Recently we have shown the presence of catalytically active IgGs, capable to cleave histone H1 and bovine myelin basic protein (MBP), in blood serum of SLE patients. Here we present data that demonstrate the correlati...Recently we have shown the presence of catalytically active IgGs, capable to cleave histone H1 and bovine myelin basic protein (MBP), in blood serum of SLE patients. Here we present data that demonstrate the correlation between a) proteolytic activity towards histone H1 and MBP of IgG-antibodies from blood serum of SLE patients and b) disease severity level in these patients. IgGs were isolated from blood serum by chromatography on protein G-sepharose. Commercial preparations of bovine myelin basic proteins (MBP) and calf thymus histone H1 were used as substrates. Analysis of the proteolytic activity showed that 16 of 38 lgG-preparations (42,1%) obtained from blood serum of SLE patients were capable of cleaving both histone H1 and MBP with different efficiency. It was revealed that the presence in blood serum of lgGs possessing proteolytic activity towards both histone H1 and bMBP closely correlates with manifestation of the disease severity in SLE patients.展开更多
Objective:To evaluate the effects of rituximab versus mycophenolate mofetil or cyclophosphamide as control in lupus nephritis by meta-analysis.Methods:A systematic search was carried out up to January 2022,obtaining 7...Objective:To evaluate the effects of rituximab versus mycophenolate mofetil or cyclophosphamide as control in lupus nephritis by meta-analysis.Methods:A systematic search was carried out up to January 2022,obtaining 7 studies involving 645 participants with lupus nephritis at the commencement of the investigation;198 of them were treated with rituximab,while 447 were treated with mycophenolate mofetil or cyclophosphamide.We determined the odds ratio(OR)and mean difference(MD)with 95%confidence index(CI)to compare rituximab’s efficacy to that of mycophenolate mofetil or cyclophosphamide as control in lupus nephritis using random-or fixed-effects model by dichotomous or continuous techniques.Results:The rituximab group showed significantly higher complete renal remission rate(OR=2.52;95%CI 1.30-4.91,P=0.006)and total renal remission rates(OR=2.22;95%CI 1.36-3.63,P=0.001)than the control group.However,there was no significant difference in terms of end Systemic Lupus Erythematosus Disease Activity Index(SLEDAI)score(MD-1.16;95%CI-2.88-0.57,P=0.19),proteinuria(MD-0.31;95%CI-0.70-0.09,P=0.013),and serum creatinine(MD 0.01;95%CI-0.04-0.07,P=0.64)between the rituximab group and the control.Conclusion:Rituximab exhibited significantly greater complete renal remission rate and total renal remission rates,with no significant difference in terms of shorter-end SLEDAI,proteinuria,and serum creatinine,compared with the control in individuals with lupus nephritis.展开更多
Activated phosphoinositide 3-kinase d syndrome 1(APDS1)is a primary immunode-ficiency disease caused by gain-of-function mutations in PIK3CD.Clinical features of autoimmune disease have been reported in patients with ...Activated phosphoinositide 3-kinase d syndrome 1(APDS1)is a primary immunode-ficiency disease caused by gain-of-function mutations in PIK3CD.Clinical features of autoimmune disease have been reported in patients with APDS1.In this study,we reported three patients with APDS1 presenting with systemic lupus erythematosus(SLE)phenotype.The clinical manifestations included recurrent respiratory tract infection,lymphoproliferation,Coombs-positive hemolytic anemia,decreased complement fractions,positive antinuclear antibodies,renal complications related to SLE associated diseases,which met the clinical spectrum of APDS1 and the classification criteria of SLE.The immunological phenotype included an inversion in the CD4:CD8 ratio,an increase in both non-circulating Tfh CD4^(+)memory T and circulating Tfh populations,a low level of recent thymic emigrant T cells,overexpression of CD57 on T cells,and a decrease in B cells with fewer antibody class