Neuropsychiatric Systemic Lupus Erythematosus with Cerebral Vasculitis and Lupus Nephritis Successfully Treated with High-dose Glucocorticoids and Mycophenolate Mofetil

Intern Med. 2022 Oct 15;61(20):3131-3135. doi: 10.2169/internalmedicine.9123-21. Epub 2022 Mar 12.

Abstract

Neuropsychiatric systemic lupus erythematosus (NPSLE) with cerebral vasculitis is rare, and its prognosis is unfavorable. High-dose glucocorticoids and cyclophosphamide are widely used for the treatment of NPSLE, but cyclophosphamide has a risk of cervical intraepithelial neoplasia and ovarian insufficiency, which may discourage its use in young women. We experienced a case of NPSLE with cerebral vasculitis and lupus nephritis that responded successfully to glucocorticoids and mycophenolate mofetil (MMF). MMF might be a treatment option for NPSLE without concern for reproductive toxicity. However, there are only a few reports on the efficacy of MMF in NPSLE, and further investigations are needed.

Keywords: cerebral vasculitis; mycophenolate mofetil; neuropsychiatric systemic lupus erythematosus.

Publication types

  • Case Reports

MeSH terms

  • Cyclophosphamide / therapeutic use
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lupus Erythematosus, Systemic* / complications
  • Lupus Erythematosus, Systemic* / drug therapy
  • Lupus Nephritis* / complications
  • Lupus Nephritis* / drug therapy
  • Lupus Vasculitis, Central Nervous System* / complications
  • Lupus Vasculitis, Central Nervous System* / drug therapy
  • Mycophenolic Acid / therapeutic use

Substances

  • Glucocorticoids
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Mycophenolic Acid