Anticardiolipin Antibody Isotype Determination in Amniotic Fluid of Pregnant Patients with Systemic Lupus Erythematosus and/or Antiphospholipid Syndrome

Objective: To determine the levels and isotypes of aCl, as well as anti beta 2 glycoprotein 1 (antiβ2-GP1) antibodies in serum and amniotic fluid of pregnant patients with SLE and/or APLS, and healthy pregnant women serving as a control group. Material and Methods: We analyzed serum and amniotic fluid of pregnant patients with SLE and/or APLS, and of healthy pregnant women through ELISA. Results were compared using a Student’s T test. Results: 6 of 13 patients (46.1%), 5 with SLE and one with primary APLS had antiphospholipid antibodies in amniotic fluid. Two patients had IgG aCl and 4 patients had antiβ2-GP1 (one of them also showing IgM) in amniotic fluid. In serum, 4 patients (30%) had antiphospholipid antibodies present (one IgG aCl and three anti β2-GP1) as opposed to none in the control group having antiphospholipid antibodies in amniotic fluid. Only one control had IgM aCl in serum. Antiβ2-GP1 in the amniotic fluid of patients showed a statistically significant value when compared to controls. Conclusion: aCl and antiβ2-GP1 may be present in the amniotic fluid of patients with and without a history of fetal loss. The presence of IgM aCl and antiβ2-GP1 in amniotic fluid suggests its localized production.

Bilateral Choroidal Occlusion in Antiphospholipid Syndrome Associated with Systemic Lupus Erythematosus

This article reports a rare case of bilateral choroidal occlusion that occurred in a 24-year-old woman with antiphospholipid syndrome (APS) associated with systemic lupus erythematosus (SLE). This young lady concurred with aorta ventralis thrombosis and bilateral iliac artery occlusion when presented, and experienced a rapid deterioration of vision. She also has a history of recurrent miscarriage. Corticosteroid,immunosuppression and anticoagulation therapy were administered. Patients with APS associated with SLE are at risk for thrombotic phenomena, which may affect the ocular vessels of all sizes, including choroidal vessel.Our case alerts ophthalmologists and rheumatologists that bilateral choroidal occlusion may indeed be developed in patients with APS associated with SLE, and is a potential cause of visual morbidity.

Thrombotic microangiopathy involving the gallbladder as an unusual manifestation of systemic lupus erythematosus and antiphospholipid syndrome: Case report and review of the literature

Gallbladder disease is no more common in patients with systemic lupus erythematosus (SLE) than in the general population. We describe a 17-year-old patient with SLE, who developed nephritis that was well controlled with medications. Initial treatment consisted of azathioprine, aspirin and prednisone with stable control of her symptoms. Two years later she developed a right quadrant abdominal pain, and an abdominal ultrasound revealed microlithiasic cholecystitis. Open cholecystectomy was performed and the histopathological findings revealed vasculitis with thrombotic microangiopathy in the gallbladder. This case presentation illustrates that calculous or acalculous cholecystitis should be considered as a manifestation of active SLE and APS.

Systemic Lupus Erythematosus and Antiphospholipid Syndrome Related Retinal Vasculitis Mimicking Ocular Cysticercosis: a Case Report

MAKING accurate and timely diagnosis is often challenging when patients with a systemic disease first present with ocular manifestations.The possibility that vasculitis associated with systemic lupus erythematosus(SLE)and antiphospholipid syndrome(APS)can be misdiagnosed as cysticercosis has not been discussed in the literatures.

The Value and the Variability of Magnetic Resonance Imaging in Patients with Systemic Lupus Erythematosus and Antiphospholipid Syndrome

Objectives: The objectives of this article are twofold: To review the value and the variability of MR imaging (MRI) findings in patients with systemic lupus erythematosus (SLE) and to reveal the frequency of central nervous system (CNS) involvement. SLE is a complex multisystem autoimmune connective tissue disorder with a broad spectrum of clinical presentations. Materials and methods: We present three cases, two with neurological symptoms and one case with musculoskeletal symptoms. Patients were imaged in the MRI department of the University Hospital of Patras. All patients had a previous history of antiphospholipid syndrome and known SLE. Results: None of the pts had a normal brain MRI. Abnormal lesions were typically high on FLAIR and T2-weighted images. One patient showed myositis of the tibial muscles bilateral and although she did not reveal any neurological symptoms, because of the presence of APS, she underwent brain MRI to detect any cerebrovascular involvement. The brain MRI was abnormal and showed extensive lesions and porencephalia. In all cases the differential diagnosis of the brain MRI included vasculitis, focal ischemia, multiple sclerosis or other entities. Parenchymal volume loss-cerebral atrophy, incompatible with their age, was obvious in all patients. Conclusion: Magnetic resonance imaging is the gold standard for the investigation of central nervous system in patients suffering from lupus. Lupus patients who also develop antiphospholipid syndrome must be submitted to brain MRI because central nervous involvement is very common and serious, although symptoms may be silent as in our case.