switch recombination.These phenotypes detected in patients with APDS1 presenting with SLE were resemble that in patients with APDS1 presenting without SLE.Meanwhile,we described the effect of glucocorticoids and rapamycin therapy on patients with APDS1.The phosphorylation of S6 at Ser235/236 was inhibited in patients with APDS1 who underwent glucocorticoids therapy,including two who presented with SLE phenotype.The phosphorylation of AKT at Ser473 and phosphorylation of S6 at Ser235/236 were inhibited in other patients with APDS1 who underwent rapamycin therapy.Here,we showed the coexistence of immunodeficiency and SLE phenotype in APDS1,and the inhibition of rapamycin in activated Akt-mTOR signaling pathway.展开更多
Background：Conventional magnetic resonance imaging （MRI） is the preferred neuroimaging method in the evaluation ofneuropsychiatric systemic lupus erythematosus （NPSLE）.The purpose of this study was to investigate...Background：Conventional magnetic resonance imaging （MRI） is the preferred neuroimaging method in the evaluation ofneuropsychiatric systemic lupus erythematosus （NPSLE）.The purpose of this study was to investigate the association between clinical and immunological features with MRI abnormalities in female patients with NPSLE,to screen for the value of conventional MRI in NPSLE.Methods：A total of 59 female NPSLE patients with conventional MRI examinations were enrolled in this retrospective study.All patients were classified into different groups according to MRI abnormalities.Both clinical and immunological features were compared between MRI abnormal and normal groups.One-way analysis of variance was used to compare the systemic lupus erythematosus disease activity index （SLEDAI） score for MRI abnormalities.Multivariate logistic regression analysis investigated the correlation between immunological features,neuropsychiatric manifestations,and MRI abnormalities.Results：Thirty-six NPSLE patients （61%） showed a variety of MRI abnormalities.There were statistically significant differences in SLEDAI scores （P 〈 0.001）,incidence of neurologic disorders （P =0.001）,levels of 24-h proteinuria （P =0.001） and immunoglobulin M （P =0.004）,and incidence of acute confusional state （P =0.002）,cerebrovascular disease （P =0.004）,and seizure disorder （P =0.028） between MRI abnormal and normal groups.In the MRI abnormal group,SLEDAI scores for cerebral atrophy （CA）,cortex involvement,and restricted diffusion （RD） were much higher than in the MRI normal group （P 〈 0.001,P =0.002,P =0.038,respectively）.Statistically significant positive correlations between seizure disorder and cortex involvement （odds ratio [OR] =14.90;95% confidence interval [CI],1.50-151.70;P =0.023） and cerebrovascular disease and infratentorial involvement （OR =10.00;95% CI,1.70-60.00;P =0.012） were found.Conclusions：MRI abnormalities in NPSLE,especially CA,cortex involvement,and RD might be markers of high systemic lupus erythematosus activity.Some MRI abnormalities might correspond to neuropsychiatric manifestations and might be helpful in understanding the pathophysiology of NPSLE.展开更多
目的探讨系统性红斑狼疮(Systemic Lupus Erythematosus,SLE)患者中性粒细胞与淋巴细胞比值、血小板与淋巴细胞比值、中性粒细胞与补体C3的比值与疾病活动度的关系,并根据其水平变化评估患者疾病活动度。方法前瞻性方便选取2022年9月—2...目的探讨系统性红斑狼疮(Systemic Lupus Erythematosus,SLE)患者中性粒细胞与淋巴细胞比值、血小板与淋巴细胞比值、中性粒细胞与补体C3的比值与疾病活动度的关系,并根据其水平变化评估患者疾病活动度。方法前瞻性方便选取2022年9月—2023年9月高邮市中医医院收治的86例SLE患者为研究对象,根据患者治疗前SLE疾病活动度评分分为轻度活动组、中度活动组、重度活动组,患者均口服泼尼松连续治疗3个月,比较治疗前后疾病活动度及实验室指标。结果重度活动组中性粒细胞与淋巴细胞比值(Neutrophil to Lymphocyte Ratio,NLR)、血小板与淋巴细胞比值(Platelet to Lymphocyte Ratio,PLR)水平高于轻度活动组和中度活动组,中性粒细胞与补体C3的比值(Neutrophil to Complement C3 Ratio,NC3R)水平低于轻度活动组和中度活动组,差异有统计学意义(P均<0.05);NLR、PLR与疾病活动度正相关(r=0.053、0.299,P均<0.05),NC3R与疾病活动度负相关(r=-0.673,P<0.05)。结论SLE患者经泼尼松治疗后NLR、PLR及NC3R水平变化与疾病活动度密切相关。展开更多
