Clinical Features In 21 Children With Lupus Nephritis Combined With Neuropsychiatric Systemic Lupus Erythematosus

Objective To analyze the clinical characteristics, prognosis and the risk factors in 21 children with lupus nephritis(LN) combined with neuropsychiatric systemic lupus erythematosus (NPSLE).Methods The clinical data from 21 pediatric lupus nephritis with neuropsychiatric systemic lupus erythematosus (NPSLE) from January 2012 to October 2015 were retrospectively analyzed.Results Twenty-one cases showed neuropsychiatric systemic lupus erythematosus among 117 children with lupus nephritis.and the incidence rate Was 18.0%. The age ranged from 6.9 to 14.0 years. The male to female ratio was 1.0:4.3.In our 21 LN children, the NPSLE occurred within half a year is 71.4%, of which 8 cases with mental abnormality (61.9%),10 cases with headache (47.6%). Among 21 children,13 cases were the nephrotic syndrome type (61.9%),4 cases were the hematuria proteinuria type,2 cases were the proteinuria type, hematuria was found in 1 case and 1 case was found in accelerated nephritis model. Totally 5 cases underwent renal biopsy, and among them,3 cases(60.0%)were diagnosed as diffuse proliferative lupus nephritis(classâ…£). Followed by membranous with lupus nephritis (classâ…¤) and mesangial proliferative with lupus nephritis (classIV) in 1 case. The cerebrospinal fluid abnormality rate 25.0%(P=0.604),the EEG abnormality 90.9%(P=0.127), and the brain MRI abnormality 94.1%(P=0.000). Corticosteroid and/or cytotoxic drugs were used in all of 21 cases. After one year of follow-up, fifteen cases Lupus activity and kidney damage were complete remission, three partial remission, and 1 cases had chronic renal damage (uremia period),2 cases died. The time of starting hormone therapy(OR=48.053, P=0.021), the non-standard hormone therapy(OR=32.270, P=0.035), and the non-standard CTX treatment (OR= 22.967, P=0.043)were the important factors to occur NPSLE.Conclusions (a)Neuropsychiatric systemic lupus erythematosus can occur at any time of the disease course, but tend to occur at the half a year of the onset. (b) The neuropsychiatric symptoms are diverse and complex, But with mental abnormality and headache for its main clinical manifestations; Brain MRI can assist in making the diagnosis. (c)The time of starting hormone therapy, hormone therapy is not standard and CTX treatment is not standardare are the important factors to occur NPSLE. (d)The outcomes of lupus nephritis with neuropsychiatric systemic lupus erythematosus can be improved by adequate and appropriate treatment.