A Rare but Fatal Behçet Variant: The Hughes–Stovin Syndrome—Successful Case Report and New Evidence from Literature Review-Reference-Cited by-同舟云学术

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

Published:2023-08 Issue:04 Volume:11 Page:156-161
ISSN:2325-4637
Container-title:AORTA
language:en
Short-container-title:Aorta (Stamford)

Author:

Ascoli Marchetti Andrea¹^ORCID,Belvivere Lorella²,Argirò Renato³^ORCID,Kroegler Barbara⁴,Oddi Fabio M.¹^ORCID,Pennetta Federico¹^ORCID,Giorgi Alice de¹,Fazzini Stefano¹^ORCID,Morosetti Daniele³^ORCID,Triggianese Paola⁴^ORCID,Greco Elisabetta⁴^ORCID,D'Antonio Arianna⁴,Coccia Ilaria⁵,Tesauro Manfredi⁵,Sangiuolo Federica⁶^ORCID,Ippoliti Arnaldo¹^ORCID

Affiliation:

1. Biomedicine and Prevention Department, Vascular Surgery Unit, University of Rome Tor Vergata, Rome, Italy

2. Cardiac Surgery, Department of Experimental Medicine and Surgery, University of Rome Tor Vergata, Rome, Italy

3. Radiology Department, Interventional Radiology, University of Rome Tor Vergata, Rome, Italy

4. Department of Medicina dei Sistemi, Rheumatology, Allergology and Clinical Immunology, University of Rome Tor Vergata, Rome, Italy

5. Department of “Medicina dei Sistemi,” Internal Medicine, University of Rome Tor Vergata, Rome, Italy

6. Biomedicine and Prevention Department, Medical Genetics, University of Rome Tor Vergata, Italy

Abstract

AbstractHughes–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.

Publisher

Georg Thieme Verlag KG

Link

http://www.thieme-connect.de/products/ejournals/pdf/10.1055/s-0043-1777994.pdf

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