文摘Recently we have shown the presence of catalytically active IgGs, capable to cleave histone H1 and bovine myelin basic protein (MBP), in blood serum of SLE patients. Here we present data that demonstrate the correlation between a) proteolytic activity towards histone H1 and MBP of IgG-antibodies from blood serum of SLE patients and b) disease severity level in these patients. IgGs were isolated from blood serum by chromatography on protein G-sepharose. Commercial preparations of bovine myelin basic proteins (MBP) and calf thymus histone H1 were used as substrates. Analysis of the proteolytic activity showed that 16 of 38 lgG-preparations (42,1%) obtained from blood serum of SLE patients were capable of cleaving both histone H1 and MBP with different efficiency. It was revealed that the presence in blood serum of lgGs possessing proteolytic activity towards both histone H1 and bMBP closely correlates with manifestation of the disease severity in SLE patients.
文摘Objective:To evaluate the effects of rituximab versus mycophenolate mofetil or cyclophosphamide as control in lupus nephritis by meta-analysis.Methods:A systematic search was carried out up to January 2022,obtaining 7 studies involving 645 participants with lupus nephritis at the commencement of the investigation;198 of them were treated with rituximab,while 447 were treated with mycophenolate mofetil or cyclophosphamide.We determined the odds ratio(OR)and mean difference(MD)with 95%confidence index(CI)to compare rituximab’s efficacy to that of mycophenolate mofetil or cyclophosphamide as control in lupus nephritis using random-or fixed-effects model by dichotomous or continuous techniques.Results:The rituximab group showed significantly higher complete renal remission rate(OR=2.52;95%CI 1.30-4.91,P=0.006)and total renal remission rates(OR=2.22;95%CI 1.36-3.63,P=0.001)than the control group.However,there was no significant difference in terms of end Systemic Lupus Erythematosus Disease Activity Index(SLEDAI)score(MD-1.16;95%CI-2.88-0.57,P=0.19),proteinuria(MD-0.31;95%CI-0.70-0.09,P=0.013),and serum creatinine(MD 0.01;95%CI-0.04-0.07,P=0.64)between the rituximab group and the control.Conclusion:Rituximab exhibited significantly greater complete renal remission rate and total renal remission rates,with no significant difference in terms of shorter-end SLEDAI,proteinuria,and serum creatinine,compared with the control in individuals with lupus nephritis.
基金This work was supported by the Natural Science Foundation of China[grant number 81974255]Science and Technology Research Program of Chongqing Municipal Education Commission,China[grant number KJZD-M201800401].
文摘Activated phosphoinositide 3-kinase d syndrome 1(APDS1)is a primary immunode-ficiency disease caused by gain-of-function mutations in PIK3CD.Clinical features of autoimmune disease have been reported in patients with APDS1.In this study,we reported three patients with APDS1 presenting with systemic lupus erythematosus(SLE)phenotype.The clinical manifestations included recurrent respiratory tract infection,lymphoproliferation,Coombs-positive hemolytic anemia,decreased complement fractions,positive antinuclear antibodies,renal complications related to SLE associated diseases,which met the clinical spectrum of APDS1 and the classification criteria of SLE.The immunological phenotype included an inversion in the CD4:CD8 ratio,an increase in both non-circulating Tfh CD4^(+)memory T and circulating Tfh populations,a low level of recent thymic emigrant T cells,overexpression of CD57 on T cells,and a decrease in B cells with fewer antibody class switch recombination.These phenotypes detected in patients with APDS1 presenting with SLE were resemble that in patients with APDS1 presenting without SLE.Meanwhile,we described the effect of glucocorticoids and rapamycin therapy on patients with APDS1.The phosphorylation of S6 at Ser235/236 was inhibited in patients with APDS1 who underwent glucocorticoids therapy,including two who presented with SLE phenotype.The phosphorylation of AKT at Ser473 and phosphorylation of S6 at Ser235/236 were inhibited in other patients with APDS1 who underwent rapamycin therapy.Here,we showed the coexistence of immunodeficiency and SLE phenotype in APDS1,and the inhibition of rapamycin in activated Akt-mTOR signaling pathway.
