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.     

抗磷脂综合征100例临床特征分析

目的探讨抗磷脂综合征（APS）的临床特征和血清学特点。方法回顾性分析总结100例APS患者的临床表现和免疫学改变。结果原发性APS（PAPS）37例，继发性APS（SAPS）63例，其中继发于系统性红斑狼疮（SLE）46例、狼疮样综合征14例。80％患者出现血管性血栓形成，其中静脉血栓43例、动脉闭塞18例、静脉血栓和动脉闭塞15例。52％为单一部位，32％有2个部位，15％多个部位血栓。56％单次，25％2次，19％多次血栓形成。血栓事件以下肢深静脉血栓（36％）、肺栓塞（30％）和脑卒中（26％）常见。51％（34／67）女性出现病态妊娠，胎死宫内占37％，习惯性自发性流产13％。血小板减少71例。抗心磷脂抗体（ACL）和狼疮抗凝物（LA）的阳性率分别为84％和58％。SLE继发APS组关节炎、白细胞减少、抗核抗体（ANA）阳性和低补体血症较PAPS多见。男性患者下肢深静脉血栓和LA阳性较多见；而女性白细胞减少、ANA阳性和ACL阳性较多见。结论APS以血栓形成及病态妊娠为特点．高滴度ACL和（或）LA阳性是其免疫学特征。其临床表现受基础疾病（SLE）和性别的影响而有不同。     

抗磷脂综合征165例临床特征与分型

目的 分析抗磷脂综合征(APS)的临床特征与分型.方法 根据2006年更新的APS分类诊断标准及新的临床亚型的定义,回顾性分析北京协和医院住院的165例APS患者的临床分型,总结患者的临床表现及治疗,并分析抗磷脂抗体与血栓的相关性.结果 165例患者中,男:女为1:3.不同临床亚型的分类包括确诊APS 116例(70.3%),可能APS 34例(20.6%),血清阴性APS 10例(6.1%),微血管性APS 5例(3.0%).124例(75.2%)合并其他疾病,其中113例(91.1%)合并自身免疫病,以系统性红斑狼疮常见(79.6%).合并血栓者121例(73.3%),其中静脉血栓68例(56.2%),以下肢深静脉血栓最常见.仅有狼疮抗凝物(LA)阳性和仅有抗心磷脂抗体(ACL)阳性患者的血栓发生率分别为86.0%和65.5%(P<0.05).61例APTT延长的患者中,LA阳性50例(82.0%),ACL阳性34例(55.7%).结论 根据APS临床表现可分为多种临床亚型.APS合并血栓以静脉血栓多见.LA阳性较ACL阳性的患者血栓发生率高.APTT延长与LA的相关性较强.     

非标准抗磷脂抗体对抗磷脂综合征的临床应用价值

目的 探讨抗磷脂酰胆碱抗体(aPC)、抗磷脂酰甘油抗体(aPG)和抗鞘磷脂抗体(aSM)检测在抗磷脂综合征(APS)的临床应用价值。方法 共纳入98例原发性APS患者(PAPS组),41例继发性APS患者(SAPS组),138例系统性红斑狼疮患者(SLE组)和55例健康体检者(HC组)。采用化学发光免疫试验(CLIA)检测血清抗心磷脂抗体(aCL)、抗β₂糖蛋白I抗体(aβ₂GPI);酶联免疫吸附试验(ELISA)检测aPC、 aPG和aSM。比较分析组间各指标的差异以及其与临床表现的相关性。结果 PAPS组的aPC IgM (1.47 U/mL)、aPG IgM (1.82 U/mL)、aSM IgM (20.97 U/mL)、aPG IgG (1.22 U/mL)、aSM IgG (14.56 U/mL)浓度高于SLE组(0.91、0.67、10.83、0.88、9.54 U/mL),差异有统计学意义(均P<0.05);SAPS组aPC IgM (2.00 U/mL)、aPG IgM (1.59 U/mL)、 aSM IgM (17...     

