系统性红斑狼疮合并抗磷脂综合征患者的临床分析

目的 研究系统性红斑狼疮合并抗磷脂综合征(SLE-APS)患者的临床特点.方法 对39例SLE-APS患者的临床和实验室资料进行回顾性分析.结果 39例患者中,有31例共发生了48次血栓栓塞事件,以深静脉血栓及脑梗死为主.26例已婚有生育史女性患者中,有12例发生病态妊娠.28例抗心磷脂抗体阳性;16例狼疮抗凝物阳性.24例患者先确诊SLE,平均9.5年后又出现APS样表现;12例先出现反复病态妊娠或血栓事件,平均4.8年后演变为SLE;3例一发病便同时符合SLE和APS的分类标准.有5例在发生血栓事件或病态妊娠时的狼疮活动指数<5分.结论 SLE-APS患者血栓事件及病态妊娠发生率增多.APS可出现于SLE之前、之后或同时.狼疮患者可以在病情稳定期出现APS的表现.详细询问病史、常规检测抗心磷脂抗体,有助于发现SLE-APS的高危因素,积极预防APS的发生.     

系统性红斑狼疮继发抗磷脂综合征的中西医诊治进展

抗磷脂综合征(the antiphospholipid syndrome,APS)是以动静脉血栓事件、病理妊娠、血小板减少以及持续存在抗磷脂抗体(antiphospholipid antibodies,aPL)为特征的一种自身免疫性疾病.APS常继发于系统性红斑狼疮(system ic lupus erythematosus,SLE),因SLE继发APS的不良预后越来越引起重视,寻找新的治疗手段已迫在眉睫.虽然目前诸多中医大家证实了活血化瘀、补肾健脾等治疗方法对SLE继发APS的临床疗效,但西医抗凝及抗血小板仍是最有效、最主流的治疗手段,因此中西医结合治疗可能成为另一种极富前景的治疗方法.     

系统性红斑狼疮继发抗磷脂抗体综合征临床分析

目的:探讨系统性红斑狼疮(system ic lupus erythematosus,SLE)继发抗磷脂抗体综合征(antiphospholipid syndrome,APS)的临床特点、实验室指标及相关因素,以早期发现继发性APS。方法:回顾性分析2005年1月至2014年1月收集的129例SLE和APS患者的临床资料,包括临床表现、妊娠情况、抗核抗体、抗心磷脂抗体等。结果:129例患者中,原发性APS(primary antiphospholipid syndrome,PAPS)8例;SLE者121例,其中SLE合并APS患者(SLE-APS)41例,仅诊断为SLE患者(SLE-APA-)40例及出现抗磷脂抗体(antiphospholipid antibody,APA)异常但APS诊断依据不足(SLE-APA+)的SLE患者40例。8例原发性APS患者中,3例男性均表现为下肢深静脉血栓形成及手微动脉血栓形成,有婚育史的女性患者中5例均有病态妊娠表现,自发性流产和(或)死胎,其中2例有肺栓塞、门(脾)静脉栓塞。41例SLE-APS患者中,表现为下肢深静脉血栓有15例,肺栓塞患者3例,脑梗死10例;SLE-APS组抗β2-糖蛋白1(抗β2-GP-1)浓度明显高于SLE-APA+组及SLE-APA-组,而血小板计数明显减低。结论:SLE合并APS患者的血清中存在高浓度抗β2-GP-1,提示高浓度抗β2-GP-1是SLE继发APS的独立危险因素。     

抗磷脂综合征合并疣状心内膜炎误诊为亚急性细菌性心内膜炎1例报告并文献复习

目的：探讨疣状心内膜炎的误诊原因。方法：报告1例抗磷脂综合征合并疣状心内膜炎误诊为亚急性细菌性心内膜炎病例。结果：1例32岁女性患者因持续发热，血小板降低，体检发现心前区收缩期吹风样杂音，超声提示二尖瓣赘生物，CT发现多发性脑梗死误诊亚急性细菌性心内膜炎。结论：抗磷脂综合征多伴有血小板降低、多发性血栓形成及反复流产，可并发Libman—Sacks无菌性疣状心内膜炎。     

