Is Hughes-Stovin syndrome Behçet's disease ?

D. Erkan¹, Y. Yazici^1,2, A. Sanders³, D. Trost⁴, H. Yazici⁵

¹Department of Rheumatology, Hospital for Special Surgery, Weill Medical College of Cornell University, New York; ²Brooklyn Heights Arthritis Associates, Long Island College Hospital, Brooklyn; ³Department of Pulmonary Diseases, Weill Medical College of Cornell University, New York; ⁴Department of Radiology, Weill Medical College of Cornell University, New York, New York, USA; ⁵Department of Rheumatology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey.

ABSTRACT
Hughes-Stovin syndrome (HSS) is a rare clinical disorder, which has been described as the presence of pulmonary artery aneurysm in the setting of systemic thrombosis. The term "Incomplete Behçet's Disease" has also been used to describe this syndrome due to the clinical and histopathological similarities between Behçet's disease and HSS. Indeed, pulmonary involvement can be indistinguishable between these two conditions of unknown pathophysiology. We describe an HSS patient who presented with a recurrent pulmonary artery aneurysm, review the clinical and pathological manifestations of HSS, discuss its similarities to Behçet's disease, and finally make the argument that HSS is in fact Behçet's disease.

Key words
Hughes-Stovin syndrome, Behçet's disease, pulmonary artery aneurysm.

Please address correspondence to: Doruk Erkan, MD, Assistant Attending Physician, Department of Rheumatology, Hospital for Special Surgery, 535 E70th Street, New York, NY 10021, USA.
E-mail: derkan@pol.net

Clin Exp Rheumatol 2004; 22 (Suppl. 34): S64-S68.
© Copyright Clinical and Experimental Rheumatology 2004.