An update on VEXAS syndrome

A Al-Hakim, S Savic - Expert Review of Clinical Immunology, 2023 - Taylor & Francis
Introduction VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome
is a recently described, late-onset, acquired autoinflammatory disorder caused by mutations …

Clonal hematopoiesis, inflammation, and hematologic malignancy

R Kanagal-Shamanna, DB Beck… - Annual Review of …, 2024 - annualreviews.org
Somatic or acquired mutations are postzygotic genetic variations that can occur within any
tissue. These mutations accumulate during aging and have classically been linked to …

VEXAS syndrome: clinical, hematologic features and a practical approach to diagnosis and management

MJ Koster, TL Lasho, H Olteanu… - American journal of …, 2024 - Wiley Online Library
Abstract VEXAS (Vacuoles, E1 enzyme, X‐linked, Autoinflammatory, Somatic) syndrome is a
newly identified disease caused by somatic alterations in UBA1 which produce a recalcitrant …

Pulmonary manifestations in VEXAS syndrome

MC Moura, M Baqir, YK Tandon, MJ Samec… - Respiratory …, 2023 - Elsevier
Abstract Background Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS)
syndrome is a recently recognized multisystem disorder caused by somatic mutations in the …

Diagnostic capabilities, clinical features, and longitudinal UBA1 clonal dynamics of a nationwide VEXAS cohort

C Gurnari, MR Pascale, A Vitale, E Diral… - American Journal of …, 2024 - Wiley Online Library
VEXAS is a prototypic hemato‐inflammatory disease combining rheumatologic and
hematologic disorders in a molecularly defined nosological entity. In this nationwide study …

[HTML][HTML] Autoimmune manifestations in VEXAS: opportunities for integration and pitfalls to interpretation

A Bruno, C Gurnari, T Alexander, JA Snowden… - Journal of Allergy and …, 2023 - Elsevier
VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a novel entity
manifesting with a multiplicity of clinical features. Somatic mutations of the UBA1 gene in …

VEXAS syndrome, a new kid on the block of auto-inflammatory diseases: A hematologist's point of view

M Heiblig, B Patel, Y Jamilloux - Best Practice & Research Clinical …, 2023 - Elsevier
The recently discovered VEXAS syndrome is caused by the clonal expansion of
hematopoietic stem or progenitor cells with acquired mutations in UBA1 gene, which …

Allogeneic hematopoietic cell transplantation for VEXAS syndrome: results of a multicenter study of the EBMT

C Gurnari, L Koster, L Baaij, M Heiblig… - Blood …, 2024 - ashpublications.org
Somatic mutations of the X-linked UBA1 gene in myeloid progenitors constitute the
molecular underpinnings of VEXAS, typically occurring in men during the sixth decade of …

[HTML][HTML] VEXAS Syndrome—Diagnostic Clues for the Dermatologist and Gaps in Our Current Understanding: A Narrative Review

LT Nicholson, EW Cowen, D Beck, M Ferrada… - JID Innovations, 2024 - Elsevier
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome is a newly recognized,
acquired autoinflammatory disorder with broad systemic implications and a poor global …

[HTML][HTML] Association between myeloid disorders and adult onset-inflammatory syndromes, successful treatment with JAK-inhibitors: Case series and literature review

R Mishra, C Calabrese, AG Jain, A Singh - Leukemia Research, 2024 - Elsevier
Approximately one-third of patients with myeloid disorders like myelodysplastic syndrome
(MDS) and chronic myelomonocytic leukemia (CMML) exhibit inflammatory and autoimmune …