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CASE REPORT
Association of severe and therapy-refractory systemic lupus erythematosus and neuromyelitis optica: a management challenge
  1. Inês Furtado,
  2. Guiomar Pinheiro,
  3. Ana Campar,
  4. Teresa Mendonça
  1. Internal Medicine Department, Centro Hospitalar do Porto, Porto, Portugal
  1. Correspondence to Dr Inês Furtado, inessilvafurtado{at}gmail.com

Summary

Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disorder. Neuromyelitis optica (NMO) is an infrequent neuroinflammatory disorder, whose association with SLE remains rare. The authors report the case of an 18-year-old woman, with SLE refractory to multiple immunosuppressive therapies and novel biological agents. Under immunosuppressive therapy, the patient presented with transverse myelitis with contiguous spinal cord lesions and urinary incontinence, having been diagnosed with seropositive NMO, which was also proven to be refractory to common treatments. Partial recovery of the neurological deficits occurred with plasmapheresis, although not averting the brain involvement by NMO that ensued. The patient was listed nationally for allogeneic bone marrow transplant, but, unfortunately, no match was found and the patient died of severe cerebral NMO flare with coma due to brain swelling and consequent respiratory failure. Although the association of SLE and NMO is very rare, early diagnosis is crucial to facilitate initiation of immunosuppressive therapy.

  • systemic lupus erythematosus
  • neurology
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Footnotes

  • Contributors IF was responsible for the conception and design of the work as well as data collection, analysis and interpretation. IF and GP drafted the article which was reviewed by AC and then critically revised. IF, GP, AC and TM were responsible for the final approval of the version to be published.

  • Funding The authors have 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.

  • Patient consent Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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