The Clinical Features Of Juvenile Neuropsychiatric Systemic Lupus Erythematosus

Neuropsychiatric systemic lupus erythematosus is one of the most serious Complications in the systemic lupus erythematosus, and the incidence is merely less than lupus nephritis。According to the diagnosis standard of ACR, the incidence is substantial 37% to 95%. The pathogenesis of NPSLE is quite complex that at present what has been widely accepted is that it has some relations with sediment of immune complex and blood vessles inflammation. NPSLE has various kinds of clinical features which are quite complicated, and these features are usually divided into two subtypes, within and without nerve system. Clinical features within nerve system includes high fever, damage of skin mucosa, gall of articulation, and damage of kidney. The other clinical features could be divided into thoese in nerve system and peripheral nerve system, mainly including headache, epileptic seizure, coma, etc. Treatment of NPSLE mostly focuses on individuality treatment on the basis of SLE. Presently, the treatment is chiefly chosen immunosuppressant and glucocorticoid. Prognosis of NPSLE usually has mortality rate at about 10%. Analysis reports on children's SLE are more than that of the NPSLE's, nationwide.Objective: To invegestigate the clinical features, Laboratory Examinations, Imageology examination, and Electrophysiology examination for NPSEL.Methods: To analysis the clinical datas retrospectirly ,which come from all patients who go inpatient and Outpatient from 2000.1 to 2009.12, and to record general situation (Gender, age, duration,ect),clinical manifestations (Ferver, damage of skin mucosa, gall of articulation, and damage of kidney, ect), Laboratory examinations(Blood routine, antinuclear antibodies, anti-dsDNA, anti-Sm, Anti-SSA), Imageology examtion and Electrophysiology examination.Results: In 25 NPSLE, male to female ratio of 1/4, 17 (68%) cases'duration are less than 1 year. Some patients have nervous system symptoms. 13 patients have headache in 52%, 4 patients have memory loss in 16%, seizures 3 patients appear seizure in 12%,and 2 patients perform emotional disorders in 8%. And some patients have neurological signs.6 patients perform positive of Babinski in 24%, tendon reflexia's is 9 patients appear positive of tendon reflexia in 36%, 5 patients have papilledema in 20%, 1 patients appear coma in 4%, 2 patients have hemiplegia in 8%, 1 patients appear aphasia in 4%. 4 patients have respiratory manifestations, 4 patients have some manife- stations of digestive system, 22 patients appear renal damage, and 4 patients have damage of circulatory system. Some patients have damage of blood system , 5 patients are Pale, 3 patients appear damage of mucosa which come from oral,nasal and gums bleeding. 2 patients appear swelling of superficial lymph nodes,3 patients are splenomegaly, All patients join in laboratory examinations. 24 patients have positive ANA in 96%, 16 patients have positive anti dsDNA in 64%, 15 patients have positive anti-Sm in 60%, 8 patients have positive anti-SSA in 32%,18 patients have a low of C3 in 72%, 12 patients have a low of C4 in 48% , 9 patients have a high of IgG in 36%.There 5 cases appearing elevation of CSF pressure in 11 patients(45%) . 25 patients have blood routine.8 patients appear leukocytopenia in 32%, 5 cases appear thrombocytopenia in 20%, 20 patients appear aglobulia in 80%,and 16 patients perfrom anemia in 64%. There are 25 patients having Urinalysis.20 patients appear proteinuria (80%). 11 patients have a high of ALT in 44%, 10 patients have a high of AST in 40%. 22 patients have a high of ESR in 88%. 6 patients appear positive RF in 24%。22 patients have a examination of CT, and 16 patients perfrom abnormal result in 72% ;6 patients have examination of MRI which,and 4 patients appear abnormal which include 1 patient with normal CT.There are 21 having a abnormal result of EEG in all pations (84%).All of the patients were given regular treatment, and all the patients have a follow up from 5 to 7 year, Average 5.1±1.2 years,23 cases have a good state of an illness, who have no symptom of nervous system.2 cases are died ,the cause of death come from NPSLE,The survival rate for 5 years is 92%.Conclusion: Most NPSLE patients have a course less than one year. Patients with high fever are the typical clinical features out of the nerve system. And headache has been the most obvious features within the nerve system. In Laboratory Examinations, the rate of ANA positive are at a high rate. And in the Imageology and the Electrophysiology,MRI(CT) and EEG have a high positives rate. But MRI's sensibility is better than CT's.