| Research backgroundSystemic lupus erythematosus (SLE) is a involving multiple organ systems and a variety of autoantibodies appear chronic systemic autoimmune disease, its clinical symptoms varied, involving the skin and mucous membrane, skeletal muscle, kidney, heart, lung, nervous system, digestive system such as multiple organ systems, with the current early diagnosis means increased and the treatment of SLE and increased the level of SLE prognosis has improved significantly, but the SLE multiple organ serious injury and infection, especially with severe neuropsychiatric systemic lupus erythematosus acute stage, is still one of the main causes of death in patients with. SLE invasion of the central nervous system may display for the symptoms of a variety of nervous and mental disease, patients with this type of system is called neuropsychiatric systemic lupus erythematosus (neuropsychiatric systemic lupus erythematosus, NPSLE, SLE) is a serious condition, also is one of the leading causes of death in patients with SLE.At present, due to systemic lupus erythematosus encephalopathy etiology is unknown, the condition is complex, the prognosis is poor, often need to Rheumatology and neural Department of internal medicine physician collaborative diagnosis and treatment, but sometimes showed only slight early neuropsychiatric symptoms or a sub clinical manifestations, the clinical problems and atypical cases, still do not eliminate misdiagnosis may, therefore, timely and correct diagnosis and treatment, the remission of disease, systemic lupus erythematosus encephalopathy patients to reduce the complication, improve the prognosis and reduce mortality is very important.ObjectiveObjective to study the clinical characteristics of patients with systemic lupus erythematosus encephalopathy, discuss the related clinical risk of systemic lupus erythematosus encephalopathy occurred factors, provides the objective basis for the treatment of systemic lupus erythematosus with damage in nervous system, in order to achieve the treatment and prevention of clinical encephalopathy in systemic lupus erythematosus provide new ideas.Objects and methodsSelection of Rheumatology in 2002 January to 2014 January Southern Medical University south hospital treatment of patients with systemic lupus erythematosus, diagnostic criteria of SLE diagnostic criteria for the 1997 American College of Rheumatology setting, follow-up to 2014 October. The clinical data of 138 patients with SLE is relatively intact were analyzed retrospectively, including 8 cases of male patients,130 cases of female patients, aged between 11 to 71 years of age, the occurrence of the damage of the central nervous system (50 cases of encephalopathy group), without the occurrence of nervous system damage in 88 cases (non encephalopathy group). Excluding those with hypertensive encephalopathy, diabetes, viral meningitis, encephalitis, senile dementia, brain trauma history, congenital epilepsy, brain tumor, acute cerebral hemorrhage and effect of lupus encephalopathy diagnosed patients.Methods:We conducted a case-control study. Read the patient medical records, the collection of the clinical and laboratory data, including clinical data include:name, sex, age, course of disease, there is no history of infections, pregnancy history (including abortion, birth history, family history, the history of menopause) (family in patients with connective tissue disease), the use of drugs in the treatment of SLE (non contraceptive history, penicillin, procainamide, methyldopa, isoniazid), diagnosis and treatment of the year, whether newly diagnosed, times of hospitalization, disease diagnosis, treatment, prognosis and follow-up. Routine laboratory data, including blood, urine, stool routine,24 hours urinary protein quantitative determination of biochemical:such as electrolytes, blood fat, liver function (Alt, aspertate aminotransferase, albumin, globulin, white globulin ratio) and renal function (serum urea nitrogen, serum creatinine), erythrocyte sedimentation rate (ESR), c-reactive protein (CRP), we, blood coagulation function;Immunological indexes include:the humoral immune six [immunoglobulin G (IgG), immunoglobulin A (IgA), immunoglobulin M (1gM), total serum complement activity (CH50), C3, complement C4], autoantibody qualitative [antinuclear antibody (ANA), double-stranded DNA antibodies (ds-DNA), anti SSA (SSA), SSB antibodies (SSB), Sm (Sm), UlRnp antibody (U1Rnp) resistance, resistance to ribosomal P protein antibodies (ARPA/Rib-P), PCNA antibody (PCNA), antimitochondrial