A novel therapeutic approach using the Zipper method to treat chorea in a pediatric-onset systemic lupus erythematosus patient

Lupus. 2021 Mar;30(3):502-509. doi: 10.1177/0961203320984013. Epub 2021 Jan 7.

Abstract

Pediatric-onset systemic lupus erythematosus is among the prototypic systemic autoimmune diseases seen in children. Although the neuropsychiatric involvement rate varies during the course of the disease, it is an important cause of morbidity and mortality. The clinical picture of neuropsychiatric SLE (NPSLE) is highly variable, and neurological features can precede systemic findings, leading to some diagnostic difficulties. NPSLE requires early and aggressive immunosuppressive therapy. Some patients can be resistant to immunosuppressive therapy. Chorea is a rare manifestation that occurs in 1.2%-2% of SLE patients and can result from an immunologically mediated mechanism, antiphospholipid autoantibodies or ischemia. Herein we present the first case of pediatric-onset SLE diagnosed with central nervous system involvement and treated with Zipper method. The Zipper method is a new immunomodulation treatment. The clinical findings of the patient, which were resistant to corticosteroids and cyclophosphamide, resolved by this novel treatment.

Keywords: Chorea; Zipper method; neuropsychiatric lupus; systemic lupus erythematosus.

Publication types

  • Case Reports

MeSH terms

  • Carotid Artery, Internal / diagnostic imaging
  • Carotid Artery, Internal / pathology
  • Caudate Nucleus / diagnostic imaging
  • Caudate Nucleus / pathology
  • Child
  • Chorea / drug therapy*
  • Chorea / etiology
  • Humans
  • Immunoglobulins, Intravenous / administration & dosage*
  • Immunomodulation
  • Lupus Vasculitis, Central Nervous System / diagnosis
  • Lupus Vasculitis, Central Nervous System / drug therapy*
  • Lupus Vasculitis, Central Nervous System / pathology
  • Male
  • Plasma Exchange / methods*

Substances

  • Immunoglobulins, Intravenous