Results 21 to 30 of about 2,223 (207)

Thrombosis in VEXAS syndrome [PDF]

Journal of Thrombosis and Thrombolysis, 2021
VEXAS syndrome, an autoinflammatory syndrome due to a Ubiquitin Like Modifier Activating Enzyme 1 (UBA1) somatic mutation, has a high thrombotic burden. We report a case of a 69-year-old male that was diagnosed with VEXAS syndrome who developed venous thromboembolism (VTE).
Thet Mon Oo, Jie Tian Jeanette Koay, Siew Fen Lee, Shang Ming Samuel Lee, Xin Rong Lim, Bingwen Eugene Fan   +5 more
openaire   +2 more sources

Distinction between clonal and paraclonal cutaneous involvements in VEXAS syndrome

Experimental Hematology & Oncology, 2022
VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome is an inflammatory disorder with hematological and systemic features. A recent study demonstrated that the dermal infiltrate in neutrophilic dermatosis from VEXAS patients is ...
Valentin Lacombe, Annaelle Beucher, Geoffrey Urbanski, Yannick Le Corre, Laurane Cottin, Anne Croué, Anne Bouvier   +6 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

Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients

RMD Open, 2022
Objective A new adult-onset autoinflammatory syndrome has been described, named VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic).
Vincent Jachiet, Benjamin Terrier, Laurence Bouillet, Claire de Moreuil, Joris Galland, Mathilde Devaux, Mathieu Gerfaud-Valentin, Alexandre Maria, Jean Schmidt, Sylvain Audia, Guillaume Denis, Mohamed-Yacine Khitri, Alexis F Guedon, Maelle le Besnerais, Jean Sebastien Allain, Vincent Grobost, Olivier Kosmider, Anael Dumont, Benjamin Subran, Paola Marianetti-Guingel, Sylvain Palat, Marielle Roux-Sauvat, Pierre Hirsch   +22 more
doaj   +1 more source

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

American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel. [PDF]

Arthritis Rheumatol
Objective Vacuoles E1 enzyme X‐linked autoinflammatory somatic syndrome (VEXAS) is a recently identified rare genetic disorder associated with somatic mutations in the UBA1 gene. VEXAS presents with a combination of inflammatory and hematologic manifestations, leading to increased morbidity and mortality.
Mekinian A, Georgin-Lavialle S, Ferrada MA, Savic S, Koster MJ, Kosmider O, Comont T, Heiblig M, Arostegui JI, Bosco A, Bourguiba R, Calvo KR, Cargo C, Cattaneo C, Chasset F, Coelho H, Campochiaro C, Crisafulli F, Ducharme-Benard S, Faria R, Franceschini F, Frassi M, Groarke EM, Gurnari C, Hakobyan Y, Jamilloux Y, Jurcut C, Kirino Y, Kulasekararaj A, Kunimoto H, Madigan LM, Mann HF, Marvisi C, Milchert M, Morais S, Sockel K, Muratore F, Nakajima H, Patnaik MM, Regadas L, Robin M, Rutgers A, Salvarani C, Sammel AM, Seebach J, Sujobert P, Tomelleri A, Urbanski G, Vandergheynst F, Vieira R, Viswanatha DS, Więsik-Szewczyk E, Diral E, Terrier B, Patel BA, Fenaux P, Grayson PC, Beck DB, International VEXAS working group, and with endorsement of EuroBloodNet, the European Reference Network in Rare Hematological Diseases.   +60 more
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

VEXAS syndrome

International Journal of Hematology
Abstract VEXAS syndrome is a recently identified, adult-onset autoinflammatory disease caused by somatic mutations in UBA1. UBA1 is an X-linked gene encoding E1 ubiquitin activating enzyme and its mutation in hematopoietic stem and progenitor cells leads to their clonal expansion and myeloid-skewed differentiation. UBA1 mutations in VEXAS are
Hideaki Nakajima, Hiroyoshi Kunimoto
  +5 more sources

Una nota sobre el teorema de Gleason-Kahane-Zelasko [PDF]

, 1980
Siendo A un álgebra conmutativa compleja, localmente convexa Hausdorff de inversión continua (no necesariamente semicompleta), se demuestra que todo hiperplano de A que solo contenga elemento singulares, es un ideal maximal de
Pérez González, Fernando
core   +2 more sources

VEXAS syndrome [PDF]

International Journal of Hematology, 2022
Kaori Uchino, Jo Kanasugi, Megumi Enomoto, Fumiya Kitamura, Naomi Tsuchida, Yuri Uchiyama, Ayaka Maeda, Yohei Kirino, Naomichi Matsumoto, Akiyoshi Takami   +9 more
  +5 more sources