Large-Vessel Vasculitis With Autoimmune Myelodysplastic Syndrome: An Uncommon Case of Large-Vessel Vasculitis With Cytopenia That Is Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic Syndrome (VEXAS) Negative.

Benjoe George P S, Ann John, Smitha Krishnamoorthy, Caroline Grace Jacob, Saket Chandak

Abstract readCase Reports

In one paragraph

Article in Cureus, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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5 authors.

Benjoe George P SInternal Medicine, Amrita Institute of Medical Sciences and Research Centre, Ernakulam, IND.

Ann JohnInternal Medicine, Amrita Institute of Medical Sciences and Research Centre, Ernakulam, IND.

Smitha KrishnamoorthyInternal Medicine, Amrita Institute of Medical Sciences and Research Centre, Ernakulam, IND.

Caroline Grace JacobInternal Medicine, Amrita Institute of Medical Sciences and Research Centre, Ernakulam, IND.

Saket ChandakInternal Medicine, Amrita Institute of Medical Sciences and Research Centre, Ernakulam, IND.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Autoimmune myelodysplastic syndrome (MDS) associated with large-vessel vasculitis (LVV) is an extremely rare clinical presentation. We report the case of an 80-year-old lady presenting with prolonged fever, cytopenias, and inflammatory features, later diagnosed as LVV with autoimmune MDS. Extensive evaluation excluded infectious, malignant, and other autoimmune etiologies, including VEXAS syndrome. The patient responded favorably to corticosteroids and immunosuppressive therapy, showing hematologic and clinical improvement. This case highlights the importance of considering autoimmune bone marrow disorders as a potential cause of unexplained fever with cytopenia and large vessel vasculitis in elderly individuals.

Indexed as

autoimmune myelodysplastic syndromecytopeniagiant cell arteritis-like vasculitislarge vessel vasculitisvexas-negative

Identifiers

PMID41822647

DOI10.7759/cureus.103259

PMCPMC12978621

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