Pulmonary and Vascular Manifestations of Behçet Disease: Imaging Findings

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1 Cardiopulmonary Imaging Pictorial Essay Ceylan et al. Pulmonary and Vascular Manifestations of ehçet Disease Cardiopulmonary Imaging Pictorial Essay Naim Ceylan 1 Selen ayraktaroglu 1 Sukru Mehmet Erturk 2 Recep Savas 1 Hudaver lper 1 Ceylan N, ayraktaroglu S, Ertürk ŞM, Savaş R, lper H Keywords: aneurysms, ehçet disease, inflammatory disorders, pulmonary artery, superior vena cava DOI: /JR Received March 16, 2009; accepted after revision ugust 8, Department of Radiology, Ege University School of Medicine, ornova, Izmir 35100, Turkey. ddress correspondence to N. Ceylan (ceylannaim@gmail.com). 2 Department of Radiology, Sisli Etfal Training and Research Hospital, Istanbul, Turkey. WE This is a Web exclusive article. JR 2010; 194:W158 W X/10/1942 W158 merican Roentgen Ray Society Pulmonary and Vascular Manifestations of ehçet Disease: Imaging Findings OJECTIVE. This article focuses on the radiologic findings related to vascular system involvement and pulmonary, pleural, and mediastinal involvement of ehçet disease. CT is an important diagnostic imaging technique in the evaluation of patients with ehçet disease. CONCLUSION. CT is a valuable imaging technique in the diagnostic work-up of ehçet disease. CT effectively demonstrates vascular system involvement that is the main cause of mortality in these patients. CT is also effective in detecting mediastinal, pleural, and pulmonary parenchymal findings related to the disease. ehçet disease is a rare multisystemic and chronic inflammatory disorder with an unknown cause. Genetic predisposition (human leukocyte antigen [HL], HL-51) and certain microorganisms are believed to play roles in its cause. It is most 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 and in those younger than 25 years at disease onset. ehçet disease is most frequently seen in Mediterranean, Middle East, and Far East countries. The highest prevalence rate, to our knowledge, was reported from Turkey as per 100,000 [1]. Recurrent oral and genital ulcers and uveitis are the clinical triad of the disease. ccording to the diagnostic criteria of the International Study Group for ehçet s Disease [2], the diagnosis is made on the detection of oral ulcers and at least two of the following criteria: recurrent genital ulcers, ocular lesions including uveitis and retinal vasculitis, skin lesions (folliculitis, erythema nodosum), and positive skin pathergy test (i.e., pustule formation hours after skin prick). ehçet disease may also affect joints, the gastrointestinal system, CNS, cardiovascular system, and the lungs. Vascular system involvement is seen in approximately 25 30% of patients and is the most common cause of mortality [1]. The main pathologic process in ehçet disease is vasculitis and perivascular infiltration affecting vessels of various sizes [3]. This article focuses on the vascular system involvement of ehçet disease and on the rare radiologic findings related to pulmonary, pleural, and mediastinal involvement of the disease. Imaging Considerations In general, chest radiography is the firstchoice imaging technique in patients with pulmonary symptoms. In the setting of ehçet disease, aneurysms of the thoracic aorta and pulmonary arteries or mediastinal veins can be seen as hilar or mediastinal enlargements on chest radiographs. Parenchymal involvement may be encountered and appear on radiography as nonspecific focal opacities. Chest radiography can also be used to evaluate response to therapy [4]. CT is an important imaging technique in the diagnostic workup of ehçet disease. Rapid advances in MDCT scanners allow a more detailed analysis of the aorta and other arterial and venous structures by acquiring data in shorter breathhold and acquisition times. CT angiography is more accurate than MRI because it has a higher spatial resolution and causes less artifacts. Furthermore, CT can show lung parenchyma in more detail than MRI. However, the major drawback of CT is radiation exposure [5]. Vascular System Involvement in ehçet Disease lthough the vascular system is involved in only 25 30% of patients with ehçet disease, it is still the major cause of mortality. Vasculitis can affect arteries and veins of any W158 JR:194, February 2010

