Reumatismo (Dec 2017)

Demyelinating syndrome in SLE: review of different disease subtypes and report of a case series

  • E. Chessa,
  • M. Piga,
  • A. Floris,
  • A. Mathieu,
  • A. Cauli

DOI
https://doi.org/10.4081/reumatismo.2017.1007
Journal volume & issue
Vol. 69, no. 4
pp. 175 – 183

Abstract

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Demyelinating syndrome (DS) is a rare manifestation of systemic lupus erythematosus (SLE) (1%) with high clinical heterogeneity and potentially severe prognosis. It can represent a diagnostic and therapeutic challenge for clinicians. A recent study described 5 different patterns of demyelinating disease presentation, characterised by specific clinical, laboratory and brain and spine magnetic resonance imaging abnormalities: 1) neuromyelitis optica; 2) neuromyelitis optica spectrum disorders; 3) DS prevalently involving the brain; 4) DS prevalently involving the brainstem; 5) clinically isolated syndrome. In this review we briefly discuss typical characteristics of each DS presentation in SLE and we describe 5 illustrative clinical cases, one for each subset of DS, considering both diagnostic and therapeutic options.

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