VEXAS syndrome in a patient with previous spondyloarthritis with a favourable response to intravenous immunoglobulin and anti-IL17 therapy

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Magnol, Marion | Couvaras, Loukianos | Degboé, Yannick | Delabesse, Eric | Bulai-Livideanu, Cristina | Ruyssen-Witrand, Adeline | Constantin, Arnaud

Edité par CCSD ; Oxford University Press (OUP) -

International audience. recently identified a genetic disorder that connects seemingly unrelated adult-onset inflammatory syndromes as VEXAS syndrome. We report a case of VEXAS syndrome in a patient with previous SpA with a favourable response to i.v. immunoglobulin and anti-IL17 therapy.In 2010, a diagnosis of SpA was made in a 57-year-old man, who had inflammatory back pain, peripheral arthritis, bilateral active sacroiliitis on MRI (Fig. 1A) and HLA- B27–positive testing [2]. After failure of several NSAIDs, the initiation of anti-TNF therapy (adalimumab) led to a rapid clinical response.In 2012, under anti-TNF therapy, the patient developed severe inflammatory disorders, including relapsing anterior uveitis, and auricular and nasal chondritis; abdominal pain, diarrhoea and weight loss, with multifocal aphthous lesions on colonoscopy, classified as undifferentiated IBD; progressive skin involvement, with painful, tender and oedematous red plaques, affecting upper and lower limbs

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