Hughes Stovin is a rare disorder first described in 1959. It's usually diagnosed along Behcet disease (BD), with a High mortality rate that results from the ruptured pulmonary aneurysm.
To investigate the course and spectrum of Hughes Stovin syndrome in BD and to assess the clinical and imaging features, treatment, and outcomes.
A 38 year-old male, with a family history of AngioBehçet disease (brother who passed away) presented to the hospital with Hemoptysis in the context of a 1 year neglected history of genital/oral aphtae and pseudofolliculitis. CT angiography showed multiple pulmonary arterial aneurysms (Fig. 1), pulmonary embolism along a right brachiocephalic vein thrombosis. A diagnosis of Hughes Stovin syndrome (HSS) associated with Behcet disease was made. Treatment was initiated with methylprednisolone IV 1g daily for three days then discharged from hospital with prescription of Colchicine. Six months later, the patient had recurrence of his hemoptysis with a CT revealing an alveolar hemorrhage. Corticosteroid and immunosuppressant were the mainstay of therapy but the patient wasn’t compliant to treatment. 1 year later, without any follow-up meanwhile, the patient experienced chest pain and hemodynamic instability, CT revealed an increase in aneurysmal size and intraalveolar hemorrhage. The patient was immediately stabilized and an embolization of the right lower pulmonary artery was performed along immunosuppressants and corticoids. Few months later, the patient had a similar hospitalization that led to the embolization of right upper pulmonary artery with success. He is now still being monitored.
HSS is a rare disease affecting mostly male patients between 12 and 40 years of age. Co-morbid conditions like Behçet disease and Hughes Stovin make medical management complicated and very challenging, as the history of this disease is unpredictable.
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Publié par Elsevier Masson SAS.