抗磷脂综合征相关标志物

抗磷脂综合征是一类以血栓栓塞相关事件及病态妊娠为临床表现合并抗磷脂抗体阳性的自身免疫性疾病.传统诊断标准中的抗体主要有狼疮抗凝物、抗心磷脂抗体及β2糖蛋白Ⅰ抗体,但实际临床上仍然见到少数高度怀疑抗磷脂综合征的患者,这三种抗体均阴性.现有研究表明,与磷脂成分相关的其他多种抗体亦可见于抗磷脂综合征患者中,并且具有一定的临床...     

针对不同靶抗原的抗磷脂抗体与系统性红斑狼疮合并肺动脉高压相关性分析

目的：研究针对不同靶抗原的抗磷脂抗体与系统性红斑狼疮（SLE）合并肺动脉高压（PAH）的相关性。方法：收集72例SLE合并PAH（SLE-PAH）患者血清,固相酶联免疫吸附试验（ELISA）法检测血清抗心磷脂（ACL）抗体、抗β2糖蛋白Ⅰ抗体（抗β2-GPI）、抗凝血酶抗体、抗纤溶酶抗体、抗活化蛋白C抗体（抗APC）和抗组织型纤溶酶原激活物抗体（抗t-PA）,以128例肺动脉压力正常SLE（SLE-non-PAH）患者血清作为对照。结果：与SLE-non-PAH组相比,IgG型ACL抗体、抗β2-GPI抗体、抗纤溶酶抗体和抗凝血酶抗体阳性率在SLE-PAH组较高,两组间差异有统计学意义;抗APC抗体和抗t-PA抗体的检出率两组差异无统计学意义;重度SLE-PAH患者[肺动脉收缩压（PASP）≥60mmHg]的IgG型抗纤溶酶抗体阳性率较轻中度SLE-PAH患者（PASP〈60mmHg）高,两组间差异有统计学意义（P〈0.01）;SLE-PAH组和SLE-non-PAH组相比,各抗体IgM型差异无统计学意义。结论：IgG型抗心磷脂抗体、抗β2-GPI抗体、抗纤溶酶抗体和抗凝血酶抗体与SLE患者发生肺动脉高压相关。     

狼疮肾炎肾小球微血栓形成及其与抗凝血相关因子抗体抗磷脂抗体的相关性研究

目的 评估肾小球微血栓(GMT)在狼疮肾炎(LN)中的发生率,并探讨针对某些凝血相关因子的抗体和抗磷脂抗体在LN患者GMT形成中的临床意义.方法 连续收集124例LN患者肾活检组织标本和血浆,观察组织标本中GMT的发生率.并分成LN-GMT组和LN-non-GMT组;比较两组患者的疾病活动度、相关实验室检查指标和肾组织活动,慢性指数;测定患者的狼疮抗凝物(LA)、抗心磷脂抗体(ACL)、抗β2糖蛋白I(抗β2GP I)抗体、抗凝血酶抗体、抗纤溶酶抗体、抗组织型纤溶酶原激活物(t-PA)抗体和抗膜联蛋白AⅡ(Annexin A II)抗体.结果 GMT在LN中的发生率约为20.2%;LN-GMT组系统性红斑狼疮疾病活动指数(SLEDAI)、肾组织病变活动指数、肾组织病变慢性指数、尿蛋白定量(24 h)、血清肌酐、血清尿素氮的水平和高血压的发生率都较LN-non-GMT组高(P＜0.01);LN-GMT组LA、IgG型抗B2GP I抗体和抗凝血酶抗体阳性率均显著高于LN-non-GMT组(P＜0.05);两组IgG型ACL抗体、抗纤溶酶抗体、抗t-PA抗体和抗Annexin A II抗体阳性率差异均无统计学意义(P＞0.05);两组各抗体IgM型差异无统计学意义(P＞0.05).结论 LN中伴有GMT形成的患者肾脏病变重于无GMT者;LA、IgG型抗β2GP I抗体和抗凝血酶抗体与LN患者GMT形成相关.     

