Clinical Features, Management, and Outcomes of Neuropsychiatric Lupus in Saudi Children, a Multicentric Study

Abstract Introduction: Systemic lupus erythematosus (SLE) is a complex autoimmune disorder that affects several systems, including the neurological system. At some time over the course of their illness, around one-third of all children with SLE will exhibit neuropsychiatric symptoms. A better prognosis depends on early diagnosis and timely action. Objectives: This study aims to outline the most prevalent clinical characteristics, radiographic and laboratory results, treatment options, and outcomes of pediatric neuropsychiatric lupus in Saudi Arabia. Materials and Methods: All SLE children’s data and evidence of neuropsychiatric symptoms have been extracted from the databases of three Saudi Arabian tertiary institutions. Neuropsychiatric SLE (NPSLE) is characterized by the presence of SLE in addition to at least one of the following: mobility problem, seizure, cognitive impairment, psychiatric or spinal cord symptoms, or cerebrovascular accident (CVA). Patients who had concurrent illnesses or isolated headaches were not included. Results: Twenty-five individuals (23 female) with a mean age of 10.3 years were included in our study. Most patients presented with neuropsychiatric manifestations at the onset of SLE or developed them within the first six months of diagnosis. Their clinical features were headache in 60%, seizure disorders in 60%, CVA in 32%, movement disorders in 8%, acute confusional state in 44%, cognitive dysfunction in 20%, mood disorder in 40%, and Guillain-Barré syndrome (GBS) in 4%. In 88% of cases, the dsDNA was high, and the antinuclear antibody titer was positive. A total of 25 patients, only 15 underwent MRI/MRA, and among them, 12 had positive findings. The management consisted of a range of regimens. However, the majority of patients received mycophenolate as maintenance along with cyclophosphamide or rituximab and methylprednisolone pulse treatment. Three patients died during the 24-month follow-up, but the majority of the 18/25 patients’ neuropsychiatric symptoms had subsided. Conclusion: The study demonstrates the variation of NPSLE management in children. To precisely assess the disease’s prevalence in our region, a more comprehensive investigation encompassing all of Saudi Arabia is required. To improve the outcome of the condition, a national consensus guideline about therapy is recommended.