Is Hughes-Stovin syndrome Behçet's disease?
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.
Erkan
D
D
Department of Rheumatology, Hospitalfor Special Surgery, Weill Medical College of Cornell University, New York, NY 10021, USA. derkan@pol.net
Yazici
Y
Y
Sanders
A
A
Trost
D
D
Yazici
H
H
D000328:Adult; D000783:Aneurysm / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D001528:Behcet Syndrome / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000473:pathology*; D011655:Pulmonary Embolism / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D013902:Radiography, Thoracic; D013577:Syndrome; D013924:Thrombophlebitis / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D014057:Tomography, X-Ray Computed