Detection of Anticardiolipin Antibody in Systemic Lupus Erythematosus and Its Clinical Significance

Objective To determine the frequency and the clinical significance of anticardiolipin antibodies (ACA) in patients with systemic lupus erythematosus( SLE).Methods The serum ACA in 30 patients with SLE and 30 sera from healthy volunteers was detected by the enzyme linked immunosorbend assay (ELISA).Results In normal group the binding index(BI) oflgG, IgM and IgA type of ACA was 1.48 ?

Systemic lupus erythematosus combined with primary hyperfibrinolysis and protein C and protein S deficiency:A case report

BACKGROUND Systemic lupus erythematosus(SLE)is an autoimmune disease characterized by systemic involvement and multiple autoantibodies in the serum.Patients with protein C(PC)and protein S(PS)deficiency are prone to thrombosis.In contrast,patients with primary hyperfibrino-lysis tend to bleed.CASE SUMMARY A 52-year-old female patient with bilateral pleural effusion was diagnosed with"tuberculous pleurisy"and treated with anti-tuberculosis drugs and prednisone.The coagulation-related laboratory results showed decreased fibrinogen,PC activity,PS activity,and antithrombinШactivity.The immune-related laboratory results showed positive antinuclear antibody,anti-Smith antibody,anticardiolipin antibody(ACL),anti-β2-glycoprotein I antibody(aβ2GPI)and direct Coomb’s test and decreased complement 3 and complement 4.Thoracoscopy was performed and bloody pleural fluid was drained.Pathology of the pleural biopsy showed lymphocytes,plasma cells,and a few eosinophils in adipose and fibrous connective tissue.Results of whole exome sequencing of blood showed no genetic mutations suggesting the presence of hereditary hematological diseases.The patient was finally diagnosed with SLE and primary hyperfibrinolysis,and was treated with prednisolone,hydroxychloroquine,and compound cyclophosphamide.CONCLUSION PC and PS deficiency in SLE might be related to ACL and aβ2GPI.SLE and primary hyperfibrinolysis can coexist in one patient,with both a risk of thrombosis and a risk of bleeding.

抗磷脂综合征误诊为神经精神性狼疮1例并文献复习

抗磷脂综合征和神经精神性狼疮在中枢神经系统受累时,临床症状及辅助检查相似,但诊疗策略不同,容易误诊误治。本文分析误诊原因并文献复习。回顾经中国人民解放军联勤保障部队第九六二医院收治的1例以血小板减少、脑卒中为首发症状的病例,初诊为“神经精神性狼疮”,给予羟氯喹、糖皮质激素及吗替麦考酚酯诱导缓解,并给予阿司匹林抗血小板聚集。后患者出现磷脂抗体谱阳性及静脉血栓形成,修诊为“抗磷脂综合征”,给予羟氯喹及小剂量糖皮质激素维持治疗,并给予低分子肝素抗凝-吲哚布芬抗血小板聚集序贯治疗,随访5个月患者病情稳定未复发。抗磷脂综合征与神经精神性狼疮在首发症状及血栓事件的判断是早期诊断的关键。吲哚布芬作为阿司匹林的替代方案在抗磷脂综合征的远期疗效值得进一步探讨。

Myocardial infarction in a patient with systemic lupus erythematosus and antiphospholipid syndrome

This case report we presented aims to report a-31-year-old man with systemic lupus erythematosus （SLE） and antiphospholipid syndrome （APS） who developed myocardial infarction （MI） and also aims to discuss the possible mechanisms. The results showed that traditional risk factors alone do not cause coronary heart disease with SLE, and SLE-related factors influence the atherogenic process. We found that although SLE patients with acute MI benefit from percutaneous coronary intervention （PCI） therapy, it is very important to choose the reasonable antithrombotic strategies in patients with SLE and APS undergoing PCI who require oral anticoagulant therapy.

