ArticleZeitschrift fur Rheumatologie2024
[Macrocytic anemia and polychondritis: VEXAS syndrome].
Article in Zeitschrift fur Rheumatologie, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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2 citing papers in PubMed, 3 citations in OpenAlex.
- VEXAS Syndrome-Diagnostic Clues for the Dermatologist and Gaps in Our Current Understanding: A Narrative Review.JID innovations : skin science from molecules to population health · 2024Review
- VEXAS syndrome: a newly discovered systemic rheumatic disorder.Reumatologia · 2023Review
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10 authors at 2 institutions in 1 country.
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Abstract
An adult-onset autoinflammatory syndrome caused by somatic mutations in the UBA1 gene on the X chromosome was first reported in 2020. This VEXAS syndrome (acronym for vacuoles, E1 enzyme, X‑linked, autoinflammatory, somatic) is characterized by an overlap of rheumatic inflammatory diseases with separate hematologic abnormalities. A substantial number of affected patients suffer from treatment refractory relapsing polychondritis and nearly always show signs of macrocytic anemia. This case report illustrates the diagnostic key points to recognizing patients with VEXAS syndrome.
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