Abstract

Behçet's disease (BD) causes vascular inflammation and necrosis in a wide range of organs and tissues. In the thorax, it may cause vascular complications, affecting the aorta, brachiocephalic arteries, bronchial arteries, pulmonary arteries, pulmonary veins, capillaries, and mediastinal and thoracic inlet veins. In BD, chest radiograph is commonly used for the initial assessment of pulmonary symptoms and complications and for follow-up and establishment of the response to treatment. With the advancement of helical or multislice computed tomography (CT) technologies, such noninvasive imaging techniques have been employed for the diagnosis of vascular lesions, vascular complications, and pulmonary parenchymal manifestations of BD. CT scan (especially, CT angiography) has been used to determine the presence and severity of pulmonary complications without resorting to more invasive procedures, in conjunction with gadolinium-enhanced three-dimensional (3D) gradient-echo magnetic resonance (MR) imaging with the subtraction of arterial phase images. These radiologic methods have characteristics that are complementary to each other in diagnosis of the thoracic complications in BD. 3D ultrashort echo time (UTE) MR imaging (MRI) could potentially yield superior image quality for pulmonary vessels and lung parenchyma when compared with breath-hold 3D MR angiography.

Highlights

  • Behçet’s disease (BD) is a chronic, systemic inflammatory disease of unknown origin [1,2,3,4]

  • Pulmonary artery aneurysms can be fatal due to perforation to the adjacent bronchus [3, 18,19,20] or it may be the result of the development of in situ thrombosis related to the active vasculitis [3, 8, 19]

  • Patients with mediastinal fibrosis and superior vena cava (SVC) thrombosis present with signs or symptoms related to obstruction or compression of vital mediastinal structures such as central airways, axillar, subclavian and brachiocephalic veins, and SVC [21, 22]

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Summary

Introduction

BD is a chronic, systemic inflammatory disease of unknown origin [1,2,3,4]. A clinical triad of oral ulcers and genital ulcers and uveitis is characteristic of the disease. is triple symptom complex was first described by the Turkish dermatologist Hulusi Behçet in 1937 [5]. A clinical triad of oral ulcers and genital ulcers and uveitis is characteristic of the disease. Clinical manifestations additional to this triad were described later including involvement of the skin, joints, large vessels, lung, brain, and gastrointestinal and genitourinary tracts (Table 1) [4, 6,7,8]. E variability of clinical manifestations in addition to specific histological or laboratory findings may cause the difficulty in diagnosis [8]. Ere are no specific diagnostic laboratory tests or histopathologic findings. Immunopathological findings suggest that the main underlying pathology in the patients suffering from Behçet’s disease is vasculitis affecting the small and large pulmonary arteries [3, 4, 8]. In this study we review the clinical and imaging findings of BD. Positive skin pathergy test read by a physician at 48 hours—that is, a 2 mm erythematous papule or pustule at the prick site 48 hours after the application of a sterile hypodermic 20–22 gauge needle which obliquely penetrated avascular antecubital skin to a depth of 5 mm

Clinical Findings
Imaging Findings
Involvement of Thoracic Vessels
Conclusion
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