以双下肢无力为首发表现的系统性红斑狼疮伴多系统受累1例

系统性红斑狼疮(SLE)是一种多系统受累、临床表现多样的自身免疫性疾病。多以器官受累后的症状为首发表现就诊。目前,以双下肢无力为首发表现的SLE伴多系统受累的案例较少。本文报道1例20岁女性以双下肢无力为首发表现的SLE患者,伴肌肉受损、血液系统受累、抗磷脂综合征(APS)和肝脏受累。以便为这类疾病的临床诊断提供参考。

胱抑素C及抗磷脂抗体与系统性红斑狼疮肾损伤病理类型的相关性

目的 探讨胱抑素C(CysC)、抗磷脂抗体及其他指标与系统性红斑狼疮(SLE)肾损伤病理类型的相关性,进一步研究SLE发生肾损伤的危险因素,为临床诊治提供依据。方法 选取75例单纯SLE患者和55例狼疮性肾炎(LN)患者,及同期到医院体检的健康对照者52例。行穿刺活检术的31例LN患者再分为LN-III/IV组19例和LN-II/V组12例。收集一般资料(D-二聚体、PLR、C3、C4、IgM、IgG、ESR、抗ds DNA抗体、Cysc、抗磷脂抗体、抗β2糖蛋白1抗体及肾脏穿刺病理资料)。比较Cysc等SLE疾病相关指标及抗磷脂抗体在三组之间的差异,探讨抗磷脂抗体与SLE肾损伤病理类型的相关性。结果 SLE组和LN组Cysc、IgG、PLR、D-二聚体、ESR、CRP水平高于健康对照组,C3、IgM、C4水平低于健康对照组,LN组Cysc水平高于SLE组,C3、IgG、IgM水平低于SLE组(P<0.05)。LN患者中抗β2糖蛋白1抗体、抗磷脂抗体的阳性率明显高于SLE患者(P<0.05)。LN-Ⅲ型/Ⅳ型组抗磷脂抗体(ACA)的阳性率较LN-Ⅱ/Ⅴ型组的显著升高(P=0.043)。CysC、C3、IgM、IgG是SLE发生肾损伤的危险因素。结论 抗磷脂抗体阳性可能提示肾损伤病理类型有更高的活动性,CysC、C3、IgM、IgG水平是SLE发生肾损伤的危险因素。

抗磷脂抗体在小鼠激素性股骨头坏死中的作用

目的探讨抗磷脂抗体在C3H/HeN小鼠激素性股骨头坏死中的作用。方法纯化抗磷脂抗体综合征(APS)患者的血清抗β2GPI抗体,建立实验性APS C3H/HeN小鼠动物模型。随机分为对照组、对照+激素组、模型组及模型+激素组,每组10只。于第8周末处死所有小鼠,对血标本及股骨头病理组织学标本进行分析。结果至实验结束(第8周末),对照组、对照+激素组、模型组及模型+激素组各有9、7、8、8只小鼠存活,APTT指标模型+激素组、模型组、对照+激素组及对照组依次升高(P<0.05),NO指标模型+激素组、对照+激素组、模型组及对照组依次降低(P<0.05);股骨头坏死发生率及空骨陷窝率比较,对照组、模型组、对照+激素组、模型+激素组依次升高,骨小梁面积占比对照组、模型组、对照+激素组、模型+激素组依次下降(P<0.01)。结论抗磷脂抗体可能通过加剧血管内皮损伤及高凝状态在激素性股骨头坏死中起作用。

A successful birth of severe secondary recurrent miscarriage case after a decline of phosphatidylserine-dependent anti-prothrombin antibody by intravenous immunoglobulin administration

