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2021 6
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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.
Although the prevalence of this syndrome is yet unknown, understanding the clinical phenotype can assist clinicians in prompt recognition of cases among patients with glucocorticoid-responsive but immunosuppressive-resistant inflammatory symptoms. The pathophysiology, c …
Although the prevalence of this syndrome is yet unknown, understanding the clinical phenotype can assist clinicians in prompt recogni …
French practical guidelines for the diagnosis and management of relapsing polychondritis.
Arnaud L, Costedoat-Chalumeau N, Mathian A, Sailler L, Belot A, Dion J, Morel N, Moulis G; Collaborators. Arnaud L, et al. Rev Med Interne. 2023 Jun;44(6):282-294. doi: 10.1016/j.revmed.2023.05.005. Epub 2023 May 24. Rev Med Interne. 2023. PMID: 37236870 Free article. Review.
No laboratory test is specific of relapsing polychondritis, the diagnosis is usually based on clinical evidence and the elimination of differential diagnoses. Relapsing polychondritis is a long-lasting and often unpredictable disease, evolving in the form of relapses inter …
No laboratory test is specific of relapsing polychondritis, the diagnosis is usually based on clinical evidence and the elimination o …
Pulmonary manifestations in VEXAS syndrome.
Casal Moura M, Baqir M, Tandon YK, Samec MJ, Hines AS, Reichard KK, Mangaonkar AA, Go RS, Warrington KJ, Patnaik MM, Koster MJ, Ryu JH. Casal Moura M, et al. Respir Med. 2023 Jul;213:107245. doi: 10.1016/j.rmed.2023.107245. Epub 2023 Apr 14. Respir Med. 2023. PMID: 37062498
BACKGROUND: Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a recently recognized multisystem disorder caused by somatic mutations in the UBA1 gene. METHODS: A retrospective cohort study was conducted on all patients with VEXAS …
BACKGROUND: Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a recently recognized multisystem dis …
Could it be VEXAS?
Guerineau H, Kohn M, Al Hamoud A, Sellier J, Osman J, Cabannes-Hamy A. Guerineau H, et al. Ann Hematol. 2024 Jun;103(6):2169-2171. doi: 10.1007/s00277-024-05750-8. Epub 2024 Apr 9. Ann Hematol. 2024. PMID: 38589717
We report the case of the youngest patient described with VEXAS syndrome associated with MDS-IB1, successfully treated with azacitidine-venetoclax and allogeneic stem cell transplant....
We report the case of the youngest patient described with VEXAS syndrome associated with MDS-IB1, successfully treated with az …
Successful azacitidine therapy for myelodysplastic syndrome associated with VEXAS syndrome.
Kataoka A, Mizumoto C, Kanda J, Iwasaki M, Sakurada M, Oka T, Fujimoto M, Yamamoto Y, Yamashita K, Nannya Y, Ogawa S, Takaori-Kondo A. Kataoka A, et al. Int J Hematol. 2023 Jun;117(6):919-924. doi: 10.1007/s12185-023-03532-y. Epub 2023 Jan 14. Int J Hematol. 2023. PMID: 36641501
Based on the findings of systemic inflammation, a diagnosis of VEXAS syndrome was made. The fever and skin rash improved with azacitidine therapy. ...Furthermore, azacitidine may be a good treatment option for systemic autoinflammation in MDS associated with VEXA …
Based on the findings of systemic inflammation, a diagnosis of VEXAS syndrome was made. The fever and skin rash improved with …
Azacitidine for patients with Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome (VEXAS) and myelodysplastic syndrome: data from the French VEXAS registry.
Comont T, Heiblig M, Rivière E, Terriou L, Rossignol J, Bouscary D, Rieu V, Le Guenno G, Mathian A, Aouba A, Vinit J, Dion J, Kosmider O, Terrier B, Georgin-Lavialle S, Fenaux P, Mekinian A; French VEXAS study group, Groupe Francophone des Myélodysplasies (GFM) and MedecineINterne, HEmato et ONco (MINHEMON) group. Comont T, et al. Br J Haematol. 2022 Feb;196(4):969-974. doi: 10.1111/bjh.17893. Epub 2021 Oct 14. Br J Haematol. 2022. PMID: 34651299
Based on a French nationwide registry of 116 patients with VEXAS, we report the efficacy and safety of azacitidine treatment in 11 patients with VEXAS with MDS. Clinical response of VEXAS to azacitidine was achieved in five patients (46%), during 6, 8+, 12, 21, 27+ months …
Based on a French nationwide registry of 116 patients with VEXAS, we report the efficacy and safety of azacitidine treatment in 11 patients …
Orbital and periorbital inflammation in VEXAS syndrome.
Martín-Nares E, Vargas-Serafín C, Delgado-de la Mora J, Montante-Montes de Oca D, Grayson PC, Larios E, Crispín JC. Martín-Nares E, et al. Scand J Rheumatol. 2022 Jul;51(4):338-341. doi: 10.1080/03009742.2022.2045791. Epub 2022 Mar 18. Scand J Rheumatol. 2022. PMID: 35302406 No abstract available.
Relapsing polychondritis - A single Centre study in the United Kingdom.
Sangle SR, Hughes CD, Barry L, Qureshi S, Cheah CK, Poh YJ, D'Cruz DP. Sangle SR, et al. Autoimmun Rev. 2023 Aug;22(8):103352. doi: 10.1016/j.autrev.2023.103352. Epub 2023 May 4. Autoimmun Rev. 2023. PMID: 37146927 Review.
PATIENTS AND METHODS: We retrospectively analysed patients with a clinical diagnosis of RP. Patients were investigated using pulmonary function tests, dynamic high-resolution CT scans, bronchoscopy, laryngoscopy and/or PET-CT scans along with autoimmune serology. ...
PATIENTS AND METHODS: We retrospectively analysed patients with a clinical diagnosis of RP. Patients were investigated using pulmonar …
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. The clinical manifestat …
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a novel described autoinflammatory entity for whi …
Toward a pathophysiology inspired treatment of VEXAS syndrome.
Heiblig M, Patel BA, Groarke EM, Bourbon E, Sujobert P. Heiblig M, et al. Semin Hematol. 2021 Oct;58(4):239-246. doi: 10.1053/j.seminhematol.2021.09.001. Epub 2021 Oct 5. Semin Hematol. 2021. PMID: 34802546
VEXAS syndrome has an unmet need for therapeutic interventions. Even if few data exist regarding the treatment of this newly described syndrome, different options can be proposed given the unique pathophysiological consequences of the clonal dominance of UBA1 mutate …
VEXAS syndrome has an unmet need for therapeutic interventions. Even if few data exist regarding the treatment of this newly d …
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