AIM:To establish the frequency and clinical features of connective tissue diseases(CTDs)in a cohort of Chinese patients with primary biliary cirrhosis(PBC).METHODS:Three-hundred and twenty-two Chinese PBC patients wer...AIM:To establish the frequency and clinical features of connective tissue diseases(CTDs)in a cohort of Chinese patients with primary biliary cirrhosis(PBC).METHODS:Three-hundred and twenty-two Chinese PBC patients were screened for the presence of CTD,and the systemic involvement was assessed.The differences in clinical features and laboratory findings between PBC patients with and without CTD were documented.The diversity of incidence of CTDs in PBC of different countries and areas was discussed.For the comparison of normally distributed data,Student’s t test was used,while non-parametric test(Wilcoxon test)for the non-normally distributed data and 2×2χ2or Fisher’s exact tests for the ratio.RESULTS:One-hundred and fifty(46.6%)PBC patients had one or more CTDs.The most common CTD was Sj gren’s syndrome(SS,121 cases,36.2%).There were nine cases of systemic sclerosis(SSc,2.8%),12of systemic lupus erythematosus(SLE,3.7%),nine of rheumatoid arthritis(RA,2.8%),and 10 of polymyositis(PM,3.1%)in this cohort.Compared to patients with PBC only,the PBC+SS patients were more likely to have fever and elevated erythrocyte sedimentation rate(ESR),higher serum immunoglobulin G(IgG)levels and more frequent rheumatoid factor(RF)and interstitial lung disease(ILD)incidences;PBC+SSc patients had higher frequency of ILD;PBC+SLE patients had lower white blood cell(WBC)count,hemoglobin(Hb),platelet count,γ-glutamyl transpeptidase and immunoglobulin M levels,but higher frequency of renal involvement;PBC+RA patients had lower Hb,higher serum IgG,alkaline phosphatase,faster ESR and a higher ratio of RF positivity;PBC+PM patients had higher WBC count and a tendency towards myocardial involvement.CONCLUSION:Besides the common liver manifestation of PBC,systemic involvement and overlaps with other CTDs are not infrequent in Chinese patients.When overlapping with other CTDs,PBC patients manifested some special clinical and laboratory features which may have effect on the prognosis.展开更多
目的分析硬皮病相关自身抗体谱[包括抗Scl-70抗体、抗RNA多聚酶Ⅲ抗体(ARA-Ⅲ)、抗着丝点抗体(ACA)和抗U1核糖核蛋白(U1RNP)抗体]与中国系统性硬化症(SSc)患者临床特征的相关性。方法前瞻性序贯纳入入选欧洲抗风湿病联盟硬皮病试验研究...目的分析硬皮病相关自身抗体谱[包括抗Scl-70抗体、抗RNA多聚酶Ⅲ抗体(ARA-Ⅲ)、抗着丝点抗体(ACA)和抗U1核糖核蛋白(U1RNP)抗体]与中国系统性硬化症(SSc)患者临床特征的相关性。方法前瞻性序贯纳入入选欧洲抗风湿病联盟硬皮病试验研究组(EULAR Scleroderma Trial and Research group,EUSTAR)的92例中国SSc患者,记录其临床表现和实验室检查结果,同时检测抗Scl-70抗体、ARA-Ⅲ、ACA和抗U1RNP抗体。统计分析上述自身抗体与患者各种临床特征之间的相关性。结果 92例患者中,弥漫型SSc65例,局限型SSc19例,重叠综合征6例,伴有雷诺现象的未分化结缔组织病2例。抗Scl-70抗体、ARA-Ⅲ、ACA和抗U1RNP抗体的阳性率分别为55%、13%、13%和25%。存在抗Scl-70抗体者具有较高的皮肤硬化修订Rodnan评分、免疫球蛋白(Ig)M及血小板水平,但肺动脉高压发生率、IgG水平则显著减低(P<0.05),与肺间质病变或肾危象的相关性未获证实。ARA-Ⅲ与皮肤硬化评分,毛细血管扩张,心、肺、肾脏受累等临床特点均无显著相关性。存在ACA的患者尿酸水平较高,但关节炎或关节痛症状和肺间质病变少见(P<0.05)。存在抗U1RNP抗体者肺动脉高压和心脏受累的发生率、IgG和乳酸脱氢酶水平均增高,但白细胞和血小板较低(P<0.05)。结论在SSc患者血清中检测上述硬皮病相关自身抗体有助于预测某些临床表现和脏器病变的发生。中国患者自身抗体的临床相关性可能与其他地区患者存在种族差异,值得进行更大样本的研究证实。展开更多
