Favorable response to intravenous methylprednisolone and cyclophosphamide in children with severe neuropsychiatric lupus

J Rheumatol. 1999 Feb;26(2):432-9.


Objective: To evaluate the effect of intravenous methylprednisolone (IVMP) and cyclophosphamide (IVCy) in children with severe neuropsychiatric (NP) systemic lupus erythematosus (NPSLE).

Methods: We studied 7 consecutive pediatric patients with severe NPSLE. All patients were treated initially with IVMP and IVCy followed by monthly IVCy for at least 3 months, and then every 2 and/or 3 months according to clinical response. Prednisone was given at 1-2 mg/kg during the first month. Laboratory studies included routine laboratory tests, antinuclear antibodies, anti-dsDNA, antiphospholipid antibodies, and complement components C3 and C4. Neurodiagnostic studies included cerebrospinal fluid, magnetic resonance imaging, computed tomography scanning, single photon emission computed tomography and electroencephalography.

Results: Three patients had organic brain syndrome with psychosis, 3 had seizures, 1 stroke, 1 cerebral vasculitis, 1 optic neuritis, and 1 transverse myelitis. In 3 of these cases, nervous system involvement was the initial presentation of SLE. Five patients had 2 or more NP manifestations. Most of them were accompanied by general SLE activity. Anticardiolipin antibodies were positive in 3 patients and none was anticoagulated. All patients improved, 6 patients had a complete recovery and 1 patient recovered with minor neurological deficit. All but one improved significantly within the first week of combined IVMP and IVCy. The mean time of follow-up was 37 months (range 8-55). IVCy was well tolerated with minimal side effects.

Conclusion: Early aggressive treatment with combined IVMP and IVCy followed by monthly IVCy may be an effective therapy for severe NPSLE in children.

Publication types

  • Clinical Trial

MeSH terms

  • Adolescent
  • Age of Onset
  • Anti-Inflammatory Agents / administration & dosage
  • Anti-Inflammatory Agents / therapeutic use
  • Brain Diseases / diagnosis
  • Brain Diseases / drug therapy*
  • Child
  • Cyclophosphamide / administration & dosage
  • Cyclophosphamide / therapeutic use*
  • Drug Therapy, Combination
  • Electroencephalography
  • Female
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Immunosuppressive Agents / therapeutic use
  • Lupus Erythematosus, Systemic / diagnosis*
  • Lupus Erythematosus, Systemic / drug therapy*
  • Magnetic Resonance Imaging
  • Mental Disorders / diagnosis
  • Mental Disorders / drug therapy*
  • Methylprednisolone / administration & dosage
  • Methylprednisolone / therapeutic use*
  • Tomography, Emission-Computed
  • Treatment Outcome


  • Anti-Inflammatory Agents
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Methylprednisolone