系统性红斑狼疮患者发生静脉血栓栓塞事件的风险

系统性红斑狼疮患者发生静脉血栓(venous thromboembolism,VTE)事件,包括肺栓塞(pulmonary embolism,PE)和深静脉血栓(deep vein thrombosis,DVT)的风险显著增加。其病因及发病机制相当复杂,除原发病疾病活动因素外,还涉及抗磷脂抗体、低白蛋白血症、高球蛋白血症、长期应用糖皮质激素所导致的糖、脂代谢异常、肥胖等。至今,国内外文献尚未针对SLE患者发生VTE的危险因素进行全面系统性分析,本文将对SLE发生静脉血栓栓塞事件的相关研究进展做一综述。     

补体在产科抗磷脂综合征中的作用机制

产科抗磷脂综合征(obstetric antiphospholipid syndrome, OAPS)是一种以病理妊娠为主要临床表现伴抗磷脂抗体(antiphospholipid antibodies, APLs)持续阳性为特征的自身免疫性疾病,其病理妊娠包括反复早期流产、子痫或子痫前期、胎盘功能不全相关并发症及孕中晚期胎儿死亡等。APLs介导的补体活化是OAPS重要的发病机制之一。多项研究显示APLs免疫复合物主要通过补体活化经典途径启动,通过旁路途径进一步扩增,主要通过C5a与C5a受体结合介导炎症反应,导致不良妊娠结局。以补体通路为靶向的药物未来可能成为OAPS重要的治疗手段之一,特别是对应用阿司匹林和肝素治疗后仍然出现病理妊娠结局的难治性OAPS患者。     

血栓性抗磷脂综合征的十个常见问题

既往30年里,随着抗磷脂综合征(APS)相关研究不断深入拓展,临床医生对APS的认识和诊治理念已发生显著改变。本文结合临床中APS常见的十个问题,探讨其最新诊治进展,旨在提高临床医生对APS的认识与理解,实现早期诊断、早期干预,改善患者预后。     

抗磷脂抗体综合征抗凝过程中反复脑卒中一例

正抗磷脂抗体综合征(antiphospholipid syndrome,APS)是由抗磷脂抗体(anti-phospholipid antibody,aPL)引起的一组自身免疫性疾病,主要表现为反复动静脉血栓、习惯性流产和血小板减少。APS累及神经系统时可出现脑卒中、偏头痛、癫痫、舞蹈症等,是青壮年脑卒中的常见病因,据统计病因不明的45岁以下脑卒中患者中25%存在aPL[1]。     

抗膜突蛋白抗体与系统性硬化病相关肺间质病变的临床相关性研究

Objective To identify a novel auto-antibody in sera of systemic sclerosis (SSc) patients and to analyze its relevance with SSc-associated interstitial lung disease (ILD). Methods The anti-moesin antibody in the sera of 62 SSc patients, who had participated the European League Against Rheumatism's Scl eroderma Trial and Research Group (EUSTAR), were tested by enzyme linked immunosorbent assay (ELJSA). Patients were grouped by high resolution computerized tomography (HRCT) features, pulmonary function test (PFT) abnormalities, inflammatory markers and disease course. The prevalence and titer (Optical density value) of anti-moesin antibody were compared between groups with t and χ2 test. Results The titer of anti-moesin antibody was significantly higher in the SSc-ILD group than non-ILD group (0.156±0.062 vs 0.107± 0.026, P=0.005). Among SSc patients, the diagnostic sensitivity and specificity of the anti-moesin antibody for ILD was 44.0% and 91.7% respectively (Kappa=0.2, P=0.022). Anti-rnoesin antibody was more prevalent in SSc patients with HRCT features of honeycomb-like lesion, lobular septal thickening and mediastinal lymphadenopathy (P<0.05). SSc patients with deteriorated total lung volume (TLC %) had higher titer of anti-moesin antibody significantly (0.172±0.067 vs 0.133±0.039, P=0.011), as the same tendency in patients with decreased diffusing capacity of the lung for carbon monoxide (DLco% ) but without statistical significant difference (0.153±0.580 vs 0.120±0.340, P=0.089). The anti-moesin antibody was equally prevalent between abnormal ESR, C reactive protein, immunoglobulin and complements groups and their normal controls (P> 0.05). Group of patients who had SSc courses more than or less than 5 years demonstrated similar anti-moesin antibody titers (0.146±0.047 vs 0.164±0.077, P=0.272). However, patients with ILD courses less than 12 months had higher liter of the antibody than controls (0.182±0.073 vs 0.138±0.049, P=0.040). Conclusion This study suggests that the novel anti-moesin antibody has comparatively high specificity for SSc-associated ILD patients, which may contribute to further understanding the pathogenesis of ILD in SSc patients. Further investigations are deserved to evaluate the application of anti-moesin antibody in facilitating early screening and evaluation of ILD.     