抗心磷脂抗体与系统性红斑狼疮高血压的相关性

目的 探讨抗心磷脂抗体在系统性红斑狼疮(SLE)高血压患者中的临床意义.方法 应用酶联免疫吸附法(ELISA)检测110例SLE患者和50名健康者血清中抗心磷脂抗体IgG(IgG-ACA)、抗心磷脂抗体IgM(IgM-ACA)、抗β2糖蛋白I(β2-GP1)抗体的浓度水平.结果 SLE高血压组IgG-ACA、IgM-ACA、抗β2-GP1抗体浓度水平高于SLE正常血压组、健康对照组(P＜0.05),而SLE正常血压组与健康对照组比较差异无统计学意义(P＞0.05).结论 SLE患者合并高血压时IgG-ACA、IgM-ACA、抗β2-GP1抗体浓度水平升高,提示ACA升高与狼疮性高血压的发生有关,抗心磷脂抗体检测对预测狼疮性高血压有一定参考价值.     

抗磷脂综合征合并自身免疫性溶血性贫血37例临床特征及危险因素分析

目的探讨抗磷脂综合征(APS)合并自身免疫性溶血性贫血(AIHA)的临床特点及危险因素。方法回顾性分析。选2017年5月至2021年5月北京协和医院风湿免疫科连续收治的315例APS患者, 分析其临床特征, 包括一般人口学资料、临床表型(起病首发症状, 起病至确诊APS的时间, 是否合并系统性红斑狼疮(SLE)、血栓事件、病理妊娠事件, 是否合并APS标准外表现)、实验室检查结果[血常规、抗磷脂抗体(aPLs)、血脂、同型半胱氨酸、抗核抗体谱、免疫球蛋白水平、补体水平]。对临床特征及危险因素进行单因素及多因素logistic回归分析。结果 315例APS患者中合并AIHA者37例(11.7%), 未合并AIHA者278例, 均以AIHA为首发表现或同时发病, 其发病至确诊APS的中位时间12个月;APS合并AIHA者比未合并AIHA者出现SLE的比例高[62.2%(23/37)比 19.4%(54/278), P<0.001];两者出现血栓及病理妊娠的比例差异无统计学意义(P>0.05)。合并AIHA者出现血小板减少的风险显著升高(OR=6.19, 95%CI 2.81～1...     

抗β2-糖蛋白Ⅰ抗体在系统性红斑狼疮中的临床意义

目的了解抗!2-糖蛋白Ⅰ抗体(抗!2-GPⅠ抗体)与系统性红斑狼疮(SLE)临床特点的关系及在其诊断中的价值。方法采用酶联免疫吸附试验(ELISA)检测112例SLE患者、40例类风湿关节炎(RA)患者、30例干燥综合征(SS)患者和40名正常人血清中的抗!2-GPⅠ抗体水平。同时,测定患者血清中的抗心磷脂抗体(ACL)等指标,并分析其与患者的临床特点(如:血栓、流产)的关系及其临床意义。统计学分析采用t检验、!2检验和Spearman检验。结果抗!2-GPⅠ抗体在SLE中敏感性为21.4%(24/112),特异性为88.6%,在RA和SS敏感性分别为15.0%(6/40)和6.7%(2/30),40名正常对照均为阴性。抗!2-GPⅠ抗体与血栓形成密切相关(P<0.01),与ACL-IgG和IgM型水平呈正相关(r=0.479,P=-0.032;r=0.400,P=0.045)。抗!2-GPⅠ抗体与其他临床及实验室指标无明显相关性。结论抗!2-GPⅠ抗体在SLE中具有一定的敏感性,且可能在SLE的血栓形成中发挥作用,联合检测ACL与抗!2-GPⅠ抗体能够辅助诊断SLE伴血栓形成。     

Anti-annexin II antibody is associated with thrombosis and/or pregnancy morbidity in antiphospholipid syndrome and systemic lupus erythematosus with thrombosis

