Bilateral Pulmonary-Artery Aneurysms in Beh?et's Syndrome
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《新英格兰医药杂志》
A 28-year-old Asian woman presented with pyrexia of unknown origin, oral and genital ulcers, and hemoptysis. A chest radiograph showed bilateral central hilar opacities (Panel A, arrows). A computed tomographic scan of the chest showed bilateral pulmonary-artery aneurysms with associated thickening of the vessel wall and contrast enhancement consistent with edema, which is likely to be associated with thrombosis (Panel B, arrows). The patient was given a diagnosis of Beh?et's syndrome and was treated with prednisolone and pulse cyclophosphamide. A follow-up chest radiograph (Panel C), obtained 18 months after she began the immunosuppressive treatment, showed resolution of the pulmonary-artery aneurysms. Beh?et's syndrome is classically associated with recurrent oral and genital ulcerations and uveitis; however, it is a multisystem disease that may have intestinal, articular, neurologic, and vascular involvement.
Sudhir Lohani, M.D.
Robert Niven, M.D.
Wythenshawe Hospital
Manchester M23 9LT, United Kingdom
Sudhir Lohani, M.D.
Robert Niven, M.D.
Wythenshawe Hospital
Manchester M23 9LT, United Kingdom