A case of Hughes-Stovin syndrome (incomplete Behçet's disease) with extensive arterial involvement : Unmasking the true face of a rare syndrome

Z Rheumatol. 2019 May;78(4):365-371. doi: 10.1007/s00393-019-0618-7.


Hughes-Stovin syndrome (HSS), characterized by the combination of multiple pulmonary artery aneurysms and deep vein thrombosis, is a rare and an under-recognized clinical entity with less than 40 published cases in English medical literature. Vascular venous thrombotic events, as occurring in the course of Behçet's disease (BD), are also described in HSS, e. g., vena cava, intra-cardiac, jugular vein, iliac vein, femoral vein, and dural sinus thrombosis. We describe a 35-year-old man with HSS showing classical features of the syndrome in the form of recurrent thrombophlebitis of the lower limb veins, pulmonary arterial aneurysms, and left lower limb ischemia with extensive arterial tree involvement. The patient presented with critical arterial ischemia in the left lower limb together with aortic and left common iliac artery thrombosis, occlusion of the left superficial femoral artery, and occlusion of both lower limb arteries. Urgent vascular surgeries were carried out for limb salvage. Shortly after, the patient started on pulse corticosteroid/cyclophosphamide therapy, followed by monthly cyclophosphamide for 1 year, with much improvement. We discuss arterial involvement in HSS and similarities of HSS and BD regarding thrombotic events. We summarize the current management options of HSS.

Keywords: Aortitis; Arterial vasculitis in Hughes–Stovin syndrome; Hughes–Stovin syndrome; Incomplete Behçet; Pulmonary artery aneurysms; Thrombophlebitis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aneurysm* / complications
  • Behcet Syndrome* / complications
  • Behcet Syndrome* / diagnosis
  • Diagnosis, Differential
  • Humans
  • Male
  • Pulmonary Artery / pathology
  • Pulmonary Embolism* / complications
  • Thromboembolism
  • Vasculitis* / complications
  • Vasculitis* / diagnosis