基金This study was supported by the Provincial Natural Science Foundation of Shandong
文摘Background：Conventional magnetic resonance imaging （MRI） is the preferred neuroimaging method in the evaluation ofneuropsychiatric systemic lupus erythematosus （NPSLE）.The purpose of this study was to investigate the association between clinical and immunological features with MRI abnormalities in female patients with NPSLE,to screen for the value of conventional MRI in NPSLE.Methods：A total of 59 female NPSLE patients with conventional MRI examinations were enrolled in this retrospective study.All patients were classified into different groups according to MRI abnormalities.Both clinical and immunological features were compared between MRI abnormal and normal groups.One-way analysis of variance was used to compare the systemic lupus erythematosus disease activity index （SLEDAI） score for MRI abnormalities.Multivariate logistic regression analysis investigated the correlation between immunological features,neuropsychiatric manifestations,and MRI abnormalities.Results：Thirty-six NPSLE patients （61%） showed a variety of MRI abnormalities.There were statistically significant differences in SLEDAI scores （P 〈 0.001）,incidence of neurologic disorders （P =0.001）,levels of 24-h proteinuria （P =0.001） and immunoglobulin M （P =0.004）,and incidence of acute confusional state （P =0.002）,cerebrovascular disease （P =0.004）,and seizure disorder （P =0.028） between MRI abnormal and normal groups.In the MRI abnormal group,SLEDAI scores for cerebral atrophy （CA）,cortex involvement,and restricted diffusion （RD） were much higher than in the MRI normal group （P 〈 0.001,P =0.002,P =0.038,respectively）.Statistically significant positive correlations between seizure disorder and cortex involvement （odds ratio [OR] =14.90;95% confidence interval [CI],1.50-151.70;P =0.023） and cerebrovascular disease and infratentorial involvement （OR =10.00;95% CI,1.70-60.00;P =0.012） were found.Conclusions：MRI abnormalities in NPSLE,especially CA,cortex involvement,and RD might be markers of high systemic lupus erythematosus activity.Some MRI abnormalities might correspond to neuropsychiatric manifestations and might be helpful in understanding the pathophysiology of NPSLE.
文摘目的探讨系统性红斑狼疮(Systemic Lupus Erythematosus,SLE)患者中性粒细胞与淋巴细胞比值、血小板与淋巴细胞比值、中性粒细胞与补体C3的比值与疾病活动度的关系,并根据其水平变化评估患者疾病活动度。方法前瞻性方便选取2022年9月—2023年9月高邮市中医医院收治的86例SLE患者为研究对象,根据患者治疗前SLE疾病活动度评分分为轻度活动组、中度活动组、重度活动组,患者均口服泼尼松连续治疗3个月,比较治疗前后疾病活动度及实验室指标。结果重度活动组中性粒细胞与淋巴细胞比值(Neutrophil to Lymphocyte Ratio,NLR)、血小板与淋巴细胞比值(Platelet to Lymphocyte Ratio,PLR)水平高于轻度活动组和中度活动组,中性粒细胞与补体C3的比值(Neutrophil to Complement C3 Ratio,NC3R)水平低于轻度活动组和中度活动组,差异有统计学意义(P均<0.05);NLR、PLR与疾病活动度正相关(r=0.053、0.299,P均<0.05),NC3R与疾病活动度负相关(r=-0.673,P<0.05)。结论SLE患者经泼尼松治疗后NLR、PLR及NC3R水平变化与疾病活动度密切相关。