AIM To investigate T-cell activation, the percentage of peripheral T regulatory cells(Tregs), Th17 cells and the circulating cytokine profile in systemic sclerosis(SSc).METHODS We enrolled a total of 24 SSc patients a...AIM To investigate T-cell activation, the percentage of peripheral T regulatory cells(Tregs), Th17 cells and the circulating cytokine profile in systemic sclerosis(SSc).METHODS We enrolled a total of 24 SSc patients and 16 healthy controls in the study and divided the patients as having diffuse cutaneous SSc(dc SSc, n = 13) or limited cutaneous SSc(lc SSc, n = 11). We performed a further subdivision of the patients regarding the stage of the disease-early, intermediate or late. Peripheral venous blood samples were collected from all subjects. We performed flow cytometric analysis of the activationcapacity of T-lymphocytes upon stimulation with PHA-M and of the percentage of peripheral Tregs and Th17 cells in both patients and healthy controls. We used ELISA to quantitate serum levels of human interleukin(IL)-6, IL-10, tissue growth factor-β1(TGF-β1), and IL-17 A.RESULTS We identified a decreased percentage of CD3+CD69+ cells in PHA-stimulated samples from SSc patients in comparison with healthy controls(13.35% ± 2.90% vs 37.03% ± 2.33%, P < 0.001). However, we did not establish a correlation between the down-regulated CD3+CD69+ cells and the clinical subset, nor regarding the stage of the disease. The activated CD4+CD25+ peripheral lymphocytes were represented in decreased percentage in patients when compared to controls(6.30% ± 0.68% vs 9.36% ± 1.08%, P = 0.016). Regarding the forms of the disease, dc SSc patients demonstrated lower frequency of CD4+CD25+ T cells against healthy subjects(5.95% ± 0.89% vs 9.36% ± 1.08%, P = 0.025). With regard to Th17 cells, our patients demonstrated increased percentage in comparison with controls(18.13% ± 1.55% vs 13.73% ± 1.21%, P = 0.031). We detected up-regulated Th17 cells within the lc SSc subset against controls(20.46% ± 2.41% vs 13.73% ± 1.21%, P = 0.025), nevertheless no difference was found between dc SSc and lc SSc patients. Flow cytometric analysis revealed an increased percentage of CD4+CD25-Foxp3+ in dc SSc patients compared to controls(10.94% ± 1.65% 展开更多
目的检测中国系统性硬化症（SSc）患者血清中硬皮病相关自身抗体一抗Sel-70抗体、抗着丝点抗体（ACA）和抗RNA多聚酶Ⅲ抗体（ARA），分析其与各种临床表现之间的关系。方法序贯纳入人选欧洲抗风湿病联盟硬皮病实验研究组（EULAR Sclerod...目的检测中国系统性硬化症（SSc）患者血清中硬皮病相关自身抗体一抗Sel-70抗体、抗着丝点抗体（ACA）和抗RNA多聚酶Ⅲ抗体（ARA），分析其与各种临床表现之间的关系。方法序贯纳入人选欧洲抗风湿病联盟硬皮病实验研究组（EULAR Scleroderma Trial and Research Group，EUSTAR）的135例中国SSc患者，分别用线性免疫印迹法、免疫双扩散法和间接免疫荧光法检测ARA、抗Scl-70抗体、ACA在患者血清中表达水平，并进一步分析自身抗体与患者各种临床表现之间的相关性。结果在135例SSc患者中抗Sel-70、ACA、ARA的阳性率分别为49．6％、13．3％和8．9％。抗Scl-70抗体阳性组患者的病程显著短于阴性组[（71±59）个月VS（90±103）个月，P=0．041]，肺间质病变的患炳率亦显著高于阴性组（P=0．031），但阳性组肺动脉高压的患病率显著低于阴性组（P=0．042），修订的Rodnan皮肤硬化评分（P=0．008）、面颈部皮肤硬化（P=0．002）、肘／膝关节远端皮肤硬化（P=0．004）以及指端凹陷性瘢痕／指垫消失的发生率（P=0．01）均显著高于阴性组；ACA阳性组患者的病程长于阴性组，差异具有统计学意义[（90±107）个月VS（69±64）个月，P=0．036]，肺间质病变的患病率显著低于阴性组（P＝0．045），IgM水平亦显著低于阴性组（P=0．045）；ARA阳性组和阴性组患者的病程等各项临床指标差异均无统计学意义，但阳性组血清肌酐和尿素氮水平显著高于阴性组（P〈0．001）。ACA和ARA患者各项皮肤硬化指标在阳性组和阴性组差异均无统计学意义。结论硬皮病特异相关的自身抗体与不同的临床表现紧密相关，检测此类抗体可能有助于SSc的诊断、脏器受累和预后评估。这些自身抗体在中国SSc患者的临床相关性可能不同于其他地区的SSc患者。展开更多
