Comparison Of Two Ssle Scoring Systems And Analysis Of Correlative Factors And Prognosis Of Severe Lupus Erythematosus

Objective:Through retrospectively analyzing the clinical data of patients with systemic lupus erythematosus(SLE),we evaluated two scoring standards in the diagnosis of Severe Systemic Lupus Erythematosus(SSLE),and analyzed the relative factors,treatment and prognosis of SSLE.The aim of the study is to recognize SSLE in the early stage.And to provide foundation for preventing mild or moderate SLE turning into severe SLE,and improving the prognosis of patients.Methods:A retrospective study from December 2006 to December 2016 in 198 SLE patients from Dalian Central Hospital Affiliated to Dalian Medical University.This study consists of two parts.Part One:We diagnosed SSLE according to SLEDAI score and BILAG score respectively.According toSLE classification criteria from Chinese Rheumatology Association in 2010:ASLEDAI score>15 suggests SSLE.While in BILAG score,patients with any one system up to A or at least three systems up to B are graded as SSLE.Patients fulfilled any one of these two scoes were stratified into SSLE group.Otherwise,they would be stratified into non-SSLE group.We compared these two scoresto evaluate their sensitivity and usefulness.Part Two:A case-control studywas conducted to explore the related factors of SSLE.Referring to these two standards above,we stratified patients into SSLE case group and non-SSLE control group to compare their difference.Collected clinical data included gender,ethnic group,heredity,past pregnancy,clinical manifestations,auxiliary laboratory test results related with disease activity,therapeutic schedule,outcome of disease,hospitalization expenses and so on.Multivariate logistic regression was used to analyze the risk factor of SSLE.Result:1.In the past 10 years,there were 198 SLE patients in this hospital,a total of 628 hospitalizations.According to BILAG-2004 score,127 patients met SSLE criteria and 138 hospitalizations due to critical illness.Referring to SLEDAI-2000 score,71 patients were diagnosed as SSLE and a total of 78 hospitalizations due to critical illness.68 patients and 75 hospitalizations were included following both two score systems.2.130 patients(141 hospitalizations due to critical illness)met these two score systems at the same time,accounting for 22.5%of all the hospitalizations due to SLE(141/628).Three cases(3 hospitalizations)were not eligible for SSLE criteria,but moderate SLE criteria according to BILAG-2004.In all the 3 cases,five systems were affected and the corresponding SLEDAI-2000 score ≥ 15 points,the scores were 16,15,27 respectively.In addition,among these 130 patients,59 patients(63 hospitalizations due to critical illness)did not meet the SSLE criteria according to SLEDAI-2000 score.The median SLEDAI-2000 score of these 59 patients was 8 points,but they reached the level of SSLE following BILAG-2004 score.In these 59 patients,33 cases had affected blood system up to A,15 cases had affected respiratory and circulatory system up to A,11 cases had affected the kidney up to A,5 cases had affected digestive system up to A,4 cases had more than 3 systems up to B.3.The SSLE group included 69 cases(53.1%)with the skin or mucosal diseases as the first clinical manifestations,47 cases(36.2%)with musculo skeletal system firstly affected,39 cases(30%)having non-specific symptom system,28 cases(21.5%)with urinary system firstly affected,27 cases(20.8%)with blood systemfirstly affected,5 cases(3.8%)with respiratory and circulatory systemfirstly affected,2 cases(1.5%)with digestive system firstly affected,2 cases(1.5%)have nervous system and 0%have eye system firstly affected.The non-SSLE group included 47cases(61.9%)with skin or mucosal diseases as the first clinical manifestation,44 cases(64.7%)with musculoskeletal system firstly affected,23cases(33.8%)with non-specific symptom system,12cases(17.6%)with the urinary systemfirstly affected,10cases(14.7%)with blood system firstly affected,2 cases(2.9%)with digestive systemfirstly affected,and nocases(0%)having respiratory,circulatory,nervous and eye system impaired as the first manifestation.These systems referred above were significantly different between SSLE group and non-SSLE group(P<0.05).The SSLE group had 120 cases(85.1%)with blood system mainly affected,95 cases(67.4%)with urinary system mainly affected,69 cases(48.9%)with skin and mucous membrane system mainly affected,62 cases(44.0%)with non-specific symptom system,53 cases with respiratory