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.
The significant growth of information in the field of biological therapies (BT) for the treatment of systemic lupus erythematosus (SLE) has made it necessary to update the previous SER recommendation document. Three BTs have been approved for use in SLE. The first BT available was belimumab, with an already abundant and solid body of evidence regarding its efficacy and safety. More recently, anifrolumab was added, also backed by a robust body of evidence but with less experience of practical application. Finally, the emergence of obinutuzumab, a second-generation anti-CD20 monoclonal antibody that is more effective than rituximab and has demonstrated efficacy in lupus nephritis, has revitalised ablative therapy in SLE, although more safety data is needed before it can widely replace rituximab. Despite the lack of direct comparisons between the various biological agents available, certain differences regarding their efficacy across different disease domains, as well as in the speed of action and safety-related aspects, must be taken into account when tailoring treatment decisions. Overall, biologics have been increasingly incorporated into the treatment algorithm at earlier stages of SLE, based on their ability to improve outcomes compared to standard therapy, and are now first-line treatments for certain manifestations such as lupus nephritis.
We present the case of a 40-day-old female infant with acute febrile illness, cutaneous lesions, severe respiratory distress, and shock refractory to antibiotic therapy. Skin biopsy revealed findings consistent with acute febrile neutrophilic dermatosis (Sweet syndrome). After excluding infectious, hematological, and hemophagocytic etiologies, an autoinflammatory syndrome was suspected, and treatment with anakinra and glucocorticoids was initiated, resulting in initial clinical improvement. However, after one year of treatment and dose tapering, the patient experienced relapses. Tocilizumab was then started, leading to a sustained clinical response to date.
