Diagnosis With Brief Discussion
- Behcet's disease
- Radiologic Findings
- On CT scan, airspace consolidation in right lung subpleural area and dilation of right interlobar artery with thrombus are noted. There is a well defined round highly enhancing lesion with perilesional low density. The connection between this enhancing lesion and pulmonary artery is found.
- Brief Review
- Behcet disease is a rare systemic large and small vessel vasculitis likely due to deposition of immune complexes. The clinical triad of recurrent oral and genital ulcerations and uveitis, as well as skin lesions and positive skin pathergy test are key factors in diagnosis.
Pulmonary involvement is relatively infrequent, have been reported 5% to 10% of cases. Aneurysms of the pulmonary arteries, otherwise an uncommon entity, are a typical manifestation of Behcet disease. In fact, Behcet disease is the most common cause of pulmonary artery aneurysm. Other pulmonary manifestations include thrombosis of the superior vena cava and/or other mediastinal veins, pleural effusion, mediastinal or hilar lymphadenopathy, and rarely, myocardial or pericardial involvement.
Pulmonary artery thrombosis as in this case may be associated with focal pulmonary hemorrhage. Hemoptysis may occur due to leakage or rupture of an aneurysm and may be life threatening. Therefore, aneurysm formation in the pulmonary arteries indicates a poor prognosis.
No randomized controlled studies have evaluated treatment options for pulmonary vasculitis with Behcet disease. Colchicine has been recommended as a basic treatment, but a recent controlled trial has again confirmed that this pharmacotherapy is only effective relieving erythema nodosum and urogenital ulceration. Various treatment modalities in pulmonary aneurysm include imunosuppressive drugs alone or in combination with steroids, surgery, and endovascular embolization. In case of massive hemoptysis, surgery or endovascular embolization is necessary. To avoid pulmonary amputation, aneurysmectomy or occlusion of the arterial defect by direct suture was applied.
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- Vascular, Vasculitis, Behcet's disease,