Iqbal Zein Assyidiqie, Nia Kurniati, Dina Muktiarti
Objective:Neuropsychiatric systemic lupus erythematosus (NPSLE) is a generic definition referring to a series of neurological and psychiatric symptoms related to SLE. This study aims to describe NPSLE profile in children in Cipto Mangunkusumo Hospital (CMH). Methods:We collected data from pediatric systemic lupus erythematosus (pSLE) registry in Allergy and Immunology Division about baseline characteristics, symptoms, diagnostic approaches, treatments and outcomes of NPSLE in Department of Child Health, CMH, January 2017 - April 2019. Results:There were 13 cases of NPSLE out of 89 pSLE in CMH (14%). For those cases, there were 11 girls and 2 boys. The most common age group was 6-13 years-old (83%) with 50% neuropsychiatric symptoms were identified at first diagnosed with SLE. Most common symptom was seizure (6/13), followed by behavior disorder (5/13), psychosis (3/13), hemiparesis (2/13), loss of consciousness (2/13), depression (2/13) and sleep disorder (2/13). Mean SLEDAI score at first diagnosis was 43.5 (28-59). Most patients (91%) had low complement and high anti-dsDNA. On radiological examination, we found abnormality in head CT or MRI in 7 cases and 1 patient with abnormal EEG. All patients received oral methylprednisolone (13/13), methylprednisolone pulse only (7/13), and methylprednisolone pulse followed by cyclophosphamide pulse (5/13). There were 9 patients received additional immunosuppressants such as mycophenolate, azathioprine and cyclosporine. Some patients received symptomatic therapy, especially patients with seizure (6/13) and psychiatric disorders (6/13). There was no patient died because of NPSLE, however 3 patients died due to sepsis and the others had improved condition (10 cases). Conclusions:Neuropsychiatric systemic lupus erythematosus is one of the mayor involvement organ related to SLE. There are 14% NPSLE cases in CMH with the most common symptom is seizure. All patients receive high dose steroid combined with other immunosuppressants. None of the patients died due to NPSLE.