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. 2023 Aug;11(4):156-161.
doi: 10.1055/s-0043-1777994. Epub 2024 Mar 26.

A Rare but Fatal Behçet Variant: The Hughes-Stovin Syndrome-Successful Case Report and New Evidence from Literature Review

Andrea Ascoli Marchetti  1 , Lorella Belvivere  2 , Renato Argirò  3 , Barbara Kroegler  4 , Fabio M Oddi  1 , Federico Pennetta  1 , Alice de Giorgi  1 , Stefano Fazzini  1 , Daniele Morosetti  3 , Paola Triggianese  4 , Elisabetta Greco  4 , Arianna D'Antonio  4 , Ilaria Coccia  5 , Manfredi Tesauro  5 , Federica Sangiuolo  6 , Arnaldo Ippoliti  1
Affiliations

A Rare but Fatal Behçet Variant: The Hughes-Stovin Syndrome-Successful Case Report and New Evidence from Literature Review

Andrea Ascoli Marchetti et al. Aorta (Stamford). 2023 Aug.

Abstract

Hughes-Stovin syndrome (HSS) is a rare potentially fatal vasculitis supposedly belonging to the spectrum of Behçet disease without ocular involvement. HSS tends to play by a temporal pattern, starting with thrombosis and followed by formation of pulmonary aneurysms. Since its mortality can reach 25% of cases, early recognition and appropriate therapy represent the major clinical challenges. We describe a rare case of HSS successfully treated via multidisciplinary management by an endovascular approach and immunosuppressive therapy.

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Conflict of interest statement

The authors declare no conflict of interest related to this article.

Figures

Fig. 1
Fig. 1
( A ) Preoperative image computer tomography (CT) maximum intensity projection (MIP) and shaded surface display (SSD) reconstruction showing pulmonary aneurysms: patent right great aneurysm and little left aneurysm ( yellow arrows ). ( B ) Intraoperative selective angiography confirming the nonruptured aneurysm and successful coil embolization ( brown arrow ) with aneurysm exclusion. ( C ) Postoperative image CT MIP and SSD reconstruction showing complete right aneurysm exclusion ( red arrow ) and unmodified left aneurysm ( blue arrows ).
Fig. 2
Fig. 2
Number of confirmed cases of Hughes–Stovin syndrome reported by geographic area.
Fig. 3
Fig. 3
Literature review of follow-up length of patients after Hughes–Stovin syndrome diagnosis: surgical versus medical/surgical therapy.
See this image and copyright information in PMC

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