2 Pulmonary and Vascular Manifestations of ehçet Disease size; however, venous system involvement is more frequent than arterial system involvement. Venous system involvement constitutes approximately 85% of the vascular involvement [6]. Involvement of the venous system is most frequently seen in the form of thrombophlebitis. Thrombophlebitis can affect veins of the lower extremity, superior vena cava, and inferior vena cava. Nevertheless, because deep vein thrombosis is tightly attached to the inflamed veins, the risk of pulmonary emboli is very low [4]. The most serious venous complications in ehçet disease are superior vena cava syndrome and udd-chiari syndrome. Venous occlusion is most frequently seen at the level of superior vena cava [7]. Enlarged collateral vessels in the mediastinum, chest wall, and umbilical region can be observed in the presence of superior vena cava occlusion (Fig. 1). Venous aneurysms are rare complications of ehçet disease. Venous aneurysms in patients with ehçet disease in the absence of predisposing factors, such as trauma or other inflammatory disorders, are extremely rare [8]. We reported a case of bilateral venous aneurysms in a patient who was diagnosed with ehçet disease 3 years after our report was published [9] (Fig. 2). Compared with venous system involvement, arterial system involvement is rare and accounts for only 10 15% of vascular system involvement. Inflammation of the vasa vasorum destructs elastic fibers and results in vessel lumen dilatation and aneurysm formation. rterial manifestations of the disease involve aneurysm formation (65%) and occlusions (35%) [1]. The abdominal aorta is the most commonly affected vessel [6] (Figs. 3 and 4), whereas the thoracic aorta and pulmonary artery are less commonly affected [10]. neurysm development in the aortic branches is very rare, especially at the thoracic levels (Fig. 5). Investigators have proposed the placement of stent-grafts to repair aneurysms of the thoracic or abdominal aorta [10]. The most common cause of pulmonary artery aneurysm is ehçet disease [11]. neurysms can be seen in the main pulmonary arteries or their branches (Fig. 6) and are characteristic CT findings. Patients with pulmonary aneurysms have a poor prognosis [11]. Hughes-Stovin syndrome shares similar radiologic and histopathologic findings with ehçet disease, but neither oral nor genital ulcerous lesions are seen. Pulmonary involvement in Hughes-Stovin syndrome is indistinguishable from that of ehçet disease, and Hughes-Stovin syndrome is known as incomplete ehçet disease [12]. combination of cyclophosphamide and methylprednisolone is used most frequently for the treatment of patients with pulmonary artery aneurysms. Pulmonary artery aneurysms may regress with the use of steroids and immunosuppressive agents. In fact, early recognition of an arterial aneurysm is critical for a good prognosis [13]. Occlusion of the main pulmonary arteries or their branches is another complication of ehçet disease (Figs. 6 and 7). Thrombosis of the pulmonary arteries may accompany aneurysms and occlusions (Fig. 8). When seen, hemoptysis is a result of aneurysm rupture and thrombosis. Mediastinal Involvement in ehçet Disease Fibrosing mediastinitis is a very rare condition that can be seen in patients with ehçet disease. The cause of this entity is not fully understood. Fibrosing mediastinitis has a focal form and a diffuse form. The diffuse form is seen in ehçet disease [14]. In addition, occlusions of the superior vena cava and collaterals are seen in both the mediastinum and chest wall (Fig. 9). Pulmonary Parenchymal and Pleural Involvement in ehçet Disease Involvement of ehçet disease in the pulmonary parenchyma and pleura is seen in 1 10% of patients during the course of ehçet disease [15]. Occlusion and thrombosis of the pulmonary arteries due to pulmonary vasculitis may result in infarction, atelectasis, and hemorrhage (Fig. 10). Infarction is seen as a focal and subpleural consolidation on CT images (Fig. 11). Diffuse air-space nodules represent vasculitis and hemorrhage, especially in patients with hemoptysis (Fig. 12). Infectious pneumonia and cryptogenic organizing pneumonia can be seen during the course of the disease [3] (Fig. 13). Eosinophilic pneumonia, bronchitis, fibrosis, emphysema, and small airways disease may also be encountered in patients with ehçet disease. Small airways disease is the cause of the mosaic perfusion defects on CT scans (Fig. 14). Mosaic perfusion defects can also be seen after pulmonary artery occlusion. dditionally, ehçet disease may cause tracheal and proximal airways occlusions due to ulcerative lesions [16]. Pleural manifestations of ehçet disease include pleural effusions and pleural nodules [2, 4]. Pleural effusions might be secondary to pulmonary infarction, pneumonia, or thrombosis of the superior vena cava (Fig. 15). On CT scans, pleural nodules may appear as pleural irregularities. Conclusion In ehçet disease, vascular system involvement is the main cause of mortality. Pulmonary artery aneurysm is typical for ehçet disease and is a significant sign of a poor prognosis. CT can show the vascular, mediastinal, and pulmonary parenchymal and pleural findings suggestive of the disease very effectively. References 1. Hiller N, Lieberman S, Chajek-Shaul T, ar-ziv J, Shaham D. Thoracic manifestations of ehçet disease at CT. RadioGraphics 2004; 24: [No authors listed]. Criteria for diagnosis of ehçet s disease. International Study Group for ehçet s disease. Lancet 1990; 335: Hamuryudan V, Yurdakul S, Moral F, et al. Pulmonary artery aneurysms in ehçet s syndrome: report of 24 cases. r J Rheumatol 1994; 33: Tunaci, erkmen YM, Gökmen E. Thoracic involvement in ehçet s disease: pathologic, clinical, and imaging features. JR 1995; 164: Cho YK, Lee W, Choi SI, Jae HJ, Chung JW, Park JH. Cardiovascular ehçet disease: the variable findings of rare complications with CT angiography and conventional angiography and its interventional management. J Comput ssist Tomogr 2008; 32: Kabbaj N, enjelloun G, Gueddari FZ, Dafiri R, Imani F. Vascular involvements in ehçet s disease: based on 40 patient records [in French]. J Radiol 1993; 74: hn JM, Im JG, Ryoo JW, et al. Thoracic manifestations of ehçet syndrome: radiographic and CT findings in nine patients. Radiology 1995; 194: urkill GJC, urn PR, Padley SPG. neurysm of the left brachiocephalic vein: an unusual cause of mediastinal widening. r J Radiol 1997; 70: Güney, Demirpolat G, Savaş R, lper H. n unusual cause of mediastinal widening: bilateral innominate vein aneurysms. cta Radiol 2004; 45: Park JH, Chung JW, Joh JH, et al. ortic and arterial aneurysms in ehçet disease: management with stent-grafts initial experience. Radiology 2001; 220: Celenk C, Celenk P, kan H, aşoğlu. Pulmonary artery aneurysms due to ehçet s disease: MR imaging and digital subtraction angiography findings. JR 1999; 172: JR:194, February 2010 W159

