[Hughes-Stovin syndrome: About one case in a young man with recurrent thrombosis and pulmonary artery aneurysm and literature review]

Rev Med Interne. 2019 Feb;40(2):120-125. doi: 10.1016/j.revmed.2018.07.009. Epub 2018 Jul 27.
[Article in French]


Introduction: First described in 1959, Hughes-Stovin syndrome is a very rare disorder combining vascular aneurysms, especially from pulmonary arteries, and thrombosis. The disease affects mostly the young male and is sometime associated with Behçet' disease.

Case report: Here, we report the case of a 19-year-old man with hemoptysis and dyspnea revealing recurrent pulmonary embolisms despite efficient anticoagulant therapy. The patient subsequently developed fever and an inflammatory syndrome. Physical examination showed ulcers of the tongue. Angio-CT revealed recent pulmonary embolism, femoral vein thrombosis, and a unique threatening aneurysm of a left pulmonary artery segment. The aneurysm was embolized and simultaneously a vena cava filter was inserted.

Conclusion: Hughes-Stovin syndrome requires immediate therapeutic decision, with an important risk of the anticoagulation. High dose steroids and in most cases, intensive immunosuppressive therapies are required such as cyclophosphamide.

Keywords: Aneuvrysm; Anévrysme; Aphtose; Aphtosis; Behçet; Hughes-Stovin syndrome; Maladie de Behçet; Syndrome de Hughes-Stovin; Thromboses; Thrombosis.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aneurysm / complications
  • Aneurysm / diagnosis*
  • Behcet Syndrome / complications
  • Behcet Syndrome / diagnosis
  • Behcet Syndrome / pathology
  • Dyspnea / diagnosis
  • Dyspnea / etiology
  • Hemoptysis / diagnosis
  • Hemoptysis / etiology
  • Humans
  • Male
  • Pulmonary Artery / pathology*
  • Recurrence
  • Syndrome
  • Venous Thrombosis / complications
  • Venous Thrombosis / diagnosis*
  • Young Adult