This study investigated the prevalence of antibody against annexin II (A2) in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). It also explored the role of this antibody in thrombosis. Purified recombinant A2 was used in ELISA to measure the levels of IgG anti-A2 antibody in 101 APS patients, 41 SLE patients with thrombosis, 124 SLE patients without thrombosis, and 120 healthy controls. The positive rates of IgG anti-A2 antibody in APS patients and SLE patients with thrombosis were 21.8 and 26.8%, respectively. Both values are significantly higher than that in SLE patients without thrombosis (6.5%). IgG anti-A2 antibody is associated with thrombosis and/or pregnancy morbidity (P?相似文献   

Annexin A5 polymorphism (-1C-->T) and the presence of anti-annexin A5 antibodies in the antiphospholipid syndrome

BACKGROUND: Annexin A5 is thought to have a role in the pathophysiology of the antiphospholipid syndrome (APS)-a syndrome characterised by recurrent thrombosis and pregnancy morbidity. OBJECTIVE: To investigate whether anti-annexin A5 immunoglobulin (Ig)M or IgG antibodies, or the -1C-->T polymorphism of annexin A5, is a risk factor for thrombosis or miscarriage, and whether the -1C-->T polymorphism is correlated with APS. METHODS: A cohort study was carried out with a population of 198 patients with primary APS, systemic lupus erythematosus or lupus-like disease. For the detection of anti-annexin A5 antibodies and the measurement of annexin A5 plasma levels, ELISA-type methods were used. The annexin A5 -1C-->T mutation was detected by restriction fragment length polymorphism. RESULTS: 71 patients were positive for annexin A5 IgM or IgG antibodies, of whom 53 patients were positive for anti-annexin A5 IgG antibodies and 27 of 198 patients were positive for anti-annexin A5 IgM antibodies. The prevalence of IgM or IgG anti-annexin A5 antibodies was not significantly associated with thrombosis or miscarriage on multivariate analysis. The prevalence of the -1C-->T mutation in the annexin A5 gene (46/198 patients) was significantly associated with miscarriage (odds ratio 2.7, 95% confidence interval 1.1 to 6.7, independent risk factor). CONCLUSION: The detection of anti-annexin A5 antibodies does not seem relevant for estimating the risk for thrombosis or miscarriage in APS. The -1C-->T mutation was an independent risk factor for miscarriage, which is independent of APS.     

High prevalence of anti-prothrombin antibody in patients with deep vein thrombosis

The present study was designed to determine the prevalence of lupus anticoagulant (LA) antibody and several antibodies for antiphospholipid syndrome (APS) in patients with deep vein thrombosis (DVT)/pulmonary embolism (PE) (n = 48), cerebral thrombosis (CT, n = 30), systemic lupus erythematosus (SLE, n = 22), and idiopathic thrombocytopenic purpura (ITP, n = 30). The presence of antibodies was examined by using the respective ELISA kits. LA was positive in 38.6% of patients with DVT/PE, suggesting that LA is one of the most important risk factors in DVT/PE. The highest prevalence of anti-beta(2) glycoprotein I (beta(2)GPI) IgG was in CT and SLE, followed by DVT, and none in ITP and healthy volunteers (control, n = 40), suggesting that it is related to thrombosis, particularly arterial thrombosis. The highest prevalence of anti-prothrombin (aPT) IgG antibody was in DVT, followed by CT and SLE, and none in ITP and the control, suggesting that it is related to thrombosis, especially venous thrombosis. The highest prevalence of antiphospholipid (aPL) IgG was in DVT, CT, and SLE, but 0% in ITP and control. On the other hand, aPL IgM, anti-annexin V IgG, and anti-annexin V IgM were positive in patients both with and without thrombosis, suggesting that they are not related to thrombosis. Our results indicated that among the anti-phospholipid antibodies, LA is the most sensitive marker for APS while anti-beta(2)GPI IgG, aPT IgG, and aPL IgG are risk factors for thrombosis. In particular, aPT IgG is a significant marker for DVT/PE.     

