대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Behcet's disease

Radiologic Findings

Chest radiograph shows an ill-defined patchy opacity in the both lower lung zones.

CT images show the diffuse luminal narrowing of the proximal portion of right subclavian artery and collateral draining veins along the right chest wall. Diffuse luminal narrowing of SVC is also noted (not shown here). There is multifocal low-density thrombi in the left lower lobar and segmental pulmonary arteries. Large thrombus in the right atrium was identified by echocardiography (not shown here). Multiple ill-defined pleural based patch consolidations are also noted in both lower lobes, suggestive of pulmonary infarction with/without hemorrhage due to pulmonary thromboembolism. On abdominal CT scan was done before 7 months, the saccular thrombotic aneurysm is present in the celiac trunk

Brief Review

Behcet's disease is a rare form of vasculitis of obscure etiology. Any large or small artery, vein, or organ may be involved in an unpredictable combination. According to the criteria set by the International Study Group for Behcet's Disease, the diagnosis is based on the presence of recurrent oral ulcers plus two of the following: recurrent genital ulcers, eye lesions (anterior or posterior uveitis, retinal vasculitis), skin lesions (erythema nodosum, pseudofolliculitis, papulopustular lesions, acneiform nodules), or a positive pathergy test (pustule formation 24-48 hr after skin prick). Patients are usually 20-30 years old at the time of onset of the disease. The prevalence is considerably higher in Mediterranean countries, the Middle East, and the Far East than in the rest of the world.

Intrathoracic manifestation of Behcet's disease consist mainly of thromboembolism of the superior vena cava and/or other mediastinal veins; aneurysm of aorta and pulmonary arteries; pulmonary infarct and hemorrhage; pleural effusion; and, rarely, myocardial or pericardial involvement, cor pulmonale, and mediastinal or hilar lymphadenopathy.

Chest radiography is the best diagnostic method for evaluating thoracic involvement in Behcet's disease, and shows mediastinal widening, air-space consolidation, and lung mass. Sudden hilar enlargement or intraparenchymal round opacities is a sign of pulmonary artery aneurysm. CT scan shows that that mediastinal widening was due to

thrombosis or narrowing of the superior vena cava which caused mediastinal edema; that air-space consolidation was due to pulmonary hemorrhage or infarction; and that lung mass was due to aneurysm of the right or left pulmonary artery. They may recur, progress, or resolve, either spontaneously, within a few weeks to several months, or with corticosteroid therapy. Although no comparative studies are available, CT and MR angiography appear to be imaging techniques of choice for evaluating vascular involvement. CT also can demonstrate pulmonary parenchymal changes such as air-space nodules, cavities, and mosaic areas of lung attenuation.

Recently, helical CT findings of pulmonary artery aneurysms during treatment for Behcet’s disease were reported. Pulmonary artery aneurysms may become smaller or disappear with medical treatment. Mural thrombotic changes may be observed during the regression of pulmonary artery aneurysms. Perianeurysmal consolidation and air-space nodules, and mosaic attenuation areas may disappear during or after treatment.

Differential diagosis includes Hughes-Stovin syndrome that has indentical histologic and clinical features except for oral and genital ulcers. Both Reiter's syndrome and relapsing polychondritis may complicated by aortitis and aortic aneurysms with some overlapping clinical features. Arterial narrowings and occlusions, producing symptoms of vascular insufficiencies in Behcet's disease, may clinically mimic Takayasu's arteritis.

References

1. Tunaci A, Berkmen YM, Gokmen E. Thoracic involvement in Behcet's disease: pathologic, clinical, and imaging features. AJR 1995;164:51-56.

2. Tunaci M, Ozkorkmaz B, Tunaci A, Gul A, Engin G, Acunas B. CT findings of pulmonary artery aneurysms during treatment for Behcet's disease. AJR 1999;172:729-33.

3. Ahn JM, Im JG, Ryoo JW, et al. Thoracic manifestations of Behcet syndrome: radiographic and CT findings in nine patients. Radiology 1995;194:199-203.

Keywords

Vascular, Lung, Vascular, Behcet's disease,