第492例——反复血栓形成, 血小板减低

患者女性, 43岁。因反复血栓形成2年余, 血小板(PLT)减低1年余就诊。患者临床表现为反复动静脉血栓形成、少见部位血栓及抗凝治疗中新发血栓, 伴有进行性加重的血小板减少。外院及我院多次筛查抗核抗体、抗可溶性抗原抗体、抗磷脂抗体谱均为阴性。病程中糖皮质激素冲击及静脉免疫球蛋白(IVIG)曾使血小板升至正常, 骨髓巨核细胞成熟障碍, 支持免疫性血小板减低。入院后筛查易栓症的病因, 同时予肝素静脉泵入抗凝, 利妥昔单抗600 mg 1次;IVIG 20 g/d×3 d;口服艾曲波帕50 mg/d治疗。以上治疗3周后, 血小板减少无明显改善, 期间仍有新发血栓。后易栓症基因筛查回报PROS1基因杂合突变, MTHFR基因型为TT型, 发现少量IgGκ型M蛋白, 但仍不足以解释如此顽固的血栓倾向。在排除肿瘤、肝素诱导的血小板减少症等之后最终诊断血清阴性抗磷脂综合征可能性大。后续加用地塞米松20 mg/d×4 d, 联合口服西罗莫司2 mg/d加强免疫抑制, 抗凝方案过渡为低分子肝素后出院。1个月后电话随访, 患者颅内静脉窦血栓所致的头痛症状好转, PLT升至(20～30)×109/L, 无新...     

临床病例评析——突发胸痛左下肢肿痛颧部红斑

目的:提高临床对APS与SLE之间关系的认识。方法:报道1例以原发APS为首发临床表现,随诊过程中新发皮肤损害、抗dsDNA抗体阳性,转为患者的临床特点及诊治经过,并进行分析讨论。结果:15岁女性患者,病程初期表现为急性肺栓塞、下肢深静脉血栓,抗磷脂抗体高滴度阳性,其他自身抗体阴性,诊断原发性APS,予溶栓抗凝治疗后好...     

冠状动脉瘤样扩张伴狭窄闭塞、抗磷脂抗体综合征、肾病综合征、多囊肾一例

患者青年男性,早发心肌梗死,合并多囊肾,存在肾病综合征及抗磷脂抗体综合征,冠状动脉造影示多支冠状动脉瘤样扩张伴狭窄闭塞,植入支架后出现支架内反复再狭窄。抗磷脂抗体综合征为反复冠状动脉血栓形成的危险因素,且可累及肾脏并导致肾病综合征,而遗传性多囊肾可有肾病综合征临床表现且可因基因异常合并动脉瘤样病变。遗传因素、免疫因素、代谢因素等均参与其中,经多学科讨论并结合随诊结果,考虑患者为结缔组织病继发抗磷脂抗体综合征、肾病综合征和冠状动脉病变,针对原发病治疗后患者症状及指标均好转。本例患者的诊治拓宽了临床医师对年轻患者冠状动脉疾病背后病因的认识,也体现了多学科诊疗临床思维的重要性。     

肿瘤坏死因子-α拮抗剂用于结缔组织病相关肺动脉高压的治疗

肺动脉高压（pulmonary arterial hypertension，PAH）是结缔组织病（connec tivetissue disease，CTD）常见且严重的并发症之一，与特发性肺动脉高压相比较，其生存率更低。目前PAH的发病机制尚不明确，但有证据显示肿瘤坏死因子-α（TNF-α）在结缔组织病和肺动脉高压发病过程中起重要作用，并且血清TNF-α升高与CTD-PAH的疾病进展相关。新开发的生物制剂（infliximab，etanercept，adalimumab）能够选择性阻断TNF-α，从而为PAH的治疗提供了新的机会。     

反复胸痛、活动后气短、咯血一例

正本文报道1例以反复肺血栓形成起病并转为慢性血栓性肺动脉高压(chronic thromboembolic pulmonary hypertension,CTEPH)的原发性抗磷脂综合征(antiphospholipid syndrome,APS)病例,探讨APS患者出现CTEPH的高危因素。1病例报告患者,男性,29岁,因胸痛5月余、活动耐