When to suspect Wegener Granulomatosis: A radiologic review Poster No.: P-0038 Congress: ESTI 2015 Type: Educational Poster Authors: A. Tilve Gómez, R. Díez Bandera, P. Rodríguez Fernández, M. Garcia Vazquez-Noguerol, E. Chavarri Ibañez, R. Mendez, A. Illade Fornos, J. Vieito; Vigo/ES Keywords: DOI: Inflammation, Hemorrhage, Education, CT, Thorax, Lung 10.1594/esti2015/P-0038 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. www.myesr.org Page 1 of 20
Learning objectives Describe the thoracic radiological findings of Wegener Granulomatosis from 9 cases of our institution. We will define this entity, its pathogenesis, clinical features, diagnosis criteria, empathizing in the chest radiographic and CT findings. Background Wegener granulomatosis, now called granulomatosis with polyangiitis is an uncommon condition characterized by granulomatous necrotizing vasculitis that involves mainly small an medium-sized vessels. Its cause remains unknown, but an autoimmune origin has been proposed because of the presence of ANCAs (mainly PR3 and MPO). Males and females are affected equally and mean age at diagnosis is 50 Classical clinical triad consists of upper and lower respiratory tract involvement and glomerulonephritis, although any organ system can be affected. The diagnosis is based on a combination of clinical features, radiologic and laboratory findings and biopsy. At presentation upper airway involvement with sinusitis, rhinitis, and otitis is the most common clinical feature. Lung symptoms (cough, dyspnea, wheezing, hemoptysis or pleuritic pain) are present in nearly 90%. Functional renal impairment is unusual at presentation, being seen in only about 10%-40% of patients, 80-90% ultimately develop renal disease. Page 2 of 20
When thorax is involved, plain x-ray used to be the first diagnosis test followed by computed tomography (CT), which contributes to an accurate evaluation of the airway, lung parenchyma and mediastinum. Imaging findings OR Procedure details Chest x-ray findings: The most characteristic pulmonary finding is discrete focal opacities that vary in character from nodules or masses to ill-defined areas of consolidation, either of which may cavitate. In our series the most frequent finding at the chest X-rasy were pulmonary nodules Fig. 1 on page 4, focal opacities Fig. 2 on page 5 and consolidation Fig. 3 on page 6. CT common manifestations: 1.- Pulmonary nodules and masses: Single or multiple with random distribution. Waxing and waning are features of the disease. Cavitation occurs in up to 50% (>2cm). Halo-sing or atoll-sign can be seen around, due to pulmonary haemorrhage. In our study lung nodules were the most frequent manifestation present in the 77,7% Fig. 4 on page 13, finding cavitation in 33,3% Fig. 5 on page 11, Fig. 6 on page 9 and atoll-sing in 33,3% Fig. 7 on page 7. 2.- Ground-glass opacity and Consolidations: Ground-glass opacities have variable distribution but basically perihilar and peribronchovascular. Consolidations are usually dense and may contain air bronchograms. 22,2% of our patients showed consolidations Fig. 8 on page 12 and ground-glass opacities Fig. 9 on page 15. Page 3 of 20
3.- Airway involvement: Circumferential wall thickening, specially subglottic portion of the trachea. Atelectasis or pneumonia may be seen due to distal airway involvement. None of the patients in our series had airway involvement. 4.- Pleural involvement: Uni or bilateral effusions. Pleural thickening (rare). Pleural effusion was discover in 22,2% in our series Fig. 10 on page 10. 5.- Mediastinal involvement: Lymphadenopathy but are relatively uncommon. We saw lymphadenopathies in 33,3% of our patients Fig. 11 on page 8. CT uncommon manifestations: 1.- Cardiac involvement: Pericarditis, coronary lesions, valvular dysfunction and conduction abnormalities. 2.- Involvement of the great arteries: Aortitis and periaortic inflammation. None of these manifestations were present in our series. Images for this section: Page 4 of 20
Fig. 1: Multiple bilateral nodules with basal predominance Page 5 of 20
Fig. 2: Right parahilar opacity Page 6 of 20
Fig. 3: Bilateral widespread opacities Page 7 of 20
Fig. 7: Atoll sign Page 8 of 20
Fig. 11: Right hilar lymphadenopaties and nodular opacity in right lower lobe Page 9 of 20
Fig. 6: Sagital reformation: lung mass with hypodenses areas and gas bubbles related with cavitation Page 10 of 20
Fig. 10: Right pleural effusion Page 11 of 20
Fig. 5: Upper right lobe cavitated nodule Page 12 of 20
Fig. 8: Bilateral ground-glass opacities and focal consolidation in right upper lobe Page 13 of 20
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Fig. 4: Nodule and ground-glass opacities in left lower lobe Page 15 of 20
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Fig. 9: Oblique reformation: ground-glass opacities and a small consolidation in the left lower lobe. Page 17 of 20
Conclusion Wegener granulomatosis is an uncommon necrotizing vasculitis that may affect any organ, although thoracic involvement predominates. Chest CT is the imagine modality of choice for diagnosis (due to it shows typical findings that the radiologist should know to suggest this entity), as well as for surveillance and follow up of these patients. References Webb & Higgings. Radiología pulmonar y Cardiovascular. Madrid. Marbán. 2009. Hansell. Imaging of diseases of the chest. 5th edition. Mosby Elsevier. 2010. Felipe Martinez, Jonathan H. Chung, Subba R. Digumarthy. Common and Uncommon manifestations of Wegener Granulomatosis at the chest CT: Radiologic-Patologic Correlation. RadioGraphics 2012; 32:51-69. Personal Information Fig. 12 on page 18 Fig. 13 on page 18 Radiology department Images for this section: Fig. 12 Page 18 of 20
Fig. 13 Page 19 of 20
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