系统性红斑狼疮患者抗磷脂综合征与肿瘤坏死因子α和P-选择素的关系

目的探讨系统性红斑狼疮（SLE）患者抗磷脂综合征（APS）的继发与血清肿瘤坏死因子α（TNFα）、P-选择素（P-selectin）水平的关系。方法采用改良Russell蝰蛇毒稀释试验（DRVVT）测定41例SLE患者及25例正常人血浆狼疮抗凝物质（LAC）,酶联免疫吸附试验（ELISA）方法检测血清抗心磷脂-IgG、IgM（ACL-IgG、IgM）抗体,ELISA方法测定SLE患者及正常对照组血清P-selectin、TNFα。结果（1）41例SLE患者中19例血浆中检测到LAC和（或）血清中检测到高滴度ACL-IgG、IgM;（2）与正常对照组（10.58±4.10）ng/L相比,SLE继发APS组、未继发APS组的P-selectin水平均升高,分别为（21.55±6.29）ng/L和（14.02±9.48）ng/L,SLE未继发APS组与继发APS组相比,P-selectin水平差异亦有统计学意义;（3）仅SLE继发APS组（30.01±13.3）pg/ml与正常对照组间（21.60±12.78）pg/mlTNFα的差异有统计学意义,其余各组间差异均无统计学意义;（4）各组内血清P-selectin水平与TNFα水平均无相关性。结论在SLE患者继发APS的病程中,P-selectin起到重要作用,且P-selectin的作用强于TNFα。     

抗磷脂抗体综合征合并中枢神经系统疾病5例报道及文献复习

目的:探讨抗磷脂抗体综合征(APS)中枢神经系统损害的临床表现及诊治特点。方法:回顾性分析APS合并中枢神经系统疾病患者5例的临床资料,结合文献进行分析。结果:5例抗磷脂抗体(aPL)均阳性,合并脑梗死2例,合并舞蹈病2例,合并癫痫1例,合并僵人综合征1例;治疗予以抗血小板、抗凝、抑制炎症、免疫抑制等,患者症状均得到改善。结论:神经系统受累的APS的诊断主要依据相关临床表现及a PL的检测,未有明确病因的神经系统疾病,可行a PL检测,有助于APS的诊治。     

抗磷脂综合征的肺表现及治疗

抗磷脂综合征 (antiphospholipidsyndrome ,APS)的主要临床表现为反复的血管性血栓形成、自发性流产和血小板减少症 ,并伴有抗磷脂抗体 (antiphosphlipidantibody ,APL)包括抗心磷脂抗体 (anticardiolipinantibodies,aCL)及 (或 )血清狼疮抗凝物 (lupusanticoagolate,LAC)中度或强阳性。部分患者还出现网状青斑、心瓣膜赘生物、溶血性贫血、偏头痛、胎儿宫内生长迟缓及妊高征等临床表现。因为血栓性疾患、流产及一过性APL阳性同存并非少见 ,故应证明APL阳性持续存在。本综合征可分为原发性和继发性两种 ,如果无明确原发病的患者可称…     

原发性抗磷脂综合征1例

抗磷脂综合征 ( anti-phospholipid syndrome, APS) 是指由抗 磷脂抗体(anti-phospholipidantibody,APL抗体)引起的一组临床 征象的总称,主要表现为血栓形成、习惯性流产、死胎、血小板减 少以及神经、精神系统损害等。APS是风湿病尤以系统性红斑 狼疮(SLE)患者中常见的临床表现,称之为继发性抗磷脂综合 征,在未明确原发病患者中出现此综合征时称为原发性APS,临 床上较少见,现报告1例。     

系统性红斑狼疮合并重度肺动脉高压及右心衰竭一例报告

几乎所有的弥漫性结缔组织病均有合并肺动脉高压（PAH）的报道,其中系统性红斑狼疮（SLE）的患者出现PAH的概率为2．9％,而合并抗磷脂综合征（APS）的SLE患者出现PAH的概率则升至7．3％;SLE继发PAH的5年和10年生存率分别为86％和68％,而无PAH者分别为90％和83％.SLE合并重度PAH,病情凶险,死亡率高。     

