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CC BY-NC-ND 4.0 · Aorta (Stamford) 2023; 11(04): 156-161
DOI: 10.1055/s-0043-1777994
Case Report

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

Andrea Ascoli Marchetti 
1   Biomedicine and Prevention Department, Vascular Surgery Unit, University of Rome Tor Vergata, Rome, Italy
,
Lorella Belvivere
2   Cardiac Surgery, Department of Experimental Medicine and Surgery, University of Rome Tor Vergata, Rome, Italy
,
Renato Argirò 
3   Radiology Department, Interventional Radiology, University of Rome Tor Vergata, Rome, Italy
,
Barbara Kroegler
4   Department of Medicina dei Sistemi, Rheumatology, Allergology and Clinical Immunology, University of Rome Tor Vergata, Rome, Italy
,
Fabio M. Oddi 
1   Biomedicine and Prevention Department, Vascular Surgery Unit, University of Rome Tor Vergata, Rome, Italy
,
Federico Pennetta 
1   Biomedicine and Prevention Department, Vascular Surgery Unit, University of Rome Tor Vergata, Rome, Italy
,
Alice de Giorgi
1   Biomedicine and Prevention Department, Vascular Surgery Unit, University of Rome Tor Vergata, Rome, Italy
,
Stefano Fazzini 
1   Biomedicine and Prevention Department, Vascular Surgery Unit, University of Rome Tor Vergata, Rome, Italy
,
Daniele Morosetti 
3   Radiology Department, Interventional Radiology, University of Rome Tor Vergata, Rome, Italy
,
Paola Triggianese 
4   Department of Medicina dei Sistemi, Rheumatology, Allergology and Clinical Immunology, University of Rome Tor Vergata, Rome, Italy
,
Elisabetta Greco 
4   Department of Medicina dei Sistemi, Rheumatology, Allergology and Clinical Immunology, University of Rome Tor Vergata, Rome, Italy
,
Arianna D'Antonio
4   Department of Medicina dei Sistemi, Rheumatology, Allergology and Clinical Immunology, University of Rome Tor Vergata, Rome, Italy
,
Ilaria Coccia
5   Department of “Medicina dei Sistemi,” Internal Medicine, University of Rome Tor Vergata, Rome, Italy
,
Manfredi Tesauro
5   Department of “Medicina dei Sistemi,” Internal Medicine, University of Rome Tor Vergata, Rome, Italy
,
Federica Sangiuolo 
6   Biomedicine and Prevention Department, Medical Genetics, University of Rome Tor Vergata, Italy
,
Arnaldo Ippoliti 
1   Biomedicine and Prevention Department, Vascular Surgery Unit, University of Rome Tor Vergata, Rome, Italy
› Author Affiliations Funding None.
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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.

Keywords

Behçet disease - Hughes–Stovin syndrome - pulmonary artery aneurysm - endovascular treatment - genetic syndrome - vena cava filter - deep venous thrombosis - CT scan

Note

This study was presented at the 70th International Congress of the European Society for Cardiovascular and Endovascular Surgery and 7th International Meeting on Aortic Diseases, Liege, Belgium, between June 20–23, 2022.


Supplementary Material



Publication History

Received: 30 October 2022

Accepted: 18 November 2023

Article published online:
26 March 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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