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

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.. Al l patients were treated initially with IVMP and IVCy followed by monthly IV Cy for at least 3 months, and then every 2 and/or 3 months according to cli nical response. Prednisone was given at 1-2 mg/kg during the first month. L aboratory studies included routine laboratory tests, antinuclear antibodies , anli-dsDNA, antiphospholipid antibodies, and complement components C3 and C4. Neurodiagnostic studies included cerebrospinal fluid, magnetic resonan ce imaging, computed tomography scanning, single photon emission computed t omography and electroencephalography. Results, Three patients had organic brain syndrome with psychosis, 3 had se izures, I 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 mon NP manifestations. Most of them were accompanied by general SLE activity. Anticardiolipin antibodies w ere positive in 3 patients and none was anticoagulated. All patients improv ed, 6 patients had a complete recovery and 1 patient recovered with minor n eurological deficit. All but one improved significantly within the first we ek of combined IVMP and IVCy, The mean time of followup was 37 months (rang e 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.