抗磷脂综合征174例临床特点及与欧洲数据的比较分析

Objective To analyze the clinical characteristics of antiphospholipid syndrome (APS) in a cohort of Chinese patients. Methods From January 1996 to October 2009, 174 patients with defined APS were retrospectively studied,χ2 test was selected. Results The cohort consisted of 151(86.8%) female and 23 (13.2%) male. Mean age at study was (42±13) years (range: 14-75 years). Thirty-one (17.8%) patients had primary APS, 143 (82.2% ) patients had APS associated with other diseases, including 138 cases of systemic lupus erythematosus (SLE). One hundred and forty-one (81.0%) had thrombosis episodes, among which the most common presenting manifestations were stroke (27.6%), deep venous thrombosis (27.6%) and pulmonary embolism (6.3% ). Stroke was more prevalent in Chinese than European patients. Forty-five patients (31.9%) had thrombotic recurrences and 62 patients (44.0%) had more than two sites of thrombosis. Patients with primary APS had higher rates of rethrombosis than those with secondary APS (P<0.05). Fetal morbidity rate of 126 married women in our study was 50.0%. Seven of APS patients with APS nephropathy were characterized by thrombotic microangiopathy. The presence of anticardiolipin antibodies was detected in 112 patients (64.4% ), anti-beta-2 glycoprotein Ⅰ antibodies in 103 patients (59.1%) and lupus anticoagulant in 50 patients (28.7%). Conclusion APS is characterized by recurrent thrombosis, pregnancy morbidity and positive antiphospholipid antibodies tests. Stroke, deep venous thrombosis and pulmonary embolism are the most common manifestations both in Chinese and European patients. Rethrombosis is more prominent in primary APS. Thrombotic microangiopathy is one of the most common histological changes of APS nephropathy. Multiple tests for aPL are proposed.     

B细胞在狼疮肾炎肾组织中的分布及临床意义

Objective To investigate B-cell distribution in lupus nephritis and evaluate its significance in pathological classification.Methods Kidney biopsy specimens were obtained consecutively from 119 patients with LN, B lymphocytes distribution were detected using immunohistochemical staining with specific antibodies and were classified into 5 groups.Chi-square test and t test were used for statistical analysis.Results ①B lymphocytes infiltration occurred in 52.1% of the LN patients (62/119), B lymphocytes were mainly distributed in the renal interstitial tissue, which occurred most frequently in class Ⅳ LN but rare in class Ⅴ LN. ②B-cell infiltrating group was associated with active disease presentations and chronic indices P＜0.01). Blood urea nitrogen(BUN)[(12±9) vs (6±3) mmol/L] and serum creatinine(Cr)[(105±84) vs(61±21) μmol/L] were higher in B-cell infiltrates group than non-B-cell infiltration group (all P＜0.01). ③Kidney biopsy specimens showed predominantly IgG deposit in immune-fluorescency intensity examination.The Ig subtype which deposited in B-cell infiltrating group was not statistically different from those deposited in non-B cell infiltrating group (P＞0.05). Conclusion This study has shown that renal B cell infiltration occurs in 52.1% of the LN patients.Patients with renal B cell infiltration have more severe kidney tissue injury and poorer renal outcomes than patients without B cell infiltration. Renal B lymphocytes infiltration may contribute to the pathogenesis of LN in a non-antibody-secreting way.     

初治类风湿关节炎患者腕关节滑膜病变的超声特点及临床分析

目的观察初治类风湿关节炎（RA）患者腕关节滑膜的超声表现。方法对98例初治RA患者行双腕关节超声扫查,取滑膜增生较重一侧腕关节,分别测量桡腕关节、腕中关节和腕掌关节的滑膜增生最厚处及腕关节背侧正中矢状面最显著处两点间的滑膜厚度。对所测滑膜的增生程度进行分级归类,并比较不同超声下所测得滑膜增生情况是否存在差异。结果98例患者桡腕关节均有滑膜增生,96例患者腕中关节有滑膜增生,60例患者腕掌关节有滑膜增生。所有患者桡腕关节、腕中关节及腕掌关节在全腕关节超声下测得的滑膜最大厚度与在腕关节背侧正中矢状面超声下测得的滑膜厚度比较差异均无统计学意义（均P＞0.05）。98例患者腕关节无疼痛7例,轻度疼痛83例,中度疼痛8例。结论RA主要侵犯滑膜小关节,滑膜组织越丰富的关节越容易受累。腕关节背侧正中矢状面的超声检查评估能较好地反映该关节滑膜增生的情况,可作为腕关节超声的一种简易评估方法。     

