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Journal of Ophthalmic Inflammation and Infection
Published in: Journal of Ophthalmic Inflammation and Infection 1/2023

Open Access 01-12-2023 | Ophthalmoparesis | Brief Report

Recurrent superior orbital fissure syndrome associated with VEXAS syndrome

Authors: Katie Myint, Namritha Patrao, Oana Vonica, Kaveh Vahdani

Published in: Journal of Ophthalmic Inflammation and Infection | Issue 1/2023

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Abstract

Purpose

To describe a case of recurrent orbital inflammation and superior orbital fissure syndrome associated with VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome.

Case presentation

VEXAS syndrome is a recently identified multi-system inflammatory disease of late adult onset. The authors describe the case of a 76-year-old man who presented with recurrent episodes of orbital inflammation, with superior orbital fissure syndrome, dacryoadenitis and orbital myositis. He had a constellation of systemic disorders including recurrent chest infections, congestive cardiac failure, pulmonary emboli and skin rashes. The underlying diagnosis of VEXAS syndrome was confirmed by genetic testing, which revealed the UBA1 mutation.

Conclusion

VEXAS syndrome should be considered in the differential diagnosis of orbital inflammatory disease associated with multi-system inflammatory disorders.
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Metadata
Title
Recurrent superior orbital fissure syndrome associated with VEXAS syndrome
Authors
Katie Myint
Namritha Patrao
Oana Vonica
Kaveh Vahdani
Publication date
01-12-2023
Publisher
Springer Berlin Heidelberg
Published in
Journal of Ophthalmic Inflammation and Infection / Issue 1/2023
Electronic ISSN: 1869-5760
DOI
https://doi.org/10.1186/s12348-023-00362-1

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