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VEXAS Syndrome Presenting With Pleuritis
Makoto Ito1,2, Yasushi Murakami3, Michita Suzuki4
1Department of Hematology Tokoname City Hospital Tokoname Japan.
Clinical Case Reports
|December 5, 2025
Summary
VEXAS syndrome, characterized by systemic inflammation and cytopenias in elderly men, can be caused by somatic UBA1 mutations. Pulmonary issues like pleuritis are key indicators, prompting early corticosteroid treatment.
Area of Science:
- Hematology
- Genetics
- Pulmonology
Background:
- VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is an emerging condition.
- It affects elderly males, presenting with systemic inflammation, cytopenias, and bone marrow vacuolization.
- Pulmonary involvement is increasingly recognized.
Purpose of the Study:
- To highlight the association between VEXAS syndrome and somatic UBA1 mutations.
- To emphasize pulmonary manifestations, such as pleuritis, as potential early signs.
- To underscore the importance of early diagnosis for timely treatment.
Main Methods:
- Case identification of elderly men with characteristic symptoms.
- Genetic analysis to detect somatic UBA1 mutations.
- Clinical review focusing on pulmonary findings.
Main Results:
- Somatic UBA1 mutations were identified in affected individuals.
- Pleuritis was a significant pulmonary manifestation, even without classic chondritis.
- Early recognition of these symptoms correlated with prompt corticosteroid initiation.
Conclusions:
- VEXAS syndrome should be considered in elderly men with unexplained inflammation, cytopenias, and bone marrow vacuolization.
- Pulmonary involvement, particularly pleuritis, is a crucial diagnostic clue.
- Timely diagnosis and treatment with corticosteroids can improve outcomes.
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