基金Supported by Grants from the Research Special Fund for Public Welfare Industry of Health,No.201202004the National Major Scientific and Technological Special Project for"Significant New Drugs Development",No.2012ZX09303006-002the National High Technology Research and Development Program of China,No.2011AA020111
文摘AIM:To establish the frequency and clinical features of connective tissue diseases(CTDs)in a cohort of Chinese patients with primary biliary cirrhosis(PBC).METHODS:Three-hundred and twenty-two Chinese PBC patients were screened for the presence of CTD,and the systemic involvement was assessed.The differences in clinical features and laboratory findings between PBC patients with and without CTD were documented.The diversity of incidence of CTDs in PBC of different countries and areas was discussed.For the comparison of normally distributed data,Student’s t test was used,while non-parametric test(Wilcoxon test)for the non-normally distributed data and 2×2χ2or Fisher’s exact tests for the ratio.RESULTS:One-hundred and fifty(46.6%)PBC patients had one or more CTDs.The most common CTD was Sj gren’s syndrome(SS,121 cases,36.2%).There were nine cases of systemic sclerosis(SSc,2.8%),12of systemic lupus erythematosus(SLE,3.7%),nine of rheumatoid arthritis(RA,2.8%),and 10 of polymyositis(PM,3.1%)in this cohort.Compared to patients with PBC only,the PBC+SS patients were more likely to have fever and elevated erythrocyte sedimentation rate(ESR),higher serum immunoglobulin G(IgG)levels and more frequent rheumatoid factor(RF)and interstitial lung disease(ILD)incidences;PBC+SSc patients had higher frequency of ILD;PBC+SLE patients had lower white blood cell(WBC)count,hemoglobin(Hb),platelet count,γ-glutamyl transpeptidase and immunoglobulin M levels,but higher frequency of renal involvement;PBC+RA patients had lower Hb,higher serum IgG,alkaline phosphatase,faster ESR and a higher ratio of RF positivity;PBC+PM patients had higher WBC count and a tendency towards myocardial involvement.CONCLUSION:Besides the common liver manifestation of PBC,systemic involvement and overlaps with other CTDs are not infrequent in Chinese patients.When overlapping with other CTDs,PBC patients manifested some special clinical and laboratory features which may have effect on the prognosis.
文摘目的分析硬皮病相关自身抗体谱[包括抗Scl-70抗体、抗RNA多聚酶Ⅲ抗体(ARA-Ⅲ)、抗着丝点抗体(ACA)和抗U1核糖核蛋白(U1RNP)抗体]与中国系统性硬化症(SSc)患者临床特征的相关性。方法前瞻性序贯纳入入选欧洲抗风湿病联盟硬皮病试验研究组(EULAR Scleroderma Trial and Research group,EUSTAR)的92例中国SSc患者,记录其临床表现和实验室检查结果,同时检测抗Scl-70抗体、ARA-Ⅲ、ACA和抗U1RNP抗体。统计分析上述自身抗体与患者各种临床特征之间的相关性。结果 92例患者中,弥漫型SSc65例,局限型SSc19例,重叠综合征6例,伴有雷诺现象的未分化结缔组织病2例。抗Scl-70抗体、ARA-Ⅲ、ACA和抗U1RNP抗体的阳性率分别为55%、13%、13%和25%。存在抗Scl-70抗体者具有较高的皮肤硬化修订Rodnan评分、免疫球蛋白(Ig)M及血小板水平,但肺动脉高压发生率、IgG水平则显著减低(P<0.05),与肺间质病变或肾危象的相关性未获证实。ARA-Ⅲ与皮肤硬化评分,毛细血管扩张,心、肺、肾脏受累等临床特点均无显著相关性。存在ACA的患者尿酸水平较高,但关节炎或关节痛症状和肺间质病变少见(P<0.05)。存在抗U1RNP抗体者肺动脉高压和心脏受累的发生率、IgG和乳酸脱氢酶水平均增高,但白细胞和血小板较低(P<0.05)。结论在SSc患者血清中检测上述硬皮病相关自身抗体有助于预测某些临床表现和脏器病变的发生。中国患者自身抗体的临床相关性可能与其他地区患者存在种族差异,值得进行更大样本的研究证实。