and circulatory system mainly affected(37.5%),32 cases(22.7%)with the nervous system mainly affected,26 cases(18.4%)with the digestive system mainly affected,1 case(0.7%)with the eye system mainly affected.The non-SSLE group included 36 cases(52.9%)with blood system mainly affected,20 cases(29.4%)with urinary system mainly affected,48 cases(70.6%)with skin and mucous membrane system mainly affected,46 cases(67.6%)with musculo skeletal system mainly affected,21 cases(30.9%)with non-specific symptom,4 cases(5.9%)with the digestive system,respiratory and circulatory system mainly affected,3 cases(4.4%)with respiratory and circulatory system mainly affected,lcase(1.5%)with eye system mainly affected,nocase(0%)with the nervous system mainly affected.Therewas no significant difference in non-specific symptom system and eye system between these two groups(P>0.05).However,in other systems,the difference was statistically significant(P<0.05).4.There were totally 27 males and 171 females in the study(M:F= 1:6.3).The SSLE group consisted of 21 males and 109 females(M:F= 1:5.2),and the non-SSLE group was made up of 6 males and 62 females(M:F= 1:10.3),no statistical significant difference was observed between these two groups(P = 0.154>0.05).The SSLE group consisted of 102 Dalian urban residents(78.5%)(including suburbs)and 28 Dalian rural residents(21.5%).Whereas the non-SSLE group consisted of 61Dalian urban residents(89.7%)(including suburbs)and 7 Dalian rural residents(10.3%).The SSLE group contained 84 patients(64.6%)with age<50 and 46 patients(35.4%)with age≥ 50.While the SSLE group contained 54 patients(79.4%)with age<50 and 14 patients(20.6%)with age ≥ 50.The SSLE group consisted of 102 cases(72.3%)without definite inducement and 39 cases(27.7%)with definite inducement.While the non-SSLE group consisted of 61 cases(89.8%)without definite inducement and 7 cases(10.2%)with definite inducement.The SSLE group consisted of 63 patients(48.5%)with the number of invovled organs ≤3 and 67 patients(51.5%)with the number of involved organs>3.The non-SSLEgroup consisted of57 patients(83.8%)with the number of invovled organs ≤ 3 and 11 patients(16.2%)with the number of invovled organs>3.These factors above were significantly different between these two groups(P<0.05).Moreover,ethnic group,occupation,age of onset,hospitalization age,tuberculosis infection history,family history,pregnancy and abortion history,course of disease were not statistically significantly different between these two groups(P>0.05)5.We compared the clinical lab indexes in SSLE group and non-SSLE group and found that 24-hour urine protein quantitation,BUN,ALB,AST,CRP,ESR,C3,C4,IgA,ANA titer and anti-SSB antibody between these two groups were significantly different(P<0.05).No significant difference was observed in the levels of ALT,TB,DB,IgG,IgM,anti-dsDNA antibody and ENA(except anti-SSB antibody)between two groups(P>0.05).6.Logistic regression analysis showed that SSLE was correlated with the inducement(wether it is clear or not),the number of involved organ systems(whether the number≥3),residence,age at diagnose(whether the age ≥50),the rate of ESR,the level of ANA,IgA and anti-SSB antibody(P<0.05).7.In the past 10 years,a total of 10 SSLE patients died,the mortality was 7.7%(10/130).Infection and multiple organ failure were leading death cause for SSLE.There was no death report in the non-SSLE group.Conclusions:1.In comparison with SLEDAI-2000 score,BILAG-2004 score has higher sensitivity and lower misdiagnosis rate.Besides,BILAG-2004 score is in great accordance with SLEDAI-2000.However,BILAG-2004 score requiresmore parameters and complicated methods,which limits its application in clinical practice.So BILAG-2004 score is recommended to assess SSLE firstly.If condition permits,the assessment can also be combined with SLEDAI-2000 score.2.The affected skin and mucous membrane system and musculo skeletal system are the most common first clinical manifestation in SSLE patients.While the blood and urinary systems are mainly involved organs,which is different from non-SSLE patients.Definite inducement,more involved organ systems,living in rural area,high age at diagnosis,higher erythrocyte sedimentation rate(ESR),medium and high level of ANA and IgA,anti-SSB antibody are all the risk factors for the development of SSLE.Compared with female,male patients are more likely to develop into SSLE;3.In the past 10 years,the in-hospital mortality of SSLE is 7.7%(10/130).Infection and multiple organ failure areleading death causes.