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VEXAS is a complex acronym for a probably frequent disease neurologist should suggest in various neurological situations.
Bert-Marcaz et al report the first case of a CIDP (Chronic Inflammatory Demyelinating Polyneuropathy) revealing in fact a vacuole, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome. This observation involved a 74-year-old man who had developed a classical CIDP. His unsatisfactory response to Ig intravenous pulses and a high dose of steroids led clinicians to investigate further in order to determine the best second-line treatment strategy. Complementary paraclinical investigations resulted in diagnosing VEXAS, leading to targeted therapy.1
VEXAS defines a treatment refractory inflammatory syndrome, which is typically characterised by the combination of rheumatologic and haematologic features.2 The prototypical description of VEXAS syndrome …
Contributors The author solely wrote the manuscript.
Funding The author has not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Commissioned; internally peer reviewed.
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