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VEXAS syndrome: Current clinical, diagnostic and treatment approaches.
Kobak S. Kobak S. Intractable Rare Dis Res. 2023 Aug;12(3):170-179. doi: 10.5582/irdr.2023.01020. Intractable Rare Dis Res. 2023. PMID: 37662628 Free PMC article. Review.
Genetic testing to identify the UBA1 gene mutation is essential for definitive diagnosis. Currently, there is no known cure for VEXAS syndrome, and treatment mainly focuses on managing the symptoms. ...In this review article, the clinical, diagnostic and t
Genetic testing to identify the UBA1 gene mutation is essential for definitive diagnosis. Currently, there is no known cure for VEXAS
An update on VEXAS syndrome.
Al-Hakim A, Savic S. Al-Hakim A, et al. Expert Rev Clin Immunol. 2023 Feb;19(2):203-215. doi: 10.1080/1744666X.2023.2157262. Epub 2022 Dec 26. Expert Rev Clin Immunol. 2023. PMID: 36537591 Free article. Review.
A detailed account of its various clinical manifestations and disease mimics is provided. Current treatment and management options are discussed. EXPERT OPINION: New rare variants in UBA1 and VEXAS-like UBA1 negative cases are reported. ...VEXAS-specific hema …
A detailed account of its various clinical manifestations and disease mimics is provided. Current treatment and management options ar …
VEXAS syndrome.
Grayson PC, Patel BA, Young NS. Grayson PC, et al. Blood. 2021 Jul 1;137(26):3591-3594. doi: 10.1182/blood.2021011455. Blood. 2021. PMID: 33971000 Free PMC article. Review.
VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a monogenic disease of adulthood caused by somatic mutations in UBA1 in hematopoietic progenitor cells. ...VEXAS syndrome represents a prototype for a new class of diseases...
VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a monogenic disease of adulthood caused by somati
JAK inhibitors for the treatment of VEXAS syndrome.
Bindoli S, Baggio C, Doria A, Bertoldo E, Sfriso P. Bindoli S, et al. Exp Biol Med (Maywood). 2023 May;248(5):394-398. doi: 10.1177/15353702231165030. Epub 2023 May 26. Exp Biol Med (Maywood). 2023. PMID: 37243340 Free PMC article. Review.
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a novel described autoinflammatory entity for which the diagnosis is defined by somatic mutations of the UBA1 X-linked gene in hematopoietic progenitor cells. ...However, recently described …
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a novel described autoinflammatory entity for whi …
VEXAS syndrome: Clinical manifestations, diagnosis, and treatment.
Loeza-Uribe MP, Hinojosa-Azaola A, Sánchez-Hernández BE, Crispín JC, Apodaca-Chávez E, Ferrada MA, Martín-Nares E. Loeza-Uribe MP, et al. Reumatol Clin (Engl Ed). 2024 Jan;20(1):47-56. doi: 10.1016/j.reumae.2023.12.004. Epub 2023 Dec 29. Reumatol Clin (Engl Ed). 2024. PMID: 38160120 Free article. Review.
Patients with VEXAS syndrome exhibit inflammatory and hematological manifestations that can lead to clinical diagnoses such as relapsing polychondritis, polyarteritis nodosa, Sweet syndrome, and myelodysplastic syndrome. ...The aim of this review is to provide an ov …
Patients with VEXAS syndrome exhibit inflammatory and hematological manifestations that can lead to clinical diagnoses such as …
VEXAS Syndrome-Review.
Zhang Y, Dong X, Wang H. Zhang Y, et al. Glob Med Genet. 2023 Jul 10;10(3):133-143. doi: 10.1055/s-0043-1770958. eCollection 2023 Sep. Glob Med Genet. 2023. PMID: 37501758 Free PMC article. Review.
To date, patients respond poorly to immune suppressive drugs, except high-dose glucocorticoids, and no treatment guidelines have been established. Given the high mortality rate, VEXAS syndrome needs to be taken seriously by physicians in all specialties. This …
To date, patients respond poorly to immune suppressive drugs, except high-dose glucocorticoids, and no treatment guidelines have been …
How to treat VEXAS syndrome: a systematic review on effectiveness and safety of current treatment strategies.
Boyadzhieva Z, Ruffer N, Kötter I, Krusche M. Boyadzhieva Z, et al. Rheumatology (Oxford). 2023 Nov 2;62(11):3518-3525. doi: 10.1093/rheumatology/kead240. Rheumatology (Oxford). 2023. PMID: 37233149
OBJECTIVES: To evaluate the effectiveness and safety of current treatment strategies for the vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome. ...Data on adverse events were available for 67 patients (67/116, 57.8%) and included: pneu …
OBJECTIVES: To evaluate the effectiveness and safety of current treatment strategies for the vacuoles, E1 enzyme, X-linked, autoinfla …
VEXAS Syndrome-A Review of Pathophysiology, Presentation, and Prognosis.
Koster MJ, Samec MJ, Warrington KJ. Koster MJ, et al. J Clin Rheumatol. 2023 Sep 1;29(6):298-306. doi: 10.1097/RHU.0000000000001905. Epub 2022 Oct 17. J Clin Rheumatol. 2023. PMID: 36251488 Review.
VEXAS ( V acuoles, E 1 enzyme, X -linked, A utoinflammatory, S omatic) syndrome is a newly identified disease caused by somatic mutations in the UBA1 gene resulting in refractory autoinflammatory features, frequently accompanied by cytopenias. ...The pathophysiology, clini
VEXAS ( V acuoles, E 1 enzyme, X -linked, A utoinflammatory, S omatic) syndrome is a newly identified disease caused by somatic mutat
Azacytidine Treatment for VEXAS Syndrome.
Raaijmakers MHGP, Hermans M, Aalbers A, Rijken M, Dalm VASH, van Daele P, Valk PJM. Raaijmakers MHGP, et al. Hemasphere. 2021 Nov 17;5(12):e661. doi: 10.1097/HS9.0000000000000661. eCollection 2021 Dec. Hemasphere. 2021. PMID: 34805767 Free PMC article. No abstract available.
Treatment Options for VEXAS Syndrome: Rapid Review.
Authors; Goring S, Horton J. Authors, et al. Ottawa (ON): Canadian Agency for Drugs and Technologies in Health; 2022 Nov. Ottawa (ON): Canadian Agency for Drugs and Technologies in Health; 2022 Nov. PMID: 38620405 Free Books & Documents. Review. No abstract available.
127 results