抗磷脂综合征

近年来,一些学者发现系统性红斑狼疮(SLE)及某些自身免疫性疾病患者血清中存在抗磷脂抗体(APLA),包括狼疮抗凝物质(LA)和抗心磷脂(ACL).随着研究的深入,对APLA的临床意义获得了较多的认识,发现静脉和动脉血栓、神经精神症状、血小板减少,反复流产等临床表现与APLA密切相关,并提出血清中具有APLA的患者伴静脉和/或动脉血栓、血小板减少等表现者称抗磷脂综合征(APS).本文复习有关文献,对APS作一综述.一、APLA的生物学特性和发生率     

Combination of Libman-Sacks endocarditis with infectious endocarditis

Out of 242 patients treated for systemic lupus erythematosus (SLE) in Novosibirsk for 15 years, valvular lesions and endocarditis were diagnosed in 41(16.9%) patients. Combination of Libman-Sax endocarditis (LSE) with infectious endocarditis (IE) was observed in three patients (two women, one man, age 18-40 year). SLE ran a subacute course in one woman, an acute one--in the other. LSE emerged early in SLE in two patients. All the patients had polyorganic lupus pathology, lupus nephritis with nephrotic syndrome (morphological class IV). Two patients had mitral valve disease, one patient--mitral-aortic disease. The rise of secondary IE was seen after massive immunosuppressive therapy. The diagnosis of secondary IE was made after SLE duration for 10-36 months. At IE diagnosis, all the patients had high titers of blood antiphospholipid antibodies. IE was of staphylococcal origin in two patients and candidosis-induced in one patient. In SLE with IE there was thromboembolic syndrome. LSE and IE have related aspects which should be regarded in clinical practice: possible "IE mask" in LSE, risk of secondary IE in about 10% of LSE patients, prophylactic measures necessary to prevent IE in hemodynamically prominent forms of LSE.     

Severe mitral stenosis as the first manifestation of systemic lupus erythematosus in a 20-year-old woman: the value of magnetic resonance imaging in the diagnosis of Libman-Sacks endocarditis

We report a case of severe mitral stenosis caused by Libman-Sacks endocarditis, as an initial manifestation of systemic lupus erythematosus (SLE) in a 20-year-old woman. Cardiac magnetic resonance imaging (MRI) demonstrated a thickening of the mitral valve with basal endocardial thickening exhibiting defect on first-pass perfusion short-axis acquisition and delayed enhancement in keeping with extensive fibrous endocarditis. The patient underwent successful mechanical mitral valve replacement. This case illustrates that MRI is useful in diagnosing this recognised but uncommon cardiac complication of SLE and excluding differential diagnosis such as valve tumour and infective endocarditis with perivalvular abscesses.     

血栓性抗磷脂综合征7例回顾性分析

目的探讨血栓性抗磷脂综合征(antiphospholipid syndrome,APS)的临床表现、实验室检验、神经影像检查、诊治。方法收集2019年1月至2020年11月收治的7例血栓性APS患者的临床资料,对其临床资料、辅助检查、治疗措施及神经影像等进行回顾性分析。结果7例抗磷脂抗体均阳性,血栓累及脑、肾、肺1例,考虑灾难性APS,下肢反复动静脉血栓1例,下肢静脉血栓3例,脑梗死3例。病例中合并系统性红斑狼疮2例,合并强直性脊柱炎1例。予以华法林或利伐沙班抗凝等治疗。因合并结核性脑膜炎去世1例,其余6例症状均得到改善。结论血栓性APS的特点是静脉、动脉或微血管血栓形成,是一类异质性很强的疾病。脑循环是最常受影响的动脉领域,血栓性APS的治疗应该评估血栓的风险,分层治疗。     

Outcome of collagen vascular diseases by treatment with plasmapheresis.

