Results 21 to 30 of about 929 (159)

An update on VEXAS syndrome

Expert Review of Clinical Immunology, 2022
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently described, late-onset, acquired autoinflammatory disorder caused by mutations in the UBA1 gene. The various clinical manifestations of VEXAS broadly divided into inflammatory or haematological.
Al-Hakim, A, Savic, S
openaire   +3 more sources

Patients with VEXAS diagnosed in a Danish tertiary rheumatology setting have highly elevated inflammatory markers, macrocytic anaemia and negative autoimmune biomarkers

RMD Open, 2022
Background Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) is an autoinflammatory condition with overlapping features of rheumatology and haematology caused by somatic mutations in the UBA1 gene.
Ellen-Margrethe Hauge, Anne Troldborg, Fruzsina Szabados, Mads Nyhuus Bendix Rasch, Jens Magnus Bernth Jensen, Kirstine Overgaard Nielsen   +5 more
doaj   +1 more source

Hypomethylating agents in vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome (VEXAS): A systematic review. [PDF]

Br J Haematol
Summary VEXAS syndrome (vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic) is an X‐linked, systemic, haemato‐inflammatory syndrome caused by somatic mutations in the UBA1 gene. No standardized treatment guidelines exist, but evidence is emerging that treatment with hypomethylating agents (HMAs) can induce improvement of the inflammatory symptoms,
Hoff FW, Trikha R, Groarke EM, Patel BA.
europepmc   +2 more sources

Vasculitis associated with VEXAS syndrome: A literature review

Frontiers in Medicine, 2022
Vasculitis is an inflammatory disorder of the blood vessels that causes damage to a wide variety of organs through tissue ischemia. Vasculitis is classified according to the size (large, medium, or small) of the blood vessels.
Ryu Watanabe, Manami Kiji, Motomu Hashimoto   +2 more
doaj   +1 more source

Performance Characteristics of Anti-Collagen II Antibodies in Relapsing Polychondritis and Related Diseases: Prospective Analysis, Systematic Review, and Meta-Analysis. [PDF]

Arthritis Care Res (Hoboken)
Objective Relapsing polychondritis (RP) is a rare disease defined by recurrent cartilaginous inflammation. Anti–collagen II (Col2) antibodies have been proposed as a diagnostic biomarker for RP, but their performance characteristics are not well defined.
Stonick K, Ferrada MA, Fike A, Quinn KA, Turturice BA, Stein C, Grayson PC.   +6 more
europepmc   +2 more sources

VEXAS-Syndrom

Zeitschrift für Rheumatologie
The VEXAS (vacuoles, E1 enzyme, X‑linked, autoinflammatory, somatic) syndrome is an acquired autoinflammatory disease, which is based on somatic mutations in the UBA1 gene and described as an hematoinflammatory disease. It is clinically expressed as an inflammatory systemic disease with general vegetative symptoms and organ manifestations of the skin ...
M, Krusche, N, Ruffer, I, Kötter, K, Sockel   +3 more
  +5 more sources

VEXAS syndrome in myelodysplastic syndrome with autoimmune disorder

Experimental Hematology & Oncology, 2021
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly-described adult-onset inflammatory syndrome characterized by vacuoles in myeloid and erythroid precursor cells and somatic mutations affecting methionine-41 (p.Met41) in
Huijun Huang, Wenjun Zhang, Wenyu Cai, Jinqin Liu, Huijun Wang, Tiejun Qin, Zefeng Xu, Bing Li, Shiqiang Qu, Lijuan Pan, Gang Huang, Robert Peter Gale, Zhijian Xiao   +12 more
doaj   +1 more source

Recurrent superior orbital fissure syndrome associated with VEXAS syndrome

Journal of Ophthalmic Inflammation and Infection, 2023
Purpose To describe a case of recurrent orbital inflammation and superior orbital fissure syndrome associated with VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome. Case presentation VEXAS syndrome is a recently identified multi-
Katie Myint, Namritha Patrao, Oana Vonica, Kaveh Vahdani   +3 more
doaj   +1 more source

Clinical Images: Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome presenting as aseptic pustular dermatosis: A rare clinical manifestation. [PDF]

Arthritis Rheumatol
Arthritis &Rheumatology, Volume 78, Issue 3, Page 763-763, March 2026.
Chen P, Briançon H, Eshagh D, Szwebel TA, Terrier B, Sohier P, Birsen R.   +6 more
europepmc   +2 more sources

Comparative Efficacy and Safety of Anakinra and Canakinumab in Patients With VEXAS Syndrome: An International Multicenter Study. [PDF]

Arthritis Rheumatol
Objective The aim of this study was to compare differences in clinical response, drug survival, and adverse event rates between anakinra and canakinumab in VEXAS (vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic) syndrome. Methods This multicenter international study includes patients with VEXAS from France, Israel, and Italy treated with ...
Eviatar T, Capelusnik D, Campochiaro C, Lacombe V, Jachiet V, Zisapel M, Sagy I, Tayer-Shifman OE, Ozeri D, Kivity S, Tomelleri A, Terrier B, Peleg H, Comont T, Sacre K, Woaye-Hune P, Arnaud L, Lazaro E, Grobost V, Lifermann F, Samson M, Ardois S, Garnier A, Maria A, Cantagrel A, Meyer A, Bouaziz JD, Heiblig M, Dagna L, Diral E, Kosmider O, Elkayam O, Hadjadj J, Georgin-Lavialle S, Fain O, Mekinian A.   +35 more
europepmc   +2 more sources