Bilateral pulmonary artery aneurysms in Behçet disease: review of literature and report of one case Poster No.: 486 Congress: ESCR 2013 Type: Poster Presentation Authors: O. Addou, B. Alami, M. Boubbou, M. Maaroufi, I. Kamaoui, S. Tizniti; Fes/MA Keywords: Cardiovascular system, Vascular, CT-Angiography, Computer Applications-Detection, diagnosis, Education and training 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.escr.org Page 1 of 13
Purpose To describe the chest angioscan imaging findings in the evaluation of pulmonary arterial involvement in Behçet disease. Methods and Materials We report a rare case of Behçet disease in a male teenager aged of 13-year-old who presented with chronic dyspnea, dry cough, and complicated recently by moderately abundant hemoptysis. The patient was assessed by chest radiography and the diagnosis of pulmonary artery aneurysms was confirmed by computed tomography angiography of the chest. Results Behçet's disease was suspected with recurrent oral aphthous ulcers and unexplained ocular findings, without genital ulcers. The chest X-ray showed a round hilar opacity in both lungs. The chest CT angiography revealed bilateral aneurysms of the proximal pulmonary arteries. Epidemiology - Behçet disease is a rare multisystemic and chronic inflammatory disorder of unknown cause that is characterized by recurrent oral and genital ulcerations, ocular manifestations, and additional clinical manifestations in multiple organ systems (table 1). - The disease may present with vascular, cutaneous, pulmonary, neurologic, rheumatologic, genitourinary and gastrointestinal manifestations [1, 2] (table 2). - Vascular system is involved in 20%-40% of patients with Behçet disease and it is the major cause of mortality [3, 4]. - Pulmonary vascular involvement can lead to the formation of pulmonary artery aneurysm, thrombotic occlusion of vessels, pulmonary infarction, or pulmonary hemorrhage. Page 2 of 13
- Pulmonary artery aneurysm has a poor prognosis: 30% of patients with pulmonary artery aneurysms die within 2 years [18]. - Behçet's disease has episodes of exacerbation and remission, frequently seen in the third decade of life and is more frequent in men. The course of the disease is more severe in men than women. - Behçet disease is most frequently seen in Mediterranean, Middle East, and Far East countries. Physiopathology - The etiopathogenesis of Behçet's disease is unknown. - Genetic predisposition (human leukocyte antigen [HLA], HLA-B51) and certain microorganisms are believed to play roles in its cause. - The underlying pathologic process in Behçet disease is vasculitis and perivascular inflammatory infiltrates affecting vessels of differing sizes in various organs [3, 18], especially aorta and pulmonary arteries. - It is started by inflammation of the vasa vasorum of the tunica media, which causes destruction of the elastic fibers of the media and dilatation of the vessel lumen. Thickening of the vessel wall is caused by inflammation and infiltration by lymphocytes, plasma cells, and neutrophils. Thrombosis of the pulmonary arteries in Behçet disease is usually in situ thrombosis. [5]. Clinical diagnosis According to the diagnostic criteria of the International Study Group for Behçet Disease [13], the diagnosis is based on the presence of recurrent oral ulcerations, along with two of the following criteria (table 1): (a) recurrent genital ulcerations, (b) eye lesions, including uveitis and retinal vasculitis, (c) skin lesions (folliculitis, erythema nodosum), and (d) positive skin pathergy test (pustule formation 24-48 hours following skin prick test). Page 3 of 13
Hemoptysis is the most common presenting symptom of pulmonary Behçet and is one of the leading causes of death. Possible causes for hemoptysis include rupture of an aneurysm with erosion into a bronchus and thrombosis of pulmonary vessels [2, 18]. Laboratory findings - Behçet's disease has no pathognomonic laboratory findings [9]. - The erythrocyte sedimentation rate, C-reactive protein may be raised. - Mild anemia, mild leukocytosis, and thrombocytopenia may be noted. Radiology * Conventional chest radiography is commonly used: (Fig. 1) - for initial assessment of pulmonary symptoms of Behçet disease, It may detect a hilar enlargement or a polylobular and round opacities, due to pulmonary artery aneurysms, and may show a mediastinal widening, which is an indirect sign of an aneurysm developing in the thoracic aorta. - for follow-up, and - for assessment of response to therapy. The pulmonary parenchymal changes are non specific and appear as focal and diffuse areas of increased opacity. * Angiography and venography should not be performed in patients with Behçet disease due to increased risk for aneurysm formation at the arterial puncture site and venous thrombosis after contrast material injection [4, 12]. * Spiral Chest computed tomography (CT) (Fig 2, 3) is the preferred imaging modality for depicting the thoracic manifestations of Behçet disease. It can demonstrate a wide spectrum of thoracic manifestations of Behçet disease, including abnormalities of the thoracic vessels, perivascular tissues, lung parenchyma, pleura, and mediastinal structures. Page 4 of 13
