Clinical Features Of Neuropsychiatric Systemic Lupus Erythematosus

ObjectivesAs a severe complication of acute or terminal phase systemic lupus erythematosus(SLE), NPSLE could always be used as a clinical marker of poor prognosis. It is suggested that 14% to 75% SLE patients could have neuropsychiatric involvements, while the morbidity rate in China is generally lower, about 20%, which could be a result of diagnosis difficulty in the early stage of cognitive dysfunction. The clinical manifestations of NPSLE are diversified while lack of specificity, which varied from subclinical cognitive dysfunction, transaction myelitis to life threatening stroke. As a consequence, the diagnosis of NPSLE is extraordinarily difficult, especially when the neuropsychiatric symptoms manifested before the clinical confirmation of SLE. The purpose of this study was to further investigate the risk factors, clinical manifestations and treatment of NPSLE using the clinical data of 42 NPSLE patients.MethodsWe analyzed the clinical data of 42 cases with NPSLE (NPSLE group), and evaluated their neuropsychiatric involvements according to the ACR NPSLE nomenclature and case definitions for neuropsychiatric lupus syndrome for NPSLE. Then we compared the clinical features, laboratory data and SLEDAI score between NPSLE group and 187 patients with non-NPSLE(control group). Moreover, we divided the NPSLE group into several subgroups according to the patients' manifestation differences, and further compared the discrepancy between relating indexes.ResultsWe noticed that 50% patients in the NPSLE group had obvious inducements before their occurrence of neuropsychiatric symptoms. Twelve of the nineteen NP symptoms were identified, and 73.8% NPSLE patients presented two or more symptoms. The most common symptoms included epilepsy(27.0%), cerebrovascular disease (20.6%) and acute confusional state (14.3%). Not only the incidence of fever, elevated ESR or thrombocytopenia, but also the mean SLEDAI score and mortality rate of the patients in NPSLE group were higher than in non-NPSLE group (P<0.05). However, the incidence of discoid erythema and arthralgia in NPSLE group were lower than in non-NPSLE group (P<0.05), also the percentage of patients treated with steroid regularly or immunosuppressive agent combined was less in NPSLE group than in non-NPSLE group(P<0.05). Moreover, comparing to the NPSLE patients who only had psychiatric symptoms, those having nervous system affected usually had higher the rate of brain injury detected by MRI and the incidence of thrombocytopenia, while the curative effect was worse (P<0.05). Similarly, the NPSLE patients with seizure were much more likely to have moderate and severe abnormal EEG results, and higher SLEDAI score than those without seizure (P<0.05). The causes of death for NPSLE patients included the disease itself, secondary infection and organ failures. ConclusionsNPSLE patients usually suffered more heavy, with rapid progressions and poor prognosis, especially when the organic damage of brain was complicated. Complications prevention and regular treatments with steroid or immunosuppressive agent could help to avoid NPSLE incidence and improve the prognosis.