Images in Clinical Medicine

Bilateral Pulmonary-Artery Aneurysms in Behçet's Syndrome

Sudhir Lohani, M.D., and Robert Niven, M.D.

N Engl J Med 2005; 353:400July 28, 2005

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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 Behçet'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. Behçet'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

Citing Articles (4)

Citing Articles

1. 1

   Jaejoon Lee, Jung-Won Noh, Ji Won Hwang, Hyungjin Kim, Joong Kyong Ahn, Eun-Mi Koh, Hoon-Suk Cha. (2008) Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes–Stovin syndrome patient. Clinical Rheumatology 27:11, 1455-1458
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2. 2

   Oğuz Uzun, Levent Erkan, Ilkser Akpolat, Serhat Findik, Atilla Güven Atıcı, Tekin Akpolat. (2008) Pulmonary Involvement in Behçet’s Disease. Respiration 75:3, 310-321
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3. 3

   Stefan Kleinert, Justus G. Müller, Heiner Langenfeld, Jens A. Wagner. (2007) Akute kardiopulmonale Dekompensation einer 39-Jährigen mit chronischer Zyanose. Intensivmedizin und Notfallmedizin 44:7, 447-451
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4. 4

   E.Y. Chan, T. Avcin, S. Dell, D. Manson, E. Cutz, R. Schneider, F. Ratjen. (2007) Massive hemoptysis in an 11-year-old girl with isolated pulmonary arteritis. Pediatric Pulmonology 42:2, 177-180
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