Prevalence of antibodies to beta2-glycoprotein I in systemic lupus erythematosus and their association with antiphospholipid antibody syndrome criteria: a single center study and literature review

OBJECTIVE: To determine the prevalence of anti-beta2-glycoprotein I antibodies (anti-beta2-GPI) in patients with systemic lupus erythematosus (SLE), and to assess their association with and predictive value for the clinical classification criteria of the antiphospholipid antibody syndrome (APS). METHODS: One hundred thirty-three consecutive patients with SLE were recruited from 2 lupus clinics in the University of Toronto. Serum and plasma samples were tested for IgG anticardiolipin antibodies (aCL), prolonged partial thromboplastin time (PTT), a panel of lupus anticoagulant (LAC) assays, and anti-beta2-GPI (IgG, IgM, IgA). Normal ranges for the assays were established using 129 healthy controls. A literature review from 1992 to 2000 was performed using beta2-GPI, SLE, APS, thrombosis, and recurrent pregnancy loss as key search words. RESULTS: The distribution of anti-beta2-GPI antibodies (of any isotype) in each group were as follows: all patients with SLE, 36.8%; SLE with clinical features of APS, 40.4%; SLE without clinical features of APS, 34.9%; and healthy controls, 3%. The positive predictive values of prolonged PTT, IgG aCL, and anti-beta2-GPI for at least one clinical feature of APS in SLE were 59.3, 50.0, and 38.8%, respectively. There were 27 patients with SLE who had antibodies to beta2-GPI but a normal PTT and negative aCL and LAC. Six (20.7%) of these had a history of thrombosis and/or recurrent pregnancy loss. Twelve studies (including ours) were identified in which patient groups were similar and the same antibody isotype was measured. No agreement was apparent after reviewing the literature regarding an association of anti-beta2-GPI IgG and clinical features of APS in patients with SLE. CONCLUSION: Antibodies to beta2-GPI were frequently seen (35%) in our SLE population. The prevalence of anti-beta2-GPI was similar in those with (19/47) and without (39/86) APS. Anti-beta2-GPI did, however, identify 6 patients with clinical features of APS who were negative for aCL and prolonged PTT. Our results indicate that anti-beta2-GPI may provide additional information for the diagnosis of APS in SLE, but do not supercede other established assays. However, when we attempted to place our results in the context of other reports, the literature review revealed that secondary diagnoses of patient groups and assay techniques are too variable among different investigators to allow useful comparison. Thus, no conclusions could be drawn regarding anti-beta3-GPI and clinical features of secondary APS in SLE.     

Clinical significance of anti-annexin V antibodies in patients with systemic lupus erythematosus

Annexin V has a calcium-dependent binding affinity for anionic phospholipids and activated platelets, and prevents prothrombinase activity. We investigated the clinical significance of IgG anti-annexin V antibodies in patients with SLE. The study population consisted of 140 patients with SLE. Sera were examined for IgG anti-annexin V antibodies by ELISA. IgG anti-annexin V antibodies were detected in 27 of 140 patients (19%). Significantly higher incidences of arterial or venous thrombosis, intrauterine fetal loss, and prolonged activated partial thromboplastin time were found in patients with anti-annexin V antibodies than in those without anti-annexin V antibodies. Three patients with thrombosis were found not to have anticardiolipin antibodies, but to show sustained serological reactions for anti-annexin V antibodies, irrespective of prednisolone administration. These results indicated the clinical characteristics of SLE patients with anti-annexin V antibodies, and that these antibodies may be associated with the pathogenesis of thrombotic events. Am. J. Hematol. 54:209–213, 1997 © Wiley-Liss, Inc.     

Antiphospholipid antibodies (aPL) in systemic lupus erythematosus. Are they specific tools for the diagnosis of aPL syndrome?

