VEXAS(vacuoles,E1 enzyme,X-linked,autoinflammatory,somatic)syndrome is a severe and progressive disease,characterized by clinical features that bridge rheumatologic and hematologic conditions.[1]VEXAS syndrome is a ra...VEXAS(vacuoles,E1 enzyme,X-linked,autoinflammatory,somatic)syndrome is a severe and progressive disease,characterized by clinical features that bridge rheumatologic and hematologic conditions.[1]VEXAS syndrome is a rare condition that was not reported until 2020.[2]Since then,interest among dermatologists,hematologists,and rheumatologists with published works has increased,[3]but none of them reported in the emergency setting,nor have any cases arisen following COVID-19 infection.展开更多
VEXAS syndrome(vacuoles,E1 enzyme,X-linked,autoinflammatory,somatic)is an adult-onset and treatment-refractory inflammatory disease,caused by acquired mutations in UBA1(ubiquitin-like modifier activating enzyme 1),an ...VEXAS syndrome(vacuoles,E1 enzyme,X-linked,autoinflammatory,somatic)is an adult-onset and treatment-refractory inflammatory disease,caused by acquired mutations in UBA1(ubiquitin-like modifier activating enzyme 1),an X-linked gene encoding an E1 enzyme of the ubiquitin-proteasome system.1 The disease occurs predominantly in elderly male patients and has heterogeneous but expanding clinical features that include fever,characteristic vacuoles in hematopoietic precursors,cytopenias,and chondritis.As such,VEXAS patients may be initially diagnosed with relapsing polychondritis,myelodysplastic syndrome,and other syndromes.展开更多
基金National High Level Hospital Clinical Research Funding(Scientific Research Seed Fund of Peking University First Hospital).
文摘VEXAS(vacuoles,E1 enzyme,X-linked,autoinflammatory,somatic)syndrome is a severe and progressive disease,characterized by clinical features that bridge rheumatologic and hematologic conditions.[1]VEXAS syndrome is a rare condition that was not reported until 2020.[2]Since then,interest among dermatologists,hematologists,and rheumatologists with published works has increased,[3]but none of them reported in the emergency setting,nor have any cases arisen following COVID-19 infection.
基金funded by the National Natural Sciences Foundation of China(No.82271280 to Y.T.82301433 to J.J.W.)+3 种基金Hunan Provincial Natural Science Foundation of China(No.2022JJ40824 to J.J.W.)Scientific Research Project of Hunan Provincial Health Commission(China)(No.B202303070054 to Y.T.)Talents Startup Fund(China)(No.2209090550 to Y.T.),Youth Science Fund(China)(No.2021Q04 to J.J.W.)the Project Program of National Clinical Research Center for Geriatric Disorders of Xiangya Hospital,Central South University,Changsha,China(No.2022LNJJ14 to H.J.Z.).
文摘VEXAS syndrome(vacuoles,E1 enzyme,X-linked,autoinflammatory,somatic)is an adult-onset and treatment-refractory inflammatory disease,caused by acquired mutations in UBA1(ubiquitin-like modifier activating enzyme 1),an X-linked gene encoding an E1 enzyme of the ubiquitin-proteasome system.1 The disease occurs predominantly in elderly male patients and has heterogeneous but expanding clinical features that include fever,characteristic vacuoles in hematopoietic precursors,cytopenias,and chondritis.As such,VEXAS patients may be initially diagnosed with relapsing polychondritis,myelodysplastic syndrome,and other syndromes.