antibody (AMA M2), the centromere antibody (CENP B) antibody, Scl-70 (Scl-70), resistance to PM-Scl antibodies (PM-Scl), nucleosome antibody (AnuA) resistance, resistance to histones antibodies (AHA), anti J0-1 (JO-1), restructuring RO52 (RO52) resistance, anti cardiolipin Body (ACA) and anti neutrophil cytoplasmic antibody (ANCA)] test. Analysis of clinical and laboratory index of all patients with measurement data statistics, independent sample mean comparison using the single factor analysis of variance and t test, the number of count data were compared with X2 test. The statistical significance (P<0.05) clinical and laboratory index of multi factor regression (Logistic regression) analysis, the level of test, a< 0.05 (bilateral), with P < 0.05 with statistical significance, the data processing with SPSS 13 statistical software for computing.Result1 To describe the clinical characteristics of systemic lupus erythematosus encephalopathy1.1 The general situation of encephalopathy in systemic lupus erythematosus:of the 50 patients,45 cases of female, male 5 cases, the ratio of 9:1; age 12-64 years old, the average age of (33.1+15.2); SLEDAI score between (10-32), (18.9±4.46) sub divided between, average.1.2 The clinical manifestation of systemic lupus erythematosus encephalopathy 50 cases of lupus encephalopathy patients, with psychiatric symptoms (dementia and disturbance of consciousness, drowsiness, coma, cognitive impairment, psychiatric syndrome, hallucinations, etc.) as the main clinical symptoms were 26 cases (52%), the psychiatric symptom mostly organic psychosis performance, only 2 cases of schizophrenia like symptoms; to nerve (epilepsy, cerebral vascular accident, headache, cranial neuropathy, transverse myelitis and meningitis like) for clinical symptoms were 21 cases, accounting for 42%; of which 8 cases of epilepsy, accounting for 16%. In order to have a headache, vomiting and other intracranial hypertension clinical manifestation in 5 cases, accounting for 10%, left facial paralysis, blurred vision and other cranial nerve deficits in 3 cases, accounting for 6%, hemiplegia (respectively, right after the left limb limb) in 2 cases, accounting for 4%, distal limb weakness and other peripheral nerve nerve injury in 3 cases, accounting for 6%; another 3 cases of mixed type, accounting for 6%.1.3 Systemic lupus erythematosus encephalopathy of laboratory examination: common abnormal biochemical indexes:blood leukocyte decreased (30%), red blood cells decreased (38%), and hemoglobin decreased (44%), platelet decline (20%), urea nitrogen increased (32%), creatinine increased (14%), elevated Alt levels (34%), higher aspertate aminotransferase (44%), low serum albumin leels (40%), white globulin inverse ratio (68%), high blood triglycerides (48%), low density lipoprotein increased (24%), urinary RBC increase (58%),24 hours urinary protein quantitative increase (62%) and urine (54%), interleukin increased C3 decreased (86%), complement C4 fall (62%), elevated erythrocyte sedimentation rate (70%), elevated c-reactive protein (30%) and so on, the common abnormal immunological indexes are: ANA antibody positive (98%), anti dsDNA antibody positive (76%), anti SSA antibody positive (76%), anti SSB antibody positive (14%), resistance to ribosomal P protein antibody positive (56%), resistance to JO-1 antibody positive (12%), resistance to Sm antibody positive (30%), anti U1-RNP antibody positive (56%), anti proliferation cell antibody positive (4%), the mitochondria Antibody positive (10%), anticentromere antibody positive (4%), anti nucleosome antibody positive (52%), anti histone antibody positive (44%); RO52 positive (54%), anticardiolipin antibody (14%) ANCA positive (4%).1.4 Examination of systemic lupus encephalopathy special and imaging:1.4.1 cerebrospinal fluid examination:50 cases of lupus encephalopathy patients, 24 patients underwent lumbar puncture and CSF examination, the pressure of CSF>180mmH2Og in 10 cases accounted for 41.6%, CSF protein quantitative>450 mg/L in 8 cases accounted for 33.3%; CSF sugar quantitative<450 mg/L in 3 cases accounted for 12.5%, CSF sugar quantitative>800 mg/L in 3 cases 12.5%. CSF glucose/glucose<60%in 7 cases accounted for 29.1%; CSF chloride<119 mmol/L in 3 cases of CSF accounted for 12.5%; white blood cell count>/L in 3 cases accounted for 12.5%, CSF accounted for 12.5% of ADA> in 3 cases;1.4.2 EEG:50 cases of lupus encephalopathy patients, there were 21 cases of normal EEG,12 cases accounted for 57.1%, mild abnormalities (mainly for the wave amplitude of 