OBJECTIVE--Antiphospholipid antibody (aPL) specificity for aPL-related events was evaluated in systemic lupus erythematosus (SLE). METHODS--A study was carried out on 105 patients affected with SLE comparing the prevalence of lupus anticoagulant (LA) and IgG and IgM anticardiolipin antibodies (aCL) between patients with and without features of antiphospholipid syndrome (APS). Antiphospholipid antibody profile was subsequently evaluated in the aPL positive patients with and without aPL-related events, thus excluding the patients with complications of APS possibly due to factors other than aPL. RESULTS--LA showed a strong association with thrombosis and livedo reticularis, and IgG aCL with thrombosis and neurological disorders, while no clinical features were associated with IgM aCL. A considerable number of aPL positive patients with no aPL-related manifestations was also observed, suggesting the low specificity of aPL assays (54.4%). When studying the 60 aPL positive patients, LA was specific (91.3%) for the diagnosis of aPL-related thrombosis, whereas aCL were not specific, although IgG aCL mean levels were higher in patients with arterial thrombosis than in those without APS features. CONCLUSIONS--LA but not aCL positivity is a specific tool for the diagnosis of thrombotic complications due to aPL in SLE.     

Specificity and sensitivity of anti-beta2-glycoprotein I as compared with anticardiolipin antibody and lupus anticoagulant in Thai systemic lupus erythematosus patients with clinical features of antiphospholipid syndrome

Antibodies to beta(2)-glycoprotein I (anti-beta(2)-GPI) have been reported to have stronger association with clinical antiphospholipid syndrome (APS) than anticardiolipin antibodies (aCL) and lupus anticoagulant (LAC). We investigated the sensitivity and specificity of ELISA for anti-beta(2)-GPI in Thai systemic lupus erythematosus (SLE) patients with clinical features of APS and compared the results with IgG/IgM aCL and LAC to find the test with the best association. The hospital records of 151 Thai SLE patients whose sera had been sent for either IgG/IgM anticardiolipin antibodies or lupus anticoagulant testing were reviewed. Sera of patients either without complete clinical records or those with APS-related manifestations other than vascular thrombosis and pregnancy morbidity (according to the international consensus statement on preliminary classification criteria for definite APS) were excluded. For the remaining subjects (112 patients), their sera were tested for anti-beta(2)-GPI antibody, IgG and IgM anticardiolipin, and lupus anticoagulant. The sensitivity and specificity of each method were compared by using the chi-square test. Among the 112 (74.2%) SLE patients in the study, 35 (31.3%) presented with preliminary clinical criteria for APS (i.e., vascular thrombosis and pregnancy morbidity) whereas 77 (68.7%) did not. The sensitivity and specificity of anti-beta(2)-GPI determination were 57.1 and 79.2%, respectively, whereas those of IgG aCL were 25.7 and 94.8%, of IgM aCL were 5.7 and 98.7%, and of LAC were 44.8 and 77.3%, respectively. The accuracy of the four tests showed similar association with clinical APS (accuracy of test = 72.3, 73.2, 69.6, and 68.3%, respectively). Concerning the sensitivity, specificity, and difficulty of the methods, the combination of anti-beta(2)-GPI and IgG aCL tests was the best for the diagnosis of APS in Thai SLE patients.     

High avidity anti-beta 2-glycoprotein I antibodies in patients with antiphospholipid syndrome

OBJECTIVE: To evaluate avidity of IgG anti-beta 2-glycoprotein I antibodies (anti-beta2-GPI) in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) in relation to thrombosis, and to demonstrate a possible affinity maturation of IgG anti-beta 2-GPI during the disease course. METHODS: 64 sera from 32 patients (18 with primary or secondary APS, 14 with SLE without APS) and their respective IgG fractions or affinity purified anti-beta 2-GPI were studied by anticardiolipin (aCL) and anti-beta 2-GPI enzyme linked immunosorbent assay and by chaotropic assay. RESULTS: Six, 12, and 14 patients had high, low, and heterogeneous avidity IgG anti-beta 2-GPI, respectively. In 12 patients an increase in antibody avidity was observed over a period of between four and 12 years. More patients with APS were in the high avidity than in the low avidity anti-beta 2-GPI group, while the opposite was observed for SLE alone (both p<0.05). The most common clinical feature among patients with high avidity anti-beta 2-GPI was thrombosis, mainly venous thrombosis (p<0.05 and p<0.02, respectively, v the low avidity anti-beta 2-GPI group). CONCLUSIONS: Patients with APS with or without SLE may have anti-beta2-GPI of high, low, or heterogeneous avidity. High avidity anti-beta 2-GPI appear to be associated with thrombosis and APS, while in pure SLE low avidity anti-beta 2-GPI may prevail. Monitoring of avidity may help elucidate the role of anti-beta 2-GPI affinity maturation in the pathogenesis of APS.     

