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

Dear Editor, Beck et al. [1] 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,...