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Inês Madureira1, Margarida Alcafache1, Maria Antónia Silva2, Rita Silva3, Margarida Paula Ramos4

1- Área de Pediatria Médica;  Hospital Dona Estefânia - Centro Hospitalar Lisboa Central, EPE
2- Serviço de Pedopsiquiatria; Hospital Dona Estefânia -Centro Hospitalar Lisboa Central, EPE
3- Serviço de Neurologia Pediátrica; Hospital Dona Estefânia - CHLC, EPE
4- Unidade Reumatologia; Hospital Dona Estefânia - Centro Hospitalar Lisboa Central, EPE

- 11th International Congress on Systemic Lupus Erythematosus in September 2015 Vienna – Austria,  in September 2015 (poster presentation).

Introduction: Up to two-thirds of patients with paediatric systemic lupus erythematosus (SLE) show involvement of the nervous system. It is a major cause of morbidity. There are a wide variety of neuropsychiatric manifestations that generally present within the first year of diagnosis. Treatment needs to be directed toward the presumptive pathophysiologic mechanism. Most patients will require symptomatic and immunosuppressive therapies, using an induction and maintenance approach. However, many cases are resistant to therapy.
Case: report - A 14-year-old boy was admitted with a 1-year history of intermittent arthralgia, constitutional symptoms, Raynaud phenomenon and dysphagia. Upon further investigation, he was diagnosed with SLE-scleroderma overlap syndrome, with class V membranous nephritis. Shortly after, he developed symptoms of anxiety and cognitive dysfunction - executive functions, memory impairment and acalculia. Electroencephalograms and brain imaging were normal. Cerebrospinal fluid analysis detected a mild IgG elevation and pleocytosis. Antiphospholipid and anti-ribosomal P antibodies were positive. He started methylprednisolone, cyclophosphamide, hydroxychloroquine, enoxaparin and gabapentin. Because of clinical deterioration, with mutism alternating with psychomotor agitation and acute confusional state, haloperidol and rituximab were added. There was rapid clinical response, with resolution of the neuropsychiatric symptoms and improvement in proteinuria. After 20 months of follow-up, no evidence of neuropsychiatric recurrence was found.
Discussion: The diagnosis of primary neuropsychiatric SLE can be challenging, Immunoserologic testing and brain imaging may contribute to an earlier and more specific diagnosis. Rituximab has shown effectiveness in treating severe refractory neuropsychiatric SLE, namely in cases with acute confusional state and cognitive dysfunction.

Keyword (Complete):  Juvenile systemic lupus erythematosus; Neuropsychiatric manifestations;  Rituximab