Clinical Analysis Of 40 Children With Severe Systemic Lupus Erythematosus

ObjectiveThe clinical features of 40 children with severe systemic lupus erythematosus(SLE)were analyzed to provide the basis for the early diagnosis of severe SLE in children,To investigate the clinical significance of anti-neutrophil cytoplasmic antibody(ANCA)in children with severe SLE.MethodsRetrospective analysis of age,clinical manifestations,laboratory tests and systemic lupus erythematosus activity index scores of 40 children with severe systemic lupus erythematosus diagnosed and first hospitalized in our hospital from January 2009 to December 2018.Analysis:1.According to the gender,divided into severe SLE male and female groups.Comparing the clinical manifestations,laboratory tests,and systemic lupus erythematosus disease activity index scores.2.The clinical data of 68 children with light SLE who were hospitalized during the same period were collected as the control group.They were divided into two groups:light and severe SLE.The differences between the two groups were compared.3.According to the children's detection of ANCA,they were divided into severe SLE ANCA-positive and severe SLE ANCA-negative groups.The clinical data of the two groups were compared.Result1.Of the 40 children with severe SLE,11 were male and 29 were female.The male to female ratio was 1:2.64,and the mean age of onset was 10.79±2.74 years.There was no significant difference in the average age of onset between men and women(t=0.465,P>0.05.2.Among the 40 cases of severe organ involvement in SLE,15 cases(37.5%)had severe renal damage,including 12 cases of nephrotic syndrome and 3 cases of acute renal failure;Severe blood system damage in 7 cases(17.5%),3 cases of hemolytic anemia,1 case of severe neutropenia(WBC<1×109/L),3 cases of severe thrombocytopenia(<50×109/L);Severe respiratory system damage was found in 7 cases(17.5%),including 6 cases of pneumonia and 1 case of pulmonary hemorrhage;Severe neurological damage was found in 5 cases(12.5%),including 2 cases of coma,3 cases of convulsion.There were 5 cases of severe cardiovascular system(12.5%),including 3 cases of myocarditis,1 case of severe pericarditis,1 case of malignant hypertension.One case(2.5%)of digestive system damage was acute pancreatitis.3.Among the 40 patients with severe SLE,27(67.5%)presented skin rash,25(62.5%)presented fever,31(77.5%)presented blood system damage,26(65%)presented kidney damage,19(47.5%)presented lymph node enlargement,and 13(32.5%)presented liver or spleen enlargement.4.Serological examination:autoantibody in resistance to ANA antibody positive 40 cases(100%),anti ds-DNA antibody positive 29 cases(72.5%),anti nucleosome antibody positive 28 cases(70.0%),anti SSA antibody positive 19 cases(47.5%),anti AHA antibody positive 17 cases(42.5%),anti Sm antibody positive 15 cases(37.5%),anti rRNP antibody positive 14 cases(35.0%),anti SSB antibody positive in 7 cases(17.5%);The increased proportion of IgA,IgG and IgM was 87.5%,85.0%and 42.5%respectively.C4 decreased in 35 cases(87.5%)and C3 decreased in 32 cases(80.0%).5.Thirty-six of the 40 patients with severe SLE a complete renal biopsy,the results showed there were kidney damage,Among them,22 cases(61.1%)were most common in lupus nephritis type IV,followed by lupus nephritis type II and lupus nephritis type ? in 6 cases(both 16.7%),and 2 cases(5.6%)of lupus nephritis.No lupus nephritis type I and lupus nephritis type VI were found.6.The number of male children with leukopenia and neutropenia and increased ALT and AST was significantly higher than that of female children.The SLEDAI score of male children was significantly higher than that of female children,and there were significant differences between the two groups(P<0.05).7.HB,PLT,C3 and C4 in children with severe SLE were significantly lower than those in children with mild SLE.WBC,LY,ALT,AST,24h urinary protein,CRP,serum creatinine,BUN,and ferritin were significantly higher in children with severe SLE than in children with mild SLE;The incidence of aseptic pyuria,proteinuria,hematuria,anti-dsDNA antibody positive,anti-nucleosome antibody positive,and ANCA positive in children with severe SLE were significantly higher than those in children with mild SLE.The difference was statistically significant(P<0.05).8.The levels of serum C3,C4 and albumin in ANCA(+)group were significantly lower than those in ANCA(-)group;the positive rate of anti-dsDNA antibody,anti-nucleosome antibody,aseptic pyuria,hematuria and proteinuria in ANCA(+)group was significantly higher than ANCA The(-)group;ANCA(+)group 24h urine protein,serum creatinine,urea nitrogen,SLEDAI score was significantly higher than the ANCA(-)group.The differences were statistically significant(P<0.05).9.The severe SLE ANCA(+)group and the severe SLE ANCA(-)group have significant differences in pathological typing composition,P=0.005,and the severe SLE ANCA(+)group has heavier pathological typing.Conclusion:1.Peripheral hemoglobin,blood platelets decreased significantly,complement C3 and C4 decreased significantly,ALT,AST,serum creatinine,urea nitrogen,ferritin increased significantly,ANCA positive may indicate severe systemic lupus erythematosus in children.2.For children with severe SLE in men,it is easier to combine with white blood cells,especially neutropenia and abnormal liver function.3.The positive rate of ANCA in children with severe SLE is high,suggesting that there may be a certain correlation between ANCA positive and severe SLE,and may indicate the activity of SLE.