A 33 years old woman was referred to our hospital since her sixth pregnancy had been revealed. In fact, at 19 years of age she had diagnosed as having systemic lupus erythematosus without organ failure. In addition, she had a past history of uncontrollable severe pregnancy-induced hypertension occurred during the second pregnancy, resulting in extremely premature delivery and following postpartum HELLP syndrome. It was so severe that we employed administration of dexamethasone and plasma exchange to ameliorate a life-threatening situation. In the course of her recovery it was revealed that she had been complicated with antiphospholipid antibodies, and at the same time we observed that phosphatidylserine-dependent anti-prothrombin antibody IgG levels were declining as her condition was getting better. There-after, she became pregnant three times, but all pregnancies ended in miscarriage despite administration of prednisolone and anticoagulant therapy. Therefore, we realized that her recurrent miscarriages could not be prevented with generally acceptable therapies, so we tried intravenous immunoglobulin shortly after fetal heart beats were detected. In fact, her sixth pregnancy was going well, but we had to terminate it at the 35th week of gestation due to the onset of HELLP syndrome-like condition. However, she could achieve an almost intact pregnancy outcome without neonatal complications or persistently worsening postpartum HELLP syndrome-like condition. Considering the etiologic relation overlapping between systemic lupus erythematosus, antiphospholipid syndrome and recurrent miscarriage, intravenous immunoglobulin can be one of the treatment options for severe secondary recurrent miscarriage, although the evidence of the treatment is always certain. In addition, a decline of phosphatidylserine-dependent anti-prothrombin antibody IgG levels we observed in this case may represent its therapeutic immunomodulatory effects.

抗氨基甲酰化蛋白抗体在结缔组织病中的研究进展

结缔组织病(CTD)是一种常见的慢性自身免疫性疾病,常累及多器官、多系统,其诊断不仅需要临床表现的支持,更有赖于特异性自身抗体的存在。抗氨基甲酰化蛋白(anti-CarP)抗体是近年来新发现的自身抗体,在多种CTD的诊断、分层及治疗评估中发挥重要作用。该文就anti-CarP抗体在CTD中的研究进展进行综述。

系统性红斑狼疮合并舞蹈症一例并文献复习

系统性红斑狼疮合并舞蹈症在国内相对罕见,目前尚无统一的诊断标准及特异性辅助检查,为排除性临床诊断。本文报道暨南大学附属第一医院风湿免疫科2022年1月收治的1例系统性红斑狼疮合并舞蹈症患者的临床资料,并结合近10年国内外相关文献进行复习,总结这些病例的临床特点,以提高风湿免疫科医生对该病的认识。

APTT纠正试验辅助诊疗慢阻肺患者持续APTT延长1例

活化部分凝血活酶时间(APTT)是常见的出凝血功能筛选试验,多种原因(如凝血因子缺乏、存在凝血因子抑制物或狼疮抗凝物)均可导致APTT延长,但其治疗方案和结局却完全不同。本文报道1例持续APTT延长的慢性阻塞性肺疾病患者诊疗经过,患者既往诊断慢性支气管炎,因发热伴气喘,活动后加重于2021年12月6日就诊于南阳市中心医院。初诊发现APTT显著延长(达危急值标准),血浆输注和维生素K制剂均无法纠正APTT延长,后选择APTT纠正试验提示疑似抗磷脂综合征,遂糖皮质激素治疗后患者状况明显好转。通过此报道,以期加深临床医师对APTT纠正试验的认识,同时提高该试验在基层检验实验室的应用,为临床医师快速识别患者出血或血栓风险、减少漏诊和误诊提供帮助。

生物制剂联合治疗在抗磷脂综合征中的应用

抗磷脂综合征(antiphospholipid syndrome,APS)是一种自身免疫性疾病,疾病分型多且不易识别,包括标准APS和非标准APS(non-criteria APS,NC-APS),又按是否合并其他疾病分为原发性APS(primary antiphospholipid syndrome,PAPS)和继发性APS(secondary antiphospholipid syndrome,SAPS),其中又以继发于系统性红斑狼疮(systemic lupus erythematosus,SLE)的APS多见。目前预防和治疗APS的指南共识主要讨论了小剂量阿司匹林、维生素K拮抗剂和肝素的使用原则,对近年出现的NC-APS临床表现的管理也主要依赖于低质量的证据,尚未形成高级别指南共识。抗磷脂抗体(antiphospholipid antibodies,aPL)阳性患者合并其他疾病或者在妊娠期、手术、感染等二次打击诱发下,可以导致APS疾病反复且危险性增加。本文主要围绕APS的特征,aPL生成的机制与临床表现,利妥昔单抗(rituximab,RTX)、贝利尤单抗(belimumab,BEL)以及泰它西普(telitacicept,TA)生物制剂治疗相关症状的研究进行综述,并探讨生物制剂联合方案治疗复发难治性APS的新策略。

系统性红斑狼疮合并B型胰岛素抵抗综合征的研究进展

B型胰岛素抵抗综合征(TBIRS)是一种由胰岛素受体抗体(INSRAB)所介导的自身免疫性血糖异常综合征,常伴发于系统性红斑狼疮(SLE),在临床工作中易漏诊、误诊。现本文对SLE合并TBIRS的研究进展进行回顾分析,提高对该疾病的认识。

