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

Yasser Emad (Corresponding Author), Yasser Ragab, A. El-Marakbi, A. Saad, Osama Ibrahim, A. Abd-Elhalim, H. Wl-Santawi, Johannes J. Rasker

    Research output: Contribution to journalArticleAcademicpeer-review

    14 Citations (Scopus)
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    Abstract

    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.
    Original languageEnglish
    Pages (from-to)365-371
    Number of pages6
    JournalZeitschrift fur Rheumatologie
    Volume78
    Issue number4
    Early online date14 Mar 2019
    DOIs
    Publication statusPublished - May 2019

    Keywords

    • UT-Hybrid-D
    • Hughes–Stovin syndrome
    • Incomplete Behçet
    • Aortitis
    • Pulmonary artery aneurysms
    • Arterial vasculitis in Hughes–Stovin syndrome
    • Thrombophlebitis

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