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

Abstract

To evaluate the effect of intravenous methylprednisolone (IVMP) and cyclophosphamide (IVCy) in children with severe neuropsychiatric (NP) systemic lupus erythematosus (NPSLE). 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. 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. Early aggressive treatment with combined IVMP and IVCy followed by monthly IVCy may be an effective therapy for severe NPSLE in children.