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

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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

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Lower Extremity
Cyclophosphamide
Aneurysm
Blood Vessels
Ischemia
Intracranial Sinus Thrombosis
Iliac Vein
Limb Salvage
Thrombophlebitis
Venae Cavae
Femoral Vein
Pulmonary Veins
Iliac Artery
Jugular Veins
Femoral Artery
Venous Thrombosis
Pulmonary Artery
Adrenal Cortex Hormones
Thrombosis
Arteries

Keywords

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

Cite this

Emad, Yasser ; Ragab, Yasser ; El-Marakbi, A. ; Saad, A. ; Ibrahim, Osama ; Abd-Elhalim, A. ; Wl-Santawi, H. ; Rasker, Johannes J. / A case of Hughes–Stovin syndrome (incomplete Behçet’s disease) with extensive arterial involvement : Unmasking the true face of a rare syndrome. In: Zeitschrift fur Rheumatologie. 2019 ; Vol. 78, No. 4. pp. 365-371.
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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 Beh{\cc}et’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.",
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A case of Hughes–Stovin syndrome (incomplete Behçet’s disease) with extensive arterial involvement : Unmasking the true face of a rare syndrome. / Emad, Yasser (Corresponding Author); Ragab, Yasser; El-Marakbi, A.; Saad, A.; Ibrahim, Osama; Abd-Elhalim, A.; Wl-Santawi, H.; Rasker, Johannes J.

In: Zeitschrift fur Rheumatologie, Vol. 78, No. 4, 05.2019, p. 365-371.

Research output: Contribution to journalArticleAcademicpeer-review

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T1 - A case of Hughes–Stovin syndrome (incomplete Behçet’s disease) with extensive arterial involvement

T2 - Unmasking the true face of a rare syndrome

AU - Emad, Yasser

AU - Ragab, Yasser

AU - El-Marakbi, A.

AU - Saad, A.

AU - Ibrahim, Osama

AU - Abd-Elhalim, A.

AU - Wl-Santawi, H.

AU - Rasker, Johannes J.

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N2 - 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.

AB - 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.

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KW - Incomplete Behçet

KW - Aortitis

KW - Pulmonary artery aneurysms

KW - Arterial vasculitis in Hughes–Stovin syndrome

KW - Thrombophlebitis

U2 - 10.1007/s00393-019-0618-7

DO - 10.1007/s00393-019-0618-7

M3 - Article

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JO - Zeitschrift fur Rheumatologie

JF - Zeitschrift fur Rheumatologie

SN - 0340-1855

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ER -