Recurrent Superior Orbital Fissure Syndrome Associated with VEXAS Syndrome

Overview

Journal J Ophthalmic Inflamm Infect

Publisher Springer

Specialty Ophthalmology

Date 2023 Sep 6

PMID 37673972

Authors

Katie Myint

Namritha Patrao

Oana Vonica

Kaveh Vahdani

Affiliations

Soon will be listed here.

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.

Citing Articles

Painful Diplopia as an Initial Presentation of VEXAS Syndrome.

Zewar A, Zollinger-Read C, Dunkley L, Jefferis J Neuroophthalmology. 2024; 48(5):360-363.

PMID: 39145321 PMC: 11321407. DOI: 10.1080/01658107.2024.2324387.

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