The IgG and IgM isotypes of anti-annexin A5 antibodies: relevance for primary antiphospholipid syndrome

Antiphospholipid syndrome (APS) is an autoimmune disease that is characterized by the presence of thromboses and/or recurrent pregnancy losses (RPL). The persistent presence of antiphospholipid antibodies (aPL Abs): IgG and/or IgM isotypes of the anticardiolipin and/or anti-β2 glycoprotein I antibodies and lupus anticoagulant is mandatory for the laboratory diagnosis of APS. Due to the heating debate on the relevance of the IgM isotype of aPL Abs as a laboratory criterion defining APS, the focus of this article was to analyze whether both the IgG and IgM isotype of anti-annexin A5 Abs have equal relevance for clinical and serological features of patients with primary APS (PAPS). The IgG isotype of anti-annexin A5 Abs is associated with RPL in PAPS patients, although it is not elucidated whether these Abs are the cause or the consequence of RPL in PAPS. No data that could substantiate the association of the IgG and/or the IgM isotypes of anti-annexin A5 Abs with the presence of arterial and/or venous thromboses and/or their main complications in PAPS is available so far. However, the presence of clinical manifestations of the PAPS is increasing with the multiple positivity for aPL Abs and the IgM isotype of anti-annexin A5 Abs. The importance of the IgM isotype of anti-annexin A5 Abs in PAPS needs further elucidation due to the facts that majority of the available articles did not differentiate between both isotypes or only investigated the IgG isotype of anti-annexin A5 Abs.     

Anticardiolipin Antibodies in Juvenile Rheumatoid Arthritis and Systemic Lupus Erythematosus

Background: Antiphospholipid antibody syndrome (APS) can either occur as a primary syndrome or associated with other autoimmune diseases such as systemic lupus erythematosus (SLE). Anticardiolipin antibody (aCL) of IgG and/or IgM isotype in blood, measured by a standardized ELISA is the most acceptable laboratory criteria. APS IgG isotype, particularly IgG2 subclass is more strongly associated with thrombosis. Objectives: This study was done to determine the prevalence of IgG aCL and its subclasses in relation to APS symptoms, in a group of juvenile rheumatoid arthritis (JRA) and juvenile systemic lupus erythematosus (SLE) patients.   Methods: In this prospective study, 28 JRA and 16 SLE patients, aged 3-18 years, were enrolled. IgG aCL was assayed by standard aCL ELISA. IgG subclasses were also assayed by ELISA on sera with medium to high titers of aCL. ACL assay was performed on at least two occasions for each patient, over 3-6 months period of follow up.   Results: 29% (8/28) of JRA patients and 44% (7/16) of SLE patients had aCL. Six of SLE patients displayed APS related manifestations: hemolytic anemia, thrombocytopenia, arterial occlusion, valvular heart disease, livedo reticularis and pulmonary hypertension, but none of them had persistant medium or high titer of aCL. The lack of association of high titer of aCL with APS related symptoms was observed in two patients. The IgG subclasses were primarily IgG1 and IgG3.   Conclusion: The prevalence of IgG aCL in this group of pediatric SLE and JRA is not uncommon but it’s relation to clinical manifestations is not clear. IgG1 and IgG3 subclasses were not associated with thrombosis, which is in agreement with previous studies.