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Clinical manifestations,laboratory test,treatment and outcome of neuropsychiatric involvement in pediatric systemic lupus erythematosus patients

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Author:
No author available
Journal Title:
CHINESE JOURNAL OF PEDIATRICS
Issue:
4
DOI:
10.3760/cma.j.issn.0578-1310.2009.04.012
Key Word:
狼疮血管炎,中枢神经系统;儿童;治疗,临床研究性;Lupus vasculitis,central nervous system;Child;Therapies,investigational

Abstract: Objective To investigate the clinical manifestations,laboratory test,treatment and outcome of neuropsychiatric (NP) involvement in pediatric systemic lupus erythematosus (SLE) patients.Methods Seventy-seven patients with NP syndromes of SLE (NPSLE) seen from 1987 to 2007 were retrospectively reviewed.The relationship between the relative factors and the relapse of NPSLE was analyzed with logistic regression model.Results NPSLE was found in 17.3% of the SLE patients and 75% of the NPSLE patients the NP involvements occurred in the first 2 years of the onset of SLE.The most frequent NP manifestations were headache (31.8%) and seizure disorder (29.1%).in the active phases,the levels of Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores of the 92.2% patients were higher than 15 and belonged to severe hipus.The patients were accompanied frequently with fever (88.3%) and rash (84.4%).The most frequently involved organs were kidney (76.6%) and blood system (67.5%).In the active phases,the ANA was positive (98.7%),the level of ESR increased (86.3%),the level of complement profile decreased (72.7%).The cerebrospinal fluid (CSF) study,the CT,the MRI and the EEG were abnormal (90.1%,60.7%,54.8%,73.9%,respectively).All the patients received glucocorticoids and immunodepressant treatment in which 79.2% received Ⅳ high-dose methylprednisolone (MP),51.9% received intrathecal(IT) methotrexate (MTX) and dexamethasone (DXM),26.0% received IVIG,2 patients received autologous peripheral blood stem cell transplantation.The mortality was 9.0%.The rate of relapse was 22.0% and in 75.0% of relapsed patients the relapse occurred within 24 months from the onset of NPSLE.The SLEDAI scores related to the relapse of the NPSLE (X2=3.987,P=0.0459,OR=1.172,95% CI 1.003 and 1.370).Conclusion SLEDAI scores were significantly helpful in predicting recurrence of NPSLE.

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