. Diagnostic and interventional imaging 95:771-7 (PMID: 24559802)
[10] Kim SJ, Lee KS, Ryu YH, Yoon YC, Choe KO, Kim TS, Sung KJ. King TE (2003) Reversed halo sign on high-resolution CT of cryptogenic organizing pneumonia: diagnostic implications. AJR Am J Roentgenol 180:1251-4 (PMID: 12704033)
[11] Muller NL, Colby TV (1997) Idiopathic interstitial pneumonias: high resolution CT and histologic findings. Radiographics 17:1016-1022 (PMID: 9225401)
[12] Haddock JAA, Hansell DM (1992) The radiology and terminology of cryptogenic organizing pneumonia. Br J Radiol 65:674-680 (PMID: 1393393)
[13] Oikonomou A, Hansell DM (2002) Organizing pneumonia: the many morphological faces. Eur Radiol 12:1486-96 (PMID: 12042959)
[14] Roberton BJ, Hansell DM (2011) Organizing pneumonia: a kaleidoscope of concepts and morphologies. Eur Radiol 21:2244-54 (PMID: 21744289)
[15] Marchiori E, Zanetti G, Hochhegger B, Irion KL, Carvalho AC, Godoy MC (2012) Reversed halo sign on computed tomography: state-of-the-art review. Lung 190:389-94 (PMID: 22573292)
[16] Barroso E, Hernández L, Gil J, García R, Aranda I, Romero S (2007) Idiopathic organizing pneumonia: a relapsing disease. 19 years of experience in a hospital setting. Respiration 74:624-31 (PMID: 17536184)
Case information
| URL: | https://www.eurorad.org/case/12943 |
| DOI: | 10.1594/EURORAD/CASE.12943 |
| ISSN: | 1563-4086 |
License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Figure 2
Axial high resolution computed tomography of the lungs.
Axial HRCT showed consolidations with ground-glass opacities, air bronchogram and bronchial dilatation predominantly peripheral and subpleural distribution in whole lobes and segments of both lungs mainly in the right lung.
Figure 3
Micrographs of lung tissue stained with haematoxylin and eosin.
Histological picture of video-assisted thoracoscopic surgery lung biopsy sample with haematoxylin and eosin stain showed polypoid granulation tissue within bronchioles, alveolar ductus and alveoli (arrow).
The specimens from the right lower and middle lobes showed preservation of the lung architecture with intra-alveolar plugs of fibrin (arrow), associated with organizing pneumonia (intraluminal loose connective tissue within the alveolar ducts and bronchioles).
Infiltration of airways and interstitium with mononuclear cells and foamy macrophages accompanies granulation tissue within distal airways (arrow).
Figure 4
Axial high resolution computed tomography of the lungs.
Axial HRCT after 10 days of continuous high dose corticosteroid therapy showed important diminution of the consolidation areas in both lungs.
Figure 5
Axial high resolution computed tomography of the lungs.
One month follow-up after tapering steroid dose, HRCT showed ground-glass densities that become more widely spread and new ground-glass opacities were seen at the upper lobes bilaterally predominance in left superior lobe.
Figure 6
Axial high resolution computed tomography of the lungs.
Four months follow-up after increasing the steroid dose, HRCT showed an almost complete disappearance of lesions, although ground-glass densities still persisted, mainly in the left upper lobe.
Conventional chest radiography.
Posteroanterior conventional chest x-ray revealed bilateral patchy air-space consolidations.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
Left lateral chest x-ray showed areas of consolidation with a lower predominance.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
Axial high resolution computed tomography of the lungs.
Axial HRCT showed consolidations with ground-glass opacities, air bronchogram and bronchial dilatation predominantly peripheral and subpleural distribution in whole lobes and segments of both lungs mainly in the right lung.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
Micrographs of lung tissue stained with haematoxylin and eosin.
Histological picture of video-assisted thoracoscopic surgery lung biopsy sample with haematoxylin and eosin stain showed polypoid granulation tissue within bronchioles, alveolar ductus and alveoli (arrow).
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
The specimens from the right lower and middle lobes showed preservation of the lung architecture with intra-alveolar plugs of fibrin (arrow), associated with organizing pneumonia (intraluminal loose connective tissue within the alveolar ducts and bronchioles).
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
Infiltration of airways and interstitium with mononuclear cells and foamy macrophages accompanies granulation tissue within distal airways (arrow).
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
Axial high resolution computed tomography of the lungs.
Axial HRCT after 10 days of continuous high dose corticosteroid therapy showed important diminution of the consolidation areas in both lungs.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
Axial high resolution computed tomography of the lungs.
One month follow-up after tapering steroid dose, HRCT showed ground-glass densities that become more widely spread and new ground-glass opacities were seen at the upper lobes bilaterally predominance in left superior lobe.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
Axial high resolution computed tomography of the lungs.
Four months follow-up after increasing the steroid dose, HRCT showed an almost complete disappearance of lesions, although ground-glass densities still persisted, mainly in the left upper lobe.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.
2015 Department of Radiology, Vall d´Hebron Hospital. Barcelona, Spain.