Rodolfo Enrique Rangel Ayón, Enrique Cuevas Orta, Jorge Luis García Ramírez, Jorge Guillermo Reyes Vaca, Antonio Bravo Oro
Females predominated (77.8%), with a mean age of onset of 10.8 years. Initial manifestations were heterogeneous: cerebrovascular events in 66.7%, chorea in 22.2%, psychosis and seizures in 11.1% each, and cerebellar syndrome in one case. In most cases, neuroimaging showed findings of ischemia, vasculitis, or arterial irregularities, while the electroencephalogram was abnormal in two-thirds of the available studies. All patients had positive antinuclear antibodies, and 44.4% presented with antiphospholipid antibodies, a finding associated with a higher risk of recurrence. Treatment included high-dose steroids in all cases, with immunosuppressants such as methotrexate (55.6%) and mycophenolate (44.4%) being the most frequently used. Cyclophosphamide was used in only one case. Anticoagulant therapy was indicated only in patients with antiphospholipid syndrome or thrombotic events.
BACKGROUND AND OBJECTIVE: Paediatric-onset systemic lupus erythematosus presents a diagnostic and therapeutic challenge, particularly when the initial presentation is neurological or neuropsychiatric. The objective of this study is to describe the clinical characteristics, neuroimaging findings, therapeutic approach, and outcomes of a cohort of paediatric patients presenting with neurolupus as the first manifestation of the disease.
PATIENTS: Nine paediatric patients, aged 4-17 years, were treated at a tertiary care hospital in Mexico.
RESULTS: Females predominated (77.8%), with a mean age of onset of 10.8 years. Initial manifestations were heterogeneous: cerebrovascular events in 66.7%, chorea in 22.2%, psychosis and seizures in 11.1% each, and cerebellar syndrome in one case. In most cases, neuroimaging showed findings of ischemia, vasculitis, or arterial irregularities, while the electroencephalogram was abnormal in two-thirds of the available studies. All patients had positive antinuclear antibodies, and 44.4% presented with antiphospholipid antibodies, a finding associated with a higher risk of recurrence. Treatment included high-dose steroids in all cases, with immunosuppressants such as methotrexate (55.6%) and mycophenolate (44.4%) being the most frequently used. Cyclophosphamide was used in only one case. Anticoagulant therapy was indicated only in patients with antiphospholipid syndrome or thrombotic events.
DISCUSSION AND CONCLUSIONS: The prognosis was variable. Patients with psychosis and seizures showed a favorable recovery, while those with extensive cerebrovascular events presented with permanent motor and cognitive sequelae. This series highlights the high frequency of vascular involvement and antiphospholipid antibodies as key factors in paediatric neurolupus, providing novel evidence in the Mexican population and underscoring the need for early screening protocols and a multidisciplinary approach.