Behçet disease

Pseudoaneurysm
of the common carotid: an unusual complication of behcet"s disease. Contrast-enhanced CT angiogram demonstrating a 7 ×7 cm sized pseudoaneurysm at the left common carotid with mass effect on the trachea and adjacent vascular structures.
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Pseudoaneurysm
of the common carotid: an unusual complication of behcet"s disease. Communication between CCA and pseudoaneurysm.
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Pseudoaneurysm
of the common carotid: an unusual complication of behcet"s disease. CT Angiogram and CT neck showing pseudoanévrysm of CCM
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Insights into
imaging of aortitis. a, b A 40-year-old male with Behçet"s disease. Sagittal maximum-intensity-projection CT image (a) shows a saccular aneurysm arising from the distal thoracic aorta (arrows). Coronal maximum-intensity-projection CT image (b) shows saccular aneurysm arising from the left subclavian artery (white arrows)
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Insights into
imaging of aortitis. A 24-year-old male with Behçet"s disease. Axial CT image shows ruptured pseudoaneurysm of the left anterior descending coronary artery (white arrow) with accompanying haematoma (white asterisk)
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Insights into
imaging of aortitis. A 34-year-old male with Behçet"s disease. 3D colour-coded volume-rendering CT image shows pseudoaneurysm of the superficial femoral artery
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Insights into
imaging of aortitis. a, b A 23-year-old male with Behçet"s disease. Axial (a), sagittal reformatted (b) contrast-enhanced CT images show partially thrombosed right pulmonary artery aneurysm (arrow)
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Insights into
imaging of aortitis. A 20-year-old male with Behçet"s disease. Sagittal reformatted CT image shows superior vena cava thrombosis (black arrows) and collateral vessels in the mediastinum and chest wall (white arrows)
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Insights into
imaging of aortitis. A 31-year-old male with Behçet"s disease. Coronal maximum-intensity projection CT image (a) shows thrombosed left lower lobe pulmonary artery aneurysm (arrow) and pleural-based, wedge-shaped infarction (arrowhead). Axial CT image (b) also shows a thrombosed left lower lobe pulmonary arterial aneurysm (arrow)
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Imaging of
intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data. Terminal ileitis in a 23-year-old female with a history of recurrent oral and genital aphthous ulcers and uveitis presenting with RLQ pain finally diagnosed as Behçet’s disease. MRE was obtained. Coronal and axial T2-W images (A, B) show mural thickening in the terminal ileum (thick white arrows) without significant predilection to the mesenteric side in contrast to Crohn’s disease. There is also a deep penetrating ulcer (thin white arrow). Axial post-contrast T1-W image (C) displays increased contrast enhancement in the thickened terminal ileal segment (white arrowheads) without surrounding mesenteric inflammatory changes. Axial DWI image and corresponding ADC map obtained at b-value of 800 s/mm2 (D, E) show restricted diffusion in the involved segment (white dotted oval). Histopathologic examination confirmed Behçet’s disease
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Imaging of
intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data. Intestinal vasculitis in a 26-year-old female with known Behçet’s disease and positive HLA-B5 complaining of episodic RLQ pain. MRE was obtained. Coronal T2-W image (A) demonstrates irregular circumferential mural thickening of the cecum and proximal ascending colon (thick black arrows) without mesenteric side predilection or pericolic infiltration. Coronal and axial post-contrast T1-W images (B, C) show marked enhancement of thickened colonic wall (thick white arrows) mildly extending to ileocecal valve (thin white arrow) with low-grade mural hyperenhancement in the adjacent terminal ileum (white arrowhead). Axial DWI image (D) obtained at a b-value of 800 s/mm2 reveals restricted diffusion in the thickened cecum (white dotted oval)
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Pictorial
review of the pulmonary vasculature: from arteries to veins. Pulmonary artery aneurysm in a patient with Behçet’s disease. a Three-dimensional reconstruction from a contrast-enhanced CT scan showing a right lower lobe pulmonary artery aneurysm in a patient with Behçet’s disease (white arrow). Digital subtraction fluoroscopic images obtained before (b) and after (c) coil embolisation of the right lower lobe pulmonary aneurysm (white arrow)
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Behçet disease is a multisystemic and chronic inflammatory vasculitis of unknown etiology.

Epidemiology

The mean age at which Behçet disease occurs is 20-30 years. The disease is most prevalent in the Mediterranean region, Middle East and East Asia. The highest incidence has been reported in Turkey. Males are affected 2-5 times more often than females.

Clinical presentation

The classic clinical triad of Behçet disease consists of:

  • oral ulceration
  • genital ulceration
  • ocular manifestations

    • Additional clinical manifestations include skin, joints, gastrointestinal tract, genitourinary tract, central nervous system, cardiovascular system, and lung involvement. Vascular complications develop in ~30% (20-40%) of patients with Behçet disease.

    Pathology

    The underlying pathologic process in Behçet disease is vasculitis, and perivascular inflammatory infiltrates affecting vessels of differing sizes in various organs.

    Associations
    Distribution

    Primarily involves four main systems:

    • cardiovascular manifestations of Behçet disease
      • affects 5-30% of cases
      • thickening of the aorta and superior vena cava (vasculitis)
      • inflammation of the veins leads to thrombosis while arterial involvement includes arterial narrowing and aneurysmal dilatation
      • pericarditis is the most common cardiac manifestation occurring in 39% of cases
    • thoracic manifestations of Behçet disease
      • affects 1-8% of cases
      • pulmonary arterial aneurysm: fusiform to saccular, commonly multiple and bilateral, located in the lower lobe or main pulmonary arteries (most common pulmonary manifestation of Behçet disease)
      • subpleural alveolar infiltrates, and wedge-shaped or rounded areas of increased density, which represent focal vasculitis and thrombosis resulting in infarction, hemorrhage, and focal atelectasis
    • gastrointestinal manifestations of Behçet disease: affects 5-60% of cases
    • CNS manifestations of Behçet disease: affects 10-50% of cases
    • musculoskeletal manifestations of Behçet disease: >50% develop articular disorders or arthralgia

    Treatment and prognosis

    The disease may recur, progress, or resolve (either spontaneously, within a few weeks to several months, or with corticosteroid therapy).

    History and etymology

    It is named after Hulusi Behçet (1889-1948), Turkish dermatologist.

    Differential diagnosis

    On imaging consider:

    Siehe auch:
    und weiter:
    Assoziationen und Differentialdiagnosen zu Morbus Behçet:
    Hughes-Stovin-Syndrom
     
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