Cryptogenic Organizing Pneumonia Diagnosis Approach Based on a Clinical-Radiologic-Pathologic Consensus

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1 Cryptogenic Organizing Pneumonia Diagnosis Approach Based on a Clinical-Radiologic-Pathologic Consensus Poster No.: C-1622 Congress: ECR 2012 Type: Scientific Exhibit Authors: C. Cordero Lares, E. Zorita Argüero, I. Herráez Ortega, A. Fuentes Morán, L. López González; León/ES Keywords: Thorax, Lung, CT-High Resolution, Imaging sequences, Inflammation DOI: /ecr2012/C-1622 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 20

2 Purpose The diagnostic approach to Idiopathic Interstitial Pneumonias (IIPs) is complex, due to the existence of several classifications according to the clinical, radiologic and histologic criteria. The American Thoracic Society and the European Respiratory Society convened a multidisciplinary classification in June 2001, based on a clinical-radiologic-pathologic consensus (Table 1), which contains seven disease entities, including the cryptogenic organizing pneumonia (COP). COP is a rare pulmonary disease and frequently idiopathic. The diagnosis is based on characteristic clinical and radiologic features, with the presence of granulation tissue polyps in the alveolar ducts and alveoli. Often there is a patchy interstitial infiltrate with preservation of lung architecture. Adequate diagnosis has become important, because most patients with COP show radiological improvement or resolution after treatment. The objectives of this study are: 1) Review the characteristic High Resolution Computed Tomography (HRCT) patterns in patients with COP. 2) Correlate COP radiologic diagnosis with a clinical-radiologic-pathologic consensus. 3) Evaluate radiologic improvement of COP after steroid treatment. Images for this section: Page 2 of 20

3 Table 1 Page 3 of 20

4 Methods and Materials Retrospective study of patients with COP diagnosis, performed between Thirty two patients were studied with HRCT. COP diagnosis was done in twenty five patients based on a clinical-radiologic-pathologic consensus and confirmed by transbronchial biopsy. In seven patients a clinical-radiologic COP diagnosis was made with a not conclusive biopsy. Clinical evaluation: The initial approach began with a careful history followed by physical examination, routine chest radiographs, and pulmonary function testing. The assessment of the clinical history included first symptoms, their progression and clinical course. This clinical evaluation allows establishing an initial IIPs clinical suspicion therefore identifying patients that require further studies. Radiological methods: HRCT has become an integral part of the valuation of the patient with IIPs. HRCT technique protocol used during the study was an helical inspiration scan performed with 0.6 mm collimation and high resolution reconstruction kernel. Additional sequential expiratory scans were performed at the aortic arch, pulmonary hilum and basal pulmonary segments, required to distinguish between air trapping and other causes of altered lung attenuation. Interpreting the HRCT of a patient with diffuse lung disease, should determine the presence or absence of a typical pattern. The presence of typical clinical and HRCT findings of COP, is sufficiently characteristic to allow a confident diagnosis. HRCT typical features include: Page 4 of 20

5 -Airspace consolidation with a subpleural or peribronchial distribution, frequently involving lower lung zones. -Ground-glass attenuation. -Small nodules, usually centrilobular. -Mild cylindrical bronchial dilatation in areas of consolidation. Radiologic reports were reviewed and a descriptive analysis of the different findings was made, correlating the radiologic features with the consensus diagnosis. In all patients a HRCT was performed one year after initial COP diagnosis, for outcome follow-up. Pathological methods: Transbronchial biopsy was guided by HRTC findings, locating the most affected areas. Specimens obtained by transbronchial biopsy were prepared and hematoxylin and eosin staining was performed. Anatomopathological diagnosis was based on the histological criteria established in the American Thoracic/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias (Table 2). Images for this section: Page 5 of 20

6 Table 2 Page 6 of 20

7 Results Patients with COP have a mean age of years. Men and women are often equally affected. The typical symptoms were dyspnea, cough and fever with no response to antibiotic treatment. COP is characterized by the presence of consolidation on chest radiographs, in 94.7 % of patients. HRCT findings were: -Consolidation % (Fig.1-2). -Ground-glass opacities % (Fig ). -Bronchiectasias - 50% (Fig.6). -Small peribronchial nodules - 35% (Fig. 7-8). -Crazy Paving pattern - 21% (Fig.9). The most frequent pattern was consolidation in 78.9%, fallowed by ground-glass opacity in 63.2% of patients. The lower lung lobes were involved in 67.4% of patients. Lung abnormalities show a characteristic peripheral and peribronchial distribution in 57.9% of cases (Fig. 10). Pleural effusion occurred in 37% of cases (Fig. 11). Lymphadenopathies were observed in 58% of patients. Lung architectural distortion was seen in 37% of cases. In 74% of cases the first diagnosis possibility that arises in radiological report is NOC coinciding with consensus diagnosis. In all cases where the radiological diagnosis of NOC was performed with a high degree of confidence (14 cases, 43.7% ) Page 7 of 20

