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Vexas grayson. Question and Answer Session. The New England Journal of Medicine. We named this disorder the VEXAS vacuoles E1 enzyme X-linked autoinflammatory.

Relapsing polychondritis RP is a rare heterogenous systemic inflammatory disease with a predilection for cartilaginous structures. Kastner and Grayson contributed equally to this article. VEXAS Syndrome Topical Steroids Increase Osteoporosis and Fracture Risk but Just a Little.

Characteristic vacuolisation of granulocytic and erythroid precursors associated with VEXAS syndrome. 1 article PMID. By using this genomic approach we have managed to find a thread that ties together patients carrying all of these seemingly unrelated disparate diagnoses Kastner says.

In this episode Peter Grayson MD MSc a study author on the recent paper published in the New England Journal of Medicine gives us further insight into the discovery of the VEXAS syndrome and the overall importance of rare rheumatic disease research at a genetic and molecular level. Hematologic mosaicism for variants within a single. Using a genotype-driven approach we identified a disorder that.

Assuming VEXAS makes it through the first round it might have to watch out for RP subtypes in the second round as thats another excellent study from the same senior author Peter Grayson and who knows if it could make it through the juggernauts coming out of the ANCA and SLE regions. How do I recognize and treat the VEXAS syndrome. The name derives from Vacuoles E1 enzyme X-linked Autoinflammatory Somatic.

Rieu JB El Kassir A Largeaud L Dion J Comont T. Clinical Manifestations of the VEXAS Syndrome. We named this disorder the VEXAS vacuoles E1 enzyme X-linked autoinflammatory somatic syndrome.