The outcome of collagen vascular diseases after treatment with plasmapheresis was studied in 9 patients with polyarteritis nodosa (PN), in 2 patients with Wegener's granulomatosis (WG), in 1 patient with allergic granulomatous angitis (AGA), and in 20 patients with systemic lupus erythematosus (SLE) associated with antiphospholipid syndrome (APS). Improvement after treatment with plasmapheresis was observed in 41.7% of the patients with PN, WG, and AGA. On the other hand, with the exception of 1 patient with thrombocytopenia and 1 patient with renal failure, all of the clinical manifestations, including thrombocytopenia, central nervous system (CNS) lupus, thrombophlebitis, lung infarction, and recurrent abortions in the SLE patients with APS, improved after plasmapheresis. Plasmapheresis is thought to be an influential strategy of treatment for patients with collagen vascular diseases.     

Antibodies to beta2-glycoprotein-I: relation of anticardiolipin antibodies with clinical and laboratory parameters in patients with systemic lupus erythematosus

OBJECTIVES: There are controversial reports on the frequency of antiphospholipid antibodies (aPL) in patients with systemic lupus erythematosus (SLE). Thus, we aimed to determine the frequency and clinical importance of aPL isotypes in Turkish patients with SLE. DESIGN AND METHODS: Fifty-nine patients with SLE and 41 healthy controls were included. Serum aPL levels were measured both in patients and healthy subjects by ELISA. RESULTS: Fifteen of the patients with SLE had the antiphospholipid syndrome (APS) (25.4%). The percentage of anticardiolipin antibody (aCL)-positive SLE patients among all patients was 56%. At least one isotype of anti-beta(2)-glycoprotein I (beta(2)-GPI) antibody was positive in 83% of patients. The positivity rates of aCL and anti-beta(2)-GPI antibodies in patients with or without APS were higher than the healthy controls. There were positive correlations between isotypes of IgM aCL, IgG and IgM anti-beta(2)-GPI and manifestations of APS. CONCLUSION: It seems that the isotypes of IgM aCL, IgG and IgM anti-beta(2)-GPI are correlated with manifestations of APS. They may play a role in pathogenesis and may be helpful in establishing the diagnosis.     

脑静脉窦血栓和抗磷脂综合征

目的 脑静脉窭血栓(CVT)是抗磷脂综合征(APS)罕见的神经系统表现,为研究APS合并CVT的临床及神经影像特点,对有关患者的资料进行总结并和既往文献报道进行比较.方法 收集APS合并CVT患者的资料,并系统回顾相关Medline文献.结果 8例患者,男女比1:1,年龄20～48岁,平均(32.3±10.0)岁;狼疮抗凝物(LA)阳性和抗心磷脂抗体(ACL)阳性分别为6例和2例;迄今为止,文献共报道51例APS合并CVT的病例,年龄3周至74岁,平均(31.8±12.6)岁;LA阳性: ACL阳性为11:14.结论 对于无明确原发高凝状态的CVT患者,常规进行LA和ACL的筛查很必要;复发性CVT在LA阳性的APS患者中常见.     

Clinical and morphological characteristics of lupus nephritis in systemic lupus erythematosus with antiphospholipid syndrome

AIM: To ascertain clinical and morphological features of lupus nephritis (LN) in systemic lupus erythematosus (SLE) associated with antiphospholipid syndrome (APS). MATERIAL AND METHODS: Immunological markers of SLE and APS, clinical picture, urine indices were examined in 138 patients with SLE, APS and renal dysfunction. RESULTS: LN associated with APS is characterized with marked arterial hypertension, such patients had arterial thromboses more frequently than patients with isolated LN. Patients with anticardiolipin antibodies have arteriolosclerosis, in APS - diffuse interstitial sclerosis. CONCLUSION: Renal impairment in SLE may run not only with LN but also with thrombotic microangiopathy modifying clinical symptoms and course of the disease.     

