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。     

抗磷脂抗体与抗磷脂血栓综合征

抗磷脂抗体（ＡＰＡ）与抗磷脂血栓综合征（ＡＰＬ－Ｔ）的发生密切相关,但ＡＰＡ引起ＡＰＴ－Ｔ的发病机制仍不清楚,近年来ＡＰＡ通过抑制蛋白Ｃ（ＰＣ）途径,引起获得抗活化蛋白Ｃ现象进而导致血栓的研究受到关注。本主要将抗磷脂血栓综合征的研究进展作一综述。     

系统性红斑狼疮合并Wegener肉芽肿继发抗心磷脂抗体综合征1例

系统性红斑狼疮（SLE）是一种有多系统损害的慢性自身免疫性疾病，可伴有抗磷脂自身抗体，但We-gener肉芽肿继发抗磷脂抗体则少见，现报道我院收治的系统性红斑狼疮（SLE）合并Wegener肉芽肿，继发抗磷脂抗体综合征1例。     

抗心磷脂抗体检测和抗磷脂抗体综合征诊断

磷脂是指分子中含有醇 ,脂肪酸和磷酸基团的一类化合物。人体内的磷脂主要是含有甘油醇的甘油磷脂 ,包括心磷脂 ,磷脂酰丝氨酸 ,磷脂酰胆固醇 ,磷脂酰乙醇胺等。抗磷脂抗体 (antiphospholipidantibody ,aPL)是一族针对带负电荷磷脂或带负电荷磷脂与蛋白复合物的异质性抗体。抗磷脂抗体综合征 (antiphospholipidsyndrome ,APS) ,是一组与抗磷脂抗体有关的自身免疫性疾病 ,典型的临床表现有动脉血栓 ,静脉血栓以及妊娠丢失。APS患者血中检出aPL是确立APS诊断的必要条件。根据一些aPL可以识别磷脂或磷脂与蛋白复合物的特性 ,采用心磷脂包…     

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.     

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。     

SLE抗核抗体研究进展

ＳＬＥ病人血中能检出多种抗体，分述如下。一、抗ｄｓ－ＤＮＡ抗体抗ｄｓ－ＤＮＡ抗体是ＳＬＥ患者的标志性抗体，与抗原形成免疫复合物后沉积在组织及器官引起的炎症反应造成组织损伤［１，２］，在狼疮肾炎的发病机理中起着极为重要的作用。但临床研究发现，肾脏损伤的出现及损伤程度与抗ｄｓ－ＤＮＡ抗体的滴度不平行。因而近年来有人试图将抗ｄｓ－ＤＮＡ抗体分为致病性和非致病性两种，现已发现致病性抗ｄｓ－ＤＮＡ抗体是亲和力高［３］、能够固定补体［４］的ＩｇＧ型（少数为ＩｇＭ型）抗体。就抗体所带电荷而言，等电点（ＰＩ）８…     

Systemic lupus erythematosus complicated with reversible posterior encephalopathy syndrome: a case report

A 28-year-old female patient was hospitalized primarily because of “intermittent fever for 28 days aggravated by systemic rashes, oral ulcer, and edema in both eyelids for 5 days.” During treatment, convulsions and loss of consciousness occurred. Magnetic resonance imaging (MRI) of the head revealed an abnormal signal with shadows in the bilateral frontal, parietal, temporal, and occipital lobes; cerebellar hemispheres; and basal nodes, with high signal intensity on T2 weighted imaging (T2WI), on fluid-attenuated inversion-recovery, and of the apparent diffusion coefficient and low signal intensity on T1WI and diffusion weighted imaging. Therefore, the patient was diagnosed with systemic lupus erythematosus (SLE) with reversible posterior encephalopathy syndrome (RPES). Intravenous high-dose methylprednisolone and cyclophosphamide were administered for blood pressure control, which effectively controlled the disease. Therefore, when patients with SLE and hypertension or renal insufficiency or those receiving high-dose methylprednisolone or immunosuppressants suddenly present with neurologic abnormalities, a diagnosis of RPES must be considered, and head MRI is the first choice for diagnosis of this disease. In terms of treatment, the blood pressure should be quickly controlled, and the primary disease should be aggressively treated.     

系统性红斑狼疮合并缺铁性贫血一例

SLE是一种典型的自身免疫性结缔组织疾病,可累及全身多个器官和组织。该文报告一例SLE合并缺铁性贫血患者,对其诊断、治疗和发生缺铁性贫血的机制进行分析。该例为28岁女性患者,入院诊断为SLE,经治疗病情稳定并纠正贫血后,再次出现中度贫血,给予口服和静脉铁剂治疗后患者贫血可短暂改善,考虑与其长期服用质子泵抑制剂有关,经停用质子泵抑制剂并继续予铁剂治疗,贫血缓解。该例诊治提示,SLE患者长期服用质子泵抑制剂可增加缺铁性贫血的发生风险,其机制可能是通过影响铁的吸收而发挥作用。     

Multisystem organ failure secondary to Haemophilus parainfluenzae infective endocarditis on an ICD lead: A case report

Infection of implanted cardiac devices (ICD) is an unusual but life threatening event, rarely caused by Haemophilus parainfluenzae. While clinical presentation varies widely, infective endocarditis (IE) involving an ICD lead requires aggressive resuscitation and a multidisciplinary approach. We present a case of a 33-year-old intravenous drug user who presented in multisystem organ failure secondary to infective endocarditis on an ICD lead. This patient had a complicated hospital course requiring removal of her ICD, highlighting the dramatic presentation of this clinical state.     

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

BACKGROUNDSystemic 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 SUMMARYA 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 antithrombin Ш activity. 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. CONCLUSIONPC 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.     

罕见视神经脊髓炎合并系统性红斑狼疮1例并文献复习

报告1例罕见病变累及8个脊髓节段的视神经脊髓炎合并系统性红斑狼疮的病例,分析其发病现状和最新进展,并复习相关文献。1名48岁女性,因"左眼视力下降2个月,双下肢麻痹无力伴胸痛3d"入院,眼底血管造影提示左眼球后视神经炎,脊髓MRI提示C_5~T_7脊髓炎,经综合治疗后病情好转。视神经脊髓炎合并系统性红斑狼疮发病非常罕见,其发病机制与广泛的自体免疫性疾病有关,及时正确诊断和治疗对患者的预后起至关重要的作用。