抗磷脂综合征174例临床特点及与欧洲数据的比较分析

目的 分析中国人群抗磷脂综合征(AIDS)的临床特点,以提高对这一疾病的认识.方法 回顾性分析1996-2009年在上海仁济医院诊治的APS患者的临床和实验室特点.采用χ2检验.结果 确诊APS 174例,男:女为1:6.6.原发性APS 31例,继发性APS 143例,138例继发于系统性红斑狼疮.141例患者发生血栓事件,以脑梗死、深静脉血栓、肺栓塞为主.62例(44.0%)多部位(≥2处)血栓,45例(31.9%)血栓复发(≥2次),且原发性APS较继发性APS更易复发血栓(P<0.05). 63例发生异常妊娠.7例病理诊断APS肾病(APSN),以肾小球微血栓为特点.174例AlaS中,抗心磷脂抗体阳性112例(64.4%),抗β2糖蛋白Ⅰ抗体阳性103例(59.1%),狼疮抗凝物(LA)阳性50例(28.7%).结论 APS以血栓事件和异常妊娠为主要表现,本组APS主要血栓事件与欧洲数据组一致;原发性APS更易复发血栓事件;肾小球微血栓为APSN常见病理表现之一;同时进行3种抗磷脂抗体检测有助于临床诊断APS.     

B细胞在狼疮肾炎肾组织中的分布及临床意义

目的 研究B细胞在狼疮肾炎患者肾组织的浸润情况,探讨 B细胞浸润与狼疮肾炎肾组织病理改变的关系及临床意义.方法 收集119例处于活动期的狼疮肾炎患者肾组织标本.应用免疫酶组织化学方法检测肾组织中B细胞的浸润情况,根据浸润程度分为5级.统计分析采用两独立样本t检验和x2检验.结果 ①119例狼疮肾炎中有B细胞浸润62例(52.1%).B细胞主要浸润于肾间质组织,多见于Ⅳ型狼疮肾炎患者,Ⅴ型少见.②狼疮肾炎肾组织中B细胞浸润同无浸润组比较肾组织活动指数及慢性指数显著升高,差异有统计学意义;B细胞浸润组同无浸润组的血尿素氮水平比较[(12±9)与(6±3)mmol/L]、血清肌酐水平[(105±84)和(61±21)μmol/L](P＜0.01).③狼疮肾炎肾组织中以IgC型抗体荧光强度最强;B细胞浸润组与无B细胞浸润组间各亚型抗体沉积率比较差异无统计学意义(P均＞0.05).结论 狼疮肾炎中伴有肾组织B细胞浸润的患者病变重于无B细胞浸润者,局部B细胞可能以非抗体生成细胞的方式参与狼疮肾炎病变.

Abstract：

Objective To investigate B-cell distribution in lupus nephritis and evaluate its significance in pathological classification.Methods Kidney biopsy specimens were obtained consecutively from 119 patients with LN, B lymphocytes distribution were detected using immunohistochemical staining with specific antibodies and were classified into 5 groups.Chi-square test and t test were used for statistical analysis.Results ①B lymphocytes infiltration occurred in 52.1% of the LN patients (62/119), B lymphocytes were mainly distributed in the renal interstitial tissue, which occurred most frequently in class Ⅳ LN but rare in class Ⅴ LN. ②B-cell infiltrating group was associated with active disease presentations and chronic indices P＜0.01). Blood urea nitrogen(BUN)[(12±9) vs (6±3) mmol/L] and serum creatinine(Cr)[(105±84) vs(61±21) μmol/L] were higher in B-cell infiltrates group than non-B-cell infiltration group (all P＜0.01). ③Kidney biopsy specimens showed predominantly IgG deposit in immune-fluorescency intensity examination.The Ig subtype which deposited in B-cell infiltrating group was not statistically different from those deposited in non-B cell infiltrating group (P＞0.05). Conclusion This study has shown that renal B cell infiltration occurs in 52.1% of the LN patients.Patients with renal B cell infiltration have more severe kidney tissue injury and poorer renal outcomes than patients without B cell infiltration. Renal B lymphocytes infiltration may contribute to the pathogenesis of LN in a non-antibody-secreting way.     