文摘AIM To investigate T-cell activation, the percentage of peripheral T regulatory cells(Tregs), Th17 cells and the circulating cytokine profile in systemic sclerosis(SSc).METHODS We enrolled a total of 24 SSc patients and 16 healthy controls in the study and divided the patients as having diffuse cutaneous SSc(dc SSc, n = 13) or limited cutaneous SSc(lc SSc, n = 11). We performed a further subdivision of the patients regarding the stage of the disease-early, intermediate or late. Peripheral venous blood samples were collected from all subjects. We performed flow cytometric analysis of the activationcapacity of T-lymphocytes upon stimulation with PHA-M and of the percentage of peripheral Tregs and Th17 cells in both patients and healthy controls. We used ELISA to quantitate serum levels of human interleukin(IL)-6, IL-10, tissue growth factor-β1(TGF-β1), and IL-17 A.RESULTS We identified a decreased percentage of CD3+CD69+ cells in PHA-stimulated samples from SSc patients in comparison with healthy controls(13.35% ± 2.90% vs 37.03% ± 2.33%, P < 0.001). However, we did not establish a correlation between the down-regulated CD3+CD69+ cells and the clinical subset, nor regarding the stage of the disease. The activated CD4+CD25+ peripheral lymphocytes were represented in decreased percentage in patients when compared to controls(6.30% ± 0.68% vs 9.36% ± 1.08%, P = 0.016). Regarding the forms of the disease, dc SSc patients demonstrated lower frequency of CD4+CD25+ T cells against healthy subjects(5.95% ± 0.89% vs 9.36% ± 1.08%, P = 0.025). With regard to Th17 cells, our patients demonstrated increased percentage in comparison with controls(18.13% ± 1.55% vs 13.73% ± 1.21%, P = 0.031). We detected up-regulated Th17 cells within the lc SSc subset against controls(20.46% ± 2.41% vs 13.73% ± 1.21%, P = 0.025), nevertheless no difference was found between dc SSc and lc SSc patients. Flow cytometric analysis revealed an increased percentage of CD4+CD25-Foxp3+ in dc SSc patients compared to controls(10.94% ± 1.65%
文摘目的检测中国系统性硬化症（SSc）患者血清中硬皮病相关自身抗体一抗Sel-70抗体、抗着丝点抗体（ACA）和抗RNA多聚酶Ⅲ抗体（ARA），分析其与各种临床表现之间的关系。方法序贯纳入人选欧洲抗风湿病联盟硬皮病实验研究组（EULAR Scleroderma Trial and Research Group，EUSTAR）的135例中国SSc患者，分别用线性免疫印迹法、免疫双扩散法和间接免疫荧光法检测ARA、抗Scl-70抗体、ACA在患者血清中表达水平，并进一步分析自身抗体与患者各种临床表现之间的相关性。结果在135例SSc患者中抗Sel-70、ACA、ARA的阳性率分别为49．6％、13．3％和8．9％。抗Scl-70抗体阳性组患者的病程显著短于阴性组[（71±59）个月VS（90±103）个月，P=0．041]，肺间质病变的患炳率亦显著高于阴性组（P=0．031），但阳性组肺动脉高压的患病率显著低于阴性组（P=0．042），修订的Rodnan皮肤硬化评分（P=0．008）、面颈部皮肤硬化（P=0．002）、肘／膝关节远端皮肤硬化（P=0．004）以及指端凹陷性瘢痕／指垫消失的发生率（P=0．01）均显著高于阴性组；ACA阳性组患者的病程长于阴性组，差异具有统计学意义[（90±107）个月VS（69±64）个月，P=0．036]，肺间质病变的患病率显著低于阴性组（P＝0．045），IgM水平亦显著低于阴性组（P=0．045）；ARA阳性组和阴性组患者的病程等各项临床指标差异均无统计学意义，但阳性组血清肌酐和尿素氮水平显著高于阴性组（P〈0．001）。ACA和ARA患者各项皮肤硬化指标在阳性组和阴性组差异均无统计学意义。结论硬皮病特异相关的自身抗体与不同的临床表现紧密相关，检测此类抗体可能有助于SSc的诊断、脏器受累和预后评估。这些自身抗体在中国SSc患者的临床相关性可能不同于其他地区的SSc患者。