3 Ceylan et al. 12. Emad Y, Ragab Y, Shawki el-h, Gheita T, El- Marakbi, Salama MH. Hughes-Stovin syndrome: is it incomplete ehçet s? Report of two cases and review of the literature. Clin Rheumatol 2007; 26: Grenier P, letry O, Cornud F, Godeau P, Nahum H. Pulmonary involvement in ehçet disease. JR 1981; 137: Harman M, Sayarlioglu M, rslan H, yakta H, Harman E. Fibrosing mediastinitis and thrombosis of superior vena cava associated with ehçet s disease. Eur J Radiol 2003; 48: Fairley C, Wilson JW, arraclough D. Pulmonary involvement in ehçet s syndrome. Chest 1989; 96: Prince JS, Duhamel DR, Levin DL, Harrell JH, Friedman PJ. Nonneoplastic lesions of the tracheobronchial wall: radiologic findings with bronchoscopic correlation. RadioGraphics 2002; 22: [Erratum in RadioGraphics 2003; 23:191] Fig year-old man with superior vena cava thrombosis who had known ehçet disease., xial contrast-enhanced CT scan shows occluded superior vena cava (long arrow) and collateral vessels in mediastinum (short arrows)., Sagittal maximum-intensity-projection CT image depicts occluded superior vena cava (lower long arrow) and collateral vessels in superior mediastinum (upper long arrow) and umbilical region (short arrows). Fig year-old man who presented with lower neck mass., xial contrast-enhanced CT scan shows aneurysm dilatation at bilateral brachiocephalic veins (arrows)., Coronal maximum-intensity-projection MR angiography image depicts extension of aneurysm dilatation to right jugular vein (short arrow) and superior vena cava (long arrow). Patient was diagnosed with ehçet disease after follow-up period of 3 years. W160 JR:194, February 2010