8 the consensus diagnosis was NOC. The main differential diagnosis were Chronic Eosinophilic Pneumonia and Nonspecific Interstitial Pneumonia. 90% of patients under steroid treatment showed radiologic improvement in HRCT follow up. In 10% of patients who did not received steroid treatment original consolidations disappeared but new ones appeared in other locations. Images for this section: Fig. 1: Transverse HRCT images show sharply demarcated consolidations with air bronchogram and peribronchovascular distribution. Page 8 of 20

9 Fig. 2: Transverse HRCT images show patchy, peribronchovascular and subpleural consolidations associated with ground-glass opacities. Page 9 of 20

10 Fig. 3: Transverse HRCT image shows focal ground-glass opacification. Page 10 of 20

11 Fig. 4: Transverse HRCT image shows bilateral pulmonary consolidations (peribronchovascular and subpleural predominance), with associated ground-glass opacities. Page 11 of 20

12 Fig. 5: Transverse HRCT image shows bilateral pulmonary consolidations (peribronchovascular and subpleural predominance), with associated ground-glass opacities. Page 12 of 20

13 Fig. 6: Transverse HRCT images show lower lobe ground-glass opacity with associated reticular abnormality and traction bronchiectasis. Page 13 of 20

14 Fig. 7: Transverse HRCT images show poorly defined centrilobular small nodules. Page 14 of 20

15 Fig. 8: Transverse HRCT images show poorly defined centrilobular small nodules. Page 15 of 20

16 Fig. 9: Crazy Paving Pattern. Transverse HRCT images show geographic areas of ground-glass opacity and subtle septal thickening. Page 16 of 20

17 Fig. 10: Coronal HRCT image shows patchy, peribronchovascular and subpleural consolidations associated with ground-glass opacities, affecting predominantly both lower lobes. Page 17 of 20

18 Fig. 11: Transverse HRCT image shows bilateral pulmonary consolidation with subpleural and peribronchovascular predominance and right pleural effusion (arrows). Page 18 of 20

19 Conclusion HRCT is a useful tool for COP diagnosis an outcome follow-up. The most common HRCT features of COP constitute consolidations with a subpleural and/or peribronchial distribution. Air bronchogram is a consistent finding when consolidation is present. Usually consolidations are associated with ground-glass opacities. HRTC demonstrates a good correlation with the clinical-radiologic-pathologic consensus, specially when COP diagnosis is made with a high confidence level. Patients with COP demonstrate radiographic improvement or resolution after steroid treatment. References 1. Demedts M, Costabel U. ATS/ERS international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Eur Respir J 2002; 19: Ujita M, Renzoni E, Wells A, Hansell DM. Organizing Pneumonia: Perilobular Pattern at Thin-Section CT. Radiology 2004; 232: Schlesinger C, Koss MN. The organizing pneumonias: an update and review. Curr Opin Pulm Med. 2005; 11:422: Lynch D, Travis W, Müller N, et al. Idiopathic Interstitial Pneumonias: CT Features. Radiology 2005; 236: Cordier JF. Cryptogenic organising pneumonia. Eur Respir J 2006; 28: Mueller-Mang C, Grosse C, Schmid K, et al. What Every Radiologist Should Know about Idiopathic Interstitial Pneumonias. RadioGraphics 2007: 27: Page 19 of 20

20 7. Ryu JH, Maldonado F. Focal organizing pneumonia on surgical lung biopsy: causes, clinicoradiologic features and outcomes. Chest, 2007; 132: Kligerman S, Groshong S, Brown K, et al. Nonspecific Interstitial Pneumonia: Radiologic, Clinical, and Pathologic Considerations. RadioGraphics 2009; 29: Kane GC, Vasu TS. Clinical and radiologic distinctions between secondary bronchiolitis obliterans organizing pneumonia and cryptogenic organizing pneumonia. Respir Care. 2009; 54: Lee JW. Cryptogenic organizing pneumonia: serial high-resolution CT findings in 22 patients. AJR Am J Roentgenol. 2010; 195: Personal Information Page 20 of 20

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