Is Hughes-Stovin syndrome Behcet's disease?

Erkan D., Yazici Y., Sanders A., Trost D., Yazici H.

CLINICAL AND EXPERIMENTAL RHEUMATOLOGY, vol.22, no.4, 2004 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 22 Issue: 4
  • Publication Date: 2004
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Istanbul University Affiliated: No


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 Behcet's Disease" has also been used to describe this syndrome due to the clinical and histopathological similarities between Behcets 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 Behcet's disease, and finally make the argument that HSS is in fact Behcet's disease.