Antiphospholipid syndrome

Antiphospholipid syndrome has received considerable attention from the medical community because of its association with a number of serious clinical disorders, including arterial and venous thromboembolism, acute ischemic encephalopathy, recurrent pregnancy loss, thrombocytopenia, and livido reticularis. It can occur within the context of several diseases, mainly autoimmune disorders, and is then called secondary antiphospholipid syndrome. However, it may be also be present without any recognizable disease, or so-called primary antiphospholipid syndrome. There is no defined racial predominance for primary antiphospholipid syndrome, although a higher prevalence of systemic lupus erythematosus (SLE) occurs in African Americans and the Hispanic population. Multiple terms exist for this syndrome, some of which can be confusing. Lupus anticoagulant syndrome, for example, is a misleading term, because patients may not necessarily have SLE, and it is associated with thrombotic rather than hemorrhagic complications. To avoid further confusion, antiphospholipid syndrome is currently the preferred term for this clinical syndrome. Antiphospholipid antibodies are found in 1% to 5% of young healthy control subjects; however, the incidence increases with age and coexistent chronic disease. The syndrome occurs most commonly in young to middle-aged adults; however, it also can occur in children and the elderly. Among patients with SLE, the prevalence of antiphospholipid antibodies is high, ranging from 12% to 30% for anticardiolipin antibodies, and 15% to 34% for lupus anticoagulant antibodies. In general, anticardiolipin antibodies occur approximately five times more often then lupus anticoagulant in patients with antiphospholipid syndrome. This syndrome is the most common cause of acquired thrombophilia, associated with either venous or arterial thrombosis or both. It is characterized by the presence of antiphospholipid antibodies, recurrent arterial and venous thrombosis, and spontaneous abortion. Rarely, patients with antiphospholipid syndrome may have fulminate multiple organ failure, or catastrophic antiphospholipid syndrome. This is caused by widespread microthrombi in multiple vascular beds, and can be devastating. Patients with catastrophic antiphospholipid syndrome may have massive venous thromboembolism, along with respiratory failure, stroke, abnormal liver enzyme concentrations, renal impairment, adrenal insufficiency, and areas of cutaneous infarction. According to the international consensus statement, at least one clinical criterion (vascular thrombosis, pregnancy complications) and one laboratory criterion (lupus anticoagulant, antipcardiolipin antibodies) should be present for a diagnosis of antiphospholipid syndrome. The hallmark result from laboratory tests that defines antiphospholipid syndrome is the presence of antibodies or abnormalities in phospholipid-dependent tests of coagulation, such as dilute Russell viper venom time. There is no consensus for treatment among physicians. Overall, there is general agreement that patients with recurrent thrombotic episodes require life-long anticoagulation therapy and that those with recurrent spontaneous abortion require anticoagulation therapy and low- dose aspirin therapy during most of gestation. Prophylactic anticoagulation therapy is not justified in patients with high titer anticardiolipin antibodies with no history of thrombosis. However, if a history of recurrent deep vein thrombosis or pulmonary embolism is established, long-term anticoagulant therapy with international normalized ratio (INR) of approximately 3 is needed.     

Infective endocarditis complicated with cerebral and splenic infarction in a hemodialysis patient

BACKGROUND:

Infective endocarditis (IE) has a high risk of morbidity and mortality. Complications are often due to systemic embolization. We treated a 47-year-old hemodialysis man with infective endocarditis complicated with cerebral and splenic infarction.

METHODS:

The patient was brought to the emergency department because of altered mental status and fecal incontinence. Although he did not meet the Duke Criteria for IE diagnosis, clinical suspicions of IE warranted further diagnostic studies. Magnetic resonance imaging of the brain revealed cerebral infarction with abnormal neurological findings. An abdominal computerized tomography revealed an incidental and unexpected splenic infarction without physical findings. Echocardiography revealed a vegetative growth (−1.2×1 cm) over the mitral posterior leaflet with severe mitral valve regurgitation. Based on these results, the patient was diagnosed with IE complicated with severe cerebral and splenic infarction.

RESULTS:

The patient was treated with intravenous teicoplanin including gentamicin, subcutaneous low molecular weight heparin, and oral acetylsalicylic acid. Mitral valve replacement surgery was performed after the patient improved clinically.

CONCLUSION:

Emergency physicians should be aware of the life-threatening complications of IE, which may be presented subtly or without clinical evidence.KEY WORDS: Infective endocarditis, Cerebral infarction, Splenic infarction