Hughes-Stovin syndrome

Epidemiology

• extremely rare (< 40 cases reported)

Clinical manifestations

phase 1

• thrombophlebitis
• chronic venous thromboembolism

phase 2

• vascular aneurysm
  • pulmonary artery aneurysm
  • bronchial artery aneurysm
  • can be multiple or anywhere in the systemic circulation

phase 3

• aneurysm rupture
  • hemoptysis
  • massive hemorrhage & death

Laboratory

• no diagnostic tests

Radiology

• pulmonary CT angiography

Differential diagnosis

• Behcet syndrome

Management

• immunosuppresion
  • glucocorticoids, cyclophosphamide & infliximab*

* inflimi

More general terms

• vasculitis
• syndrome

Additional terms

• Behcet syndrome