4 Pulmonary and Vascular Manifestations of ehçet Disease Fig year-old man with saccular aneurysm arising from abdominal aorta who had known ehçet disease. xial contrast-enhanced CT scan shows saccular aneurysm (arrows) extending to left psoas muscle with intraluminal thrombus at infrarenal aorta. Fig year-old man with known ehçet disease. Coronal maximum-intensity-projection CT image shows saccular aneurysm at brachiocephalic truncus (long white arrow) and abdominal aorta (short white arrows) and depicts stent in inferior vena cava (black arrow) due to venous thrombosis. Fig year-old man with saccular aneurysm arising from abdominal aorta who had ehçet disease. xial contrast-enhanced CT scan shows saccular aneurysm at infrarenal aorta (thin arrows). ortic stent (thick arrow) is also present. JR:194, February 2010 W161

5 Ceylan et al. Fig year-old man with pulmonary artery aneurysm and occlusion who had hemoptysis with known ehçet disease., xial contrast-enhanced CT scan shows aneurysm with thrombosis at interlobar right pulmonary artery (long arrows). Left lower lobe pulmonary artery is occluded with thrombus (short arrows)., Coronal maximum-intensity-projection CT image shows right pulmonary artery aneurysm (long straight arrow) and left pulmonary artery occlusion (curved arrow). Mediastinal collateral vessels (short straight arrows) are present due to occlusion of pulmonary artery. Fig year-old woman with hemoptysis and cough who had known ehçet disease. Coronal maximum-intensity-projection MR angiography image shows total occlusion at right pulmonary artery (short arrows) and patent left pulmonary artery (long arrows). Fig year-old man with dyspnea and hemoptysis who had ehçet disease. xial contrast-enhanced CT image shows thrombosis in left inferior segmental pulmonary arterial vessels (short arrows) and focal parenchymal consolidation (long arrow) due to hemorrhage or vasculitis. W162 JR:194, February 2010

6 Pulmonary and Vascular Manifestations of ehçet Disease Fig year-old woman with fibrosing mediastinitis who had known ehçet disease. and, xial contrast-enhanced CT images show infiltration of mediastinal fat (short thin arrows) and collateral vessels (short thick arrows) in chest wall and mediastinum. zygos vein (long arrow, ) is very prominent because of occlusion of superior vena cava. Fig year-old man with hemoptysis and cough who had ehçet disease. xial CT image (lung window) shows middle lobe atelectasis (long black arrow); ground-glass opacity (white arrows) in right lower lobe; and consolidation in lingula (short black arrow), which represents vasculitis and hemorrhage. Fig year-old woman with infarct in right lung due to right pulmonary artery occlusion who had known ehçet disease. xial CT image (lung window) shows subpleural consolidation (arrows) that represents infarct in right lower lobe. Fig year-old man with hemoptysis who had known ehçet disease. xial CT scan (lung window) shows bilateral acinar nodules that represent hemorrhage and vasculitis. JR:194, February 2010 W163

7 Ceylan et al. Fig year-old woman with cryptogenic organizing pneumonia who had ehçet disease. xial CT scan (lung window) depicts bilateral ground-glass opacities in lower lobes (arrows). Fig year-old woman (same patient shown in Fig. 11) with right pleuritic pain who had known ehçet disease. xial CT image shows right pleural effusion. Fig year-old man with history of ehçet disease. Coronal multiplanar reformatted CT image (lung window) shows extensive mosaic perfusion defects in both lungs. W164 JR:194, February 2010