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VEXAS syndrome extends the neurological complications of haemopathies
  1. Philippe Corcia
  1. Department of Neurology, ALS Centre, CHRU Tours, Tours 37044, France
  1. Correspondence to Professor Philippe Corcia, Department of Neurology, ALS Centre, CHRU Tours, Tours 37044, France; corcia{at}

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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 …

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  • 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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