Case 2-2022: A 70-Year-Old Man with a Recurrent Left Pleural Effusion

Letter Rheumatology

VEXAS syndrome with systemic lupus erythematosus: expanding the spectrum of associated conditions

Aman Sharma et al.

ARTHRITIS & RHEUMATOLOGY (2022)

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Letter Hematology

Therapeutic options in VEXAS syndrome: insights from a retrospective series

Estelle Bourbon et al.

BLOOD (2021)

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Letter Rheumatology

VEXAS syndrome: still expanding the clinical phenotype

Artem Oganesyan et al.

RHEUMATOLOGY (2021)

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Article Rheumatology

Somatic Mutations in UBA1 Define a Distinct Subset of Relapsing Polychondritis Patients With VEXAS

Marcela A. Ferrada et al.

Summary: Somatic mutations in UBA1 have been identified in a subset of patients with relapsing polychondritis (RP), leading to a newly defined syndrome called VEXAS-RP. Patients with VEXAS-RP have distinct clinical and immunologic features, including a male predominance, onset in midlife or later, specific symptoms, and higher mortality compared to typical RP patients. Early identification and intervention are important in managing VEXAS-RP due to its associated higher mortality rate.

ARTHRITIS & RHEUMATOLOGY (2021)

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Letter Oncology

Prevalence of UBA1 mutations in MDS/CMML patients with systemic inflammatory and auto-immune disease

Lin-Pierre Zhao et al.

LEUKEMIA (2021)

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Article Hematology

Benign and malignant hematologic manifestations in patients with VEXAS syndrome due to somatic mutations in UBA1

Ifeyinwa Emmanuela Obiorah et al.

Summary: Patients with VEXAS syndrome exhibit severe autoinflammatory and rheumatologic manifestations, with most also having bone marrow abnormalities such as myelodysplastic syndrome and multiple myeloma. Unique features include prominent cytoplasmic vacuoles in bone marrow and higher prevalence of thrombocytopenia and neutropenia in patients progressing to MDS.

BLOOD ADVANCES (2021)

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Letter Medicine, General & Internal