贝利尤单抗治疗重症神经精神性狼疮患者一例并文献复习

系 统 性 红 斑 狼 疮(systemic lupus erythematosus,SLE)是一种多系统累及的自身免疫性疾病,神经精神性狼疮(neuropsychiatric SLE, NPSLE)是其较为严重的并发症,可出现在SLE病程中的任何阶段,大部分发生在SLE初发阶段,是SLE预后不良的主要因素之一.NPS...     

基于ADDIE模型探索专业学位研究生的个性化规培模式——以风湿免疫科为例

目的：基于ADDIE模型,以风湿免疫科为例,探索建立双轨制模式下符合临床医学硕士专业学位研究生专业特点的个性化规培模式。方法：通过ADDIE模型,针对不同专业,分析临床学科交叉特点,在规范化培训方案基础上增加个性化教学内容。在风湿免疫科接受住院医师规范化培训的学员,按转科先后顺序分为试验组和对照组。其中试验组30人,对照组30人。试验组针对不同专业增加个性化教学内容。1个月后对个性化规培教学的效果进行评价,通过客观评价指标和临床带教的满意度进行评价。结果：采用个性化规培方案组学员对个性化规培方案持更加肯定态度(P<0.05);试验组临床综合能力较普通学员有明显提升(P<0.05)。试验组掌握知识更加全面,对自身专业及风湿免疫科交叉知识有更深刻理解,有利于工作后从事复杂的临床工作。结论：该研究基于风湿免疫科,通过ADDIE模型,初步探索建立符合专科特点的个性化住院医师规范化培训模式,使临床医学硕士专业学位研究生在规范化培训期间可以加强学科交叉知识的学习,培养临床科研思维能力,有利于新的教学模式的探索和推广。     

抗磷脂综合征174例临床特点及与欧洲数据的比较分析

Objective To analyze the clinical characteristics of antiphospholipid syndrome (APS) in a cohort of Chinese patients. Methods From January 1996 to October 2009, 174 patients with defined APS were retrospectively studied,χ2 test was selected. Results The cohort consisted of 151(86.8%) female and 23 (13.2%) male. Mean age at study was (42±13) years (range: 14-75 years). Thirty-one (17.8%) patients had primary APS, 143 (82.2% ) patients had APS associated with other diseases, including 138 cases of systemic lupus erythematosus (SLE). One hundred and forty-one (81.0%) had thrombosis episodes, among which the most common presenting manifestations were stroke (27.6%), deep venous thrombosis (27.6%) and pulmonary embolism (6.3% ). Stroke was more prevalent in Chinese than European patients. Forty-five patients (31.9%) had thrombotic recurrences and 62 patients (44.0%) had more than two sites of thrombosis. Patients with primary APS had higher rates of rethrombosis than those with secondary APS (P<0.05). Fetal morbidity rate of 126 married women in our study was 50.0%. Seven of APS patients with APS nephropathy were characterized by thrombotic microangiopathy. The presence of anticardiolipin antibodies was detected in 112 patients (64.4% ), anti-beta-2 glycoprotein Ⅰ antibodies in 103 patients (59.1%) and lupus anticoagulant in 50 patients (28.7%). Conclusion APS is characterized by recurrent thrombosis, pregnancy morbidity and positive antiphospholipid antibodies tests. Stroke, deep venous thrombosis and pulmonary embolism are the most common manifestations both in Chinese and European patients. Rethrombosis is more prominent in primary APS. Thrombotic microangiopathy is one of the most common histological changes of APS nephropathy. Multiple tests for aPL are proposed.