系统性红斑狼疮患者抗磷脂抗体与低水平补体C3、C4的关系

目的补体活化和低补体血症常见于系统性红斑狼疮（SLE）患者,15%~40%SLE患者抗磷脂抗体（antiphospholipid,a PL）呈阳性。本文探讨SLE患者血清抗磷脂抗体与低水平补体的关系。方法选取我院2013年2月至2015年2月住院及门诊部诊治的SLE患者126例（SLE组）和健康体检者162例（健康对照组）,根据SLE疾病活动指数（systemic lupus erythematosusdisease activity index,SLEDAI）将SLE组又分为SLE轻度活动组、中度活动组和重度活动组（n分别为48、52、26）。所有样本行抗β2糖蛋白1（β2GP1）抗体、抗心磷脂抗体（a CL）、C-反应蛋白、抗ds DNA抗体、补体C3、C4检测。观察a PL抗体与补体C3、C4的相关性。结果与健康对照组相比,SLE组血清CRP水平,各种亚型a PL抗体滴度显著升高,差异均有统计学意义（P均〈0.05）,SLE轻度和中度活动组患者血清C3、C4水平差异均无统计学意义（P均〉0.05）,而SLE重度活动组患者血清补体C3、C4水平显著降低,差异均有统计学意义（P均〈0.05）;SLE轻度、中度、重度活动组三者相比,各类亚型a PL抗体均呈显著升高趋势,差异均有统计学意义（P均〈0.05）;SLE患者血清补体C3、C4水平与各类亚型a PL抗体滴度均呈显著负相关（r=-0.205、-0.213、-0.207、-0.19,P〈0.05;r=-0.935、-0.933、-0.914、-0.929,P〈0.01）;与a PL抗体阴性SLE组相比,a PL阳性SLE患者血清补体C3、C4水平显著降低,差异有统计学意义（P〈0.05）,抗ds DNA抗体阳性率显著高于阴性组（P〈0.05）。结论 a PL抗体与SLE患者疾病活动度相关,且与补体C3、C4水平呈显著负相关,a PL抗体可能激活补体C3、C4共同参与SLE患者的疾病活动。

抗IgG、IgM、IgA型心磷脂抗体和抗β_2糖蛋白1抗体在系统性红斑狼疮患者中的意义

目的探讨抗IgG、IgM、IgA型心磷脂抗体和抗β2糖蛋白1抗体在系统性红斑狼疮(systemic lupus erythematosus,SLE)患者中的意义。方法分别采用化学发光免疫分析法和酶联免疫吸附法检测372例SLE患者、80例其他结缔组织病患者及60例健康体检者血清中抗IgG、IgM、IgA型心磷脂抗体和抗β2糖蛋白1抗体。同时分析这4个指标与SLE及其病情严重程度的相关性。结果在检测的372例SLE患者组中,抗IgG、IgM、IgA型心磷脂抗体和抗β2糖蛋白1抗体的阳性率分别为31.45%、14.52%、10.22%和29.03%,其表达水平分别为(90.39±35.43)U/ml、(41.25±23.16)U/ml、(32.27±15.77)U/ml、(61.42±21.69)U/ml,与SLEDAI积分呈正相关(P<0.05)。在80例疾病对照组中,抗IgG、IgM、IgA型心磷脂抗体和抗β2糖蛋白1抗体的阳性率分别为7.50%、2.50%、0和1.25%。而在60例正常对照组中,4项检测结果均为阴性。抗IgG型心磷脂抗体阳性与血小板减少、白细胞减少、血栓形成、习惯性流产和肾脏病变有关;抗IgM型心磷脂抗体阳性与血小板减少、白细胞减少、血栓形成和肾脏病变有关;抗IgA型心磷脂抗体阳性与血小板减少、血栓形成和肾脏病变有关;抗β_2糖蛋白1抗体阳性与血小板减少、白细胞减少、溶血性贫血、血栓形成、习惯性流产和肾脏病变有关。结论抗IgG、IgM、IgA型心磷脂抗体和抗β_2糖蛋白1抗体在SLE的发病及病情发展中起到了非常重要的作用,定量和联合检测对SLE的诊断、病情严重程度的评价、判断预后及疗效观察等均有重要的临床意义。