9～10Hza,30～70Uv, disperses in or short to medium range theta rhythm activity) in 5 cases accounted for 23.8%, moderate abnormality (mainly for the the fundamental frequency of partial slow, theta delta wave activity to spread as the background, a small amount of alpha waves) in 2 cases accounted for 9.52%, height anomaly (diffuse non rhythmic 1～3Hz high to very high amplitude irregular delta wave release) in 2 cases accounted for 9.52%. In 3 cases treated after EEG, results:2 cases of primary EEG abnormalities in patients with mild symptoms improved or disappeared, EEG showed theta activity decreased, with the alpha wave activity primarily, return to normal. The symptoms of 1 cases patients with abnormal EEG moderately goodTurn, the EEGs showed paroxysmal, persistent diffuse activities and spike, sharp and slow wave complex number decreased or disappeared, alpha wave number, suggesting that compared with the treatment of EEG were improved.1.4.3 transcranial Doppler (TCD):50 cases of lupus encephalopathy patients, there were 5 cases of transcranial Doppler,1 normal cases accounted for 20%, abnormal (mainly PI, RJ display, contraction/Shug Chang Be Ge (S/D) decreased,: cerebral artery mean peak flow velocity (Vm) increased significantly, diastolic velocity (VD), rough flow audio abnormal rate was increased in 4 cases 80%).1.4.4 head CT and MRI examination:50 cases of systemic lupus erythematosus encephalopathy patients,21 cases were examined with CT,11 cases of normal,2 cases of multiple lacunar cerebral infarction,3 cases of lupus encephalitis,1 cases of cerebral leukoaraiosis, cerebral atrophy in 1 cases,1 cases of cerebellar hemisphere multiple patchy low density, left pons nodular enhancement in 1 cases,1 cases of maxillary sinus and ethmoid sinus inflammation.30 cases of skull MRI examination, including 6 normal cases, demyelination encephalitis like changes in 9 cases,4 cases, 4 cases of cerebral atrophy,3 cases of cerebral infarction,4 cases of cerebral white matter degeneration.1.5 Systemic lupus erythematosus encephalopathy treatment and prognosis of systems:the group of 50 cases of systemic lupus erythematosus encephalopathy were diagnosed after patients received corticosteroid therapy,5 cases with prednisone (or the equivalent amount of prednisone) 30～120mg of/d,4 cases of small dose methylprednisolone impact 250mg/d,41 cases of high-dose 500mg/d,21 patients with 1.0g/m2 and cyclophosphamide, gamma globulin combined with hormone therapy for 10 cases, gamma globulin in 10 g/D,3 consecutive D shock treatment, 15 cases were combined with 2 and above immunosuppression, symptom relief was achieved in 41 cases,4 cases invalid,5 cases of death.2 Case (lupus encephalopathy group) compared with control group (group of lupus encephalopathy) data.2.1 in case group and control group:Patients with clinical data, photosensitive rash, acral erythema necrosis was statistically significant (P<0.05).2.2 in case group and control group laboratory index:white blood cells, red blood cells, C3, C4, ALT, AST, reduce the duration, blood urea nitrogen, creatinine, urine red blood cells,24 hours urinary protein increased, anti ribosomal P protein antibody was statistically significant (P<0.05)3 ResultsThe course, photosensitive rash, acral erythema, necrosis of white blood cells, red blood cells, C3, C4, AST, ALT decrease, urea nitrogen, creatinine, urine red blood cell,24 hour urine protein level, anti ribosomal P protein antibody positive were associated with the occurrence of systemic lupus erythematosus encephalopathy (P< 0.05);Multivariate Logistic regression analysis showed:anti ribosomal P protein antibody positive (OR,24.01; 95%CI,4.91-130.16, P=0.001, AST (OR) increased 7.21; 95%CI,2.34-22.03, P=0.02, C3 (OR) decreased 5.21 95%CI,3.01-19.82; P=0.024), red blood cells in urine increased (OR,3.26; 95%CI,1.23-8.85; P=0.008) is more important to the prediction of systemic lupus erythematosus encephalopathy.ConclusionThe course, photosensitive rash, acral erythema, necrosis of white blood cells, red blood cells, C3, C4, AST, ALT decrease, urea nitrogen, creatinine, urine red blood cell,24 hour urine protein level, anti ribosomal P protein antibody and systemic lupus erythematosus encephalopathy, including anti ribosomal P protein antibody positive (OR:24.01; 95%CI,4.91-130.16, P=0.001) AST increased (OR:7.21; 95%CI, 2.34-22.03, P=0.02) C3 decreased (OR:5.2195%CI,3.01-19.82; P=0.024) and red blood cells in urine increased (OR:3.26; 1.23-8.85; 95%CI, P=0.008) is more important. |
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