Aneurysms of the pulmonary arteries, with or without thrombosis, are a typical manifestation of Behçet disease. Other manifestations include thrombosis, vasculitis, hemorrhage, infarction, and inflammation. # Pulmonary artery aneurysm - The pulmonary artery is the second most common site of arterial involvement, preceded by the aorta. Aneurysms of the pulmonary artery are more common than thrombosis. Pulmonary artery aneurysm is one of the characteristic lesions of Behçet's disease and is mostly seen in young men [8, 9, 10, 11]. Venous thrombosis of the lower extremities and superior or inferior vena cava occlusion frequently occur in patients with pulmonary artery aneurysms [4, 10]. Hemoptysis is the most common clinical manifestation of pulmonary artery aneurysm, associated with cough, dyspnea, and chest pain. Aneurysms may be single or multiple, unilateral or bilateral, saccular or fusiform [11, 18, 19]. They may be located in the main pulmonary artery or they may be either lobar or segmental and be located proximal to an occluded artery. They are located most frequently in the right lower lobar arteries, followed by the right and left main pulmonary arteries [14, 15, 16, 20]. Pulmonary artery aneurysms also may be occluded totally or partially by a thrombus. Thrombosed aneurysms cause ischemia and infarction in pulmonary parenchyma. CT is also effective in detecting superior vena cava occlusion, intracardiac thrombus formation, aneurysms of the aortic arch and occlusion of the brachiocephalic arteries. Pleural and pulmonary parenchymal changes related to the disease have been reported. * Magnetic resonance angiography (MRA) is considered to be less sensitive than helical CT in demonstrating small aneurysms, but It can be used for imaging of pulmonary artery aneurysms, thrombosis within these aneurysms, venous occlusions, and the development of collateral vessels [17, 18]. Page 5 of 13
Images for this section: Table 1: Diagnostic criteria of Behçet's disease defined by International Study Group Table 2: Frequency of Occurrence of Various Lesions in Behçet Disease. Page 6 of 13
Fig. 1: Chest radiograph of a teenager with Behçet's disease shows bilateral pulmonary artery aneurysm as a hilar enlargement. Page 7 of 13
Fig. 2: a, b. Coronal maximum-intensity-projection CT image showing bilateral pulmonary artery aneurysm (long straight arrow), partially occluded with thrombus. Page 8 of 13
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Fig. 3: A, B. Axial contrast-enhanced chest CT scan (mediastinal window) demonstrates a bilateral pulmonary artery aneurysms with mural thrombi in a 13-year-old boy who presented with hemoptysis. Page 10 of 13
Conclusion Bilateral pulmonary artery aneurysm is rare in Behçet's disease, especially for teenagers. CT angiography of the chest was the procedure of choice in the documentation of the diagnosis and the prognosis depends on the fatal complications of ruptured aneurysm. References 1) Erkan F, Gul A, Tasali E. Pulmonary manifestations of Behçet's disease. Thorax 2001; 56:572-578. 2) Raz I, Okon E, Chajek-Shaul T. Pulmonary manifestations in Behçet's syndrome. Chest 1989; 95: 585-589. 3) Koc Y, Gullu I, Akpek G, et al. Vascular involvement in Behçet's disease. J Rheumatol 1992; 19: 402-410. 4) Erkan F, Cavdar T. Pulmonary vasculitis in Behçet's disease. Am Rev Respir Dis 1992; 146:232-239. 5) Matsumoto T, Uekusa T, Fukuda Y. Vasculo- Behçet's disease: a pathological study of eight cases. Hum Pathol 1991;22:45-51. 6) Lakhanpal S, Tani K, Lie JT, Katoh K, Ishigatsubo Y, Ohokubo T. Pathologic features of Behçet's syndrome. Hum Pathol 1985;16:790. 7) Kaklamani VG, Vaiopoulos G, Kaklamani PG. Behçet's disease. Semin Arthritis Rheum 1998;27:197-217. 8) Grenier P, Bletry O, Cornud F, Godeau P, Nahum H. Pulmonary involvement in Behçet disease. AJR Am J Roentgenol 1981;137:565. 9) Hamuryudan V, Yurdakul S, Moral F, Numan F, Tuzun H, Tuzuner N, et al. Pulmonary artery aneurysms in Behçet's syndrome: a report of 24 cases. Br J Rheumatol 1994;33:48-51. Page 11 of 13
10) Gibson RN, Morgan SH, Krausz T, Hughes GR. Pulmonary artery aneurysms in Behçet's disease. Br J Radiol 1985;58:79-82. 11) Numan F, Islak C, Bermen T, Tuzun H, Cokyuksek O. Behçet's disease: Pulmonary arterial involvement in 15 cases. Radiology 1994;192:465-8. 12) Park JH, Han MC, Bettman MA. Arterial manifestations of Behçet disease. AJR Am J Roentgenol 1984; 143:821-5. 13) Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. Lancet 1990; 335:1078-1080. 14) Tunac# M, Ozkokmaz B, Tunac# A, Gul A, Engin G Acunas B. CT findings of pulmonary artery aneurysms during treatment for Behc et's disease. AJR Am J Roentgenol 1999;172:729-33. 15) Kabbaj N, Benjelloun G, Gueddari FZ, Dafiri R, Imani F. Vascular involvements in Behçet's disease: based on 40 patient records [in French]. J Radiol 1993; 74:649-656. 16) Hamuryudan V, Yurdakul S, Yaz#c# H. Behçet's syndrome. Rheumatol Eur 1997;26:31-3. 17) Yaz#c# H, Yurdakul S, Hamuryurdan V. Behçet's syndrome. Curr Opin Rheumatol 1999;11:53-7. 18) Almog Y, Polliack G, Elhalel M, Shalit M, Rosenmann E. Bilateral pulmonary artery aneurysms in Behçet's disease. Eur Respir J 1993;6:1067-9. 19) Winer-Muram HT, Gavant ML. Pulmonary CT findings in Behçet disease. J Comput Assist Tomogr 1989;13: 346-7. 20) Tunaci M, Özkokmaz B, Tunaci A, et al. CT findings of pulmonary artery aneurysms during treatment for Behçet's disease. AJR 1999;172:729-33. Personal Information Page 12 of 13
Chief resident in radiology department. Interested in cardiovascular Imaging. Page 13 of 13