VEXAS syndrome: a newly discovered systemic rheumatic disorder

VEXAS syndrome is an adult-onset autoinflammatory disease associated with hematologic symptoms. The disease affects primarily males, and leads to death of a significant proportion of the patients. VEXAS syndrome is caused by a somatic mutation of the UBA1 gene in hematopoietic progenitor cells. The...

Descripción completa

Detalles Bibliográficos
Autor principal: Kucharz, Eugenisz J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie 2023
Materias:
Review Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10201379/
https://www.ncbi.nlm.nih.gov/pubmed/37223371
http://dx.doi.org/10.5114/reum/163090
Descripción
Sumario:VEXAS syndrome is an adult-onset autoinflammatory disease associated with hematologic symptoms. The disease affects primarily males, and leads to death of a significant proportion of the patients. VEXAS syndrome is caused by a somatic mutation of the UBA1 gene in hematopoietic progenitor cells. The clinical picture of the syndrome consists of a number of organ manifestations including those akin to rheumatic diseases, arthritis, myalgia, vasculitis and chondritis.

Ejemplares similares