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. 2024 Oct 28;10(1):260-264.
doi: 10.1016/j.ekir.2024.10.026. eCollection 2025 Jan.

A Clinicopathological Description of Kidney Features in VEXAS Syndrome

Martin Mathurin  1 , Pierre Hirsch  2   3 , Vincent Jachiet  4 , Jérôme Hadjadj  4 , Guillaume Le Guenno  5 , Mael Heiblig  6 , Solenne Pelletier  7 , Alexis Mathian  8 , Cyril Garrouste  9 , Aurélie Lavergne  10 , Samuel Ardois  11 , Joanne Flejeo  12 , Ingrid Masson  13 , Zakaria Boukerroucha  14 , François Perrin  15 , Marion Magnol  16 , Arnaud Constantin  16 , Jérémie Dion  16 , Thibault Comont  16 , Antoine Huart  17 , Jean-Louis Kemeny  18 , Cécile Picard  19 , Magali Colombat  20 , Cécile Le Naoures  21 , Laurent Benard  22 , Aurélie Sannier  23 , François Vrtovsnik  24 , Isabelle Brocheriou  25 , Cedric Pastoret  26 , Pierre Sujobert  27 , Eric Delabesse  28 , Olivier Kosmider  29 , François Delhommeau  2   3 , Arsène Mekinian  4 , Sophie Georgin-Lavialle  1 , David Buob  30 , Khalil El Karoui  31 ; FRENVEX Group
Affiliations

A Clinicopathological Description of Kidney Features in VEXAS Syndrome

Martin Mathurin et al. Kidney Int Rep. .
No abstract available

Keywords: UBA1; VEXAS; acute kidney injury; chronic kidney disease; inflammation; kidney biopsy.

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Figures

Figure 1
Figure 1
Renal biopsy: severe interstitial inflammatory infiltration with presence of lymphocytes and plasma cells, admixed with macrophages and neutrophil cells. (HES, ×20).
See this image and copyright information in PMC

References

    1. Beck D.B., Ferrada M.A., Sikora K.A., et al. Somatic mutations in UBA1 and severe adult-onset autoinflammatory disease. N Engl J Med. 2020;383:2628–2638. doi: 10.1056/NEJMoa2026834. - DOI - PMC - PubMed
    1. Beck D.B., Bodian D.L., Shah V., et al. Estimated prevalence and clinical manifestations of UBA1 variants associated with VEXAS syndrome in a clinical population. JAMA. 2023;329:318–324. doi: 10.1001/jama.2022.24836. - DOI - PMC - PubMed
    1. Georgin-Lavialle S., Terrier B., Guedon A.F., et al. Further characterization of clinical and laboratory features in VEXAS syndrome: large-scale analysis of a multicentre case series of 116 French patients. Br J Dermatol. 2022;186:564–574. doi: 10.1111/bjd.20805. - DOI - PubMed
    1. Zakine E., Schell B., Battistella M., et al. UBA1 variations in neutrophilic dermatosis skin lesions of patients with VEXAS syndrome. JAMA Dermatol. 2021;157:1349–1354. doi: 10.1001/jamadermatol.2021.3344. - DOI - PMC - PubMed
    1. Zakine È., Papageorgiou L., Bourguiba R., et al. Clinical and pathological features of cutaneous manifestations in VEXAS syndrome: a multicenter retrospective study of 59 cases. J Am Acad Dermatol. 2023;88:917–920. doi: 10.1016/j.jaad.2022.10.052. - DOI - PubMed

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