低凝血酶原血症-狼疮抗凝物综合征:1例报道并文献复习

目的探讨低凝血酶原血症-狼疮抗凝物综合征(HLAS)的病因、临床表现、诊断和治疗。方法回顾性分析我院确诊的1例伴出血时间延长的HLAS患者的临床特点,并结合文献报道加以归纳总结。结果HLAS临床表现差异很大,与体内凝血酶原活性不完全平行。年轻患者病因以结缔组织病和病毒感染为主,前者激素治疗通常有效,少数需要加用免疫抑制剂或静脉注射用免疫球蛋白(IVIG);后者主要发生在儿童,通常在3个月内自发缓解,个别患者需要使用激素治疗。年长者常见病因为肿瘤、药物相关或原因不明。结论对于不明原因的出血患者,应警惕HLAS的可能。     

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.     

大动脉炎合并肺动脉高压患者的临床分析

目的 分析大动脉炎(TA)合并肺动脉高压(PAH)的临床特点,以提高临床早期诊治的水平.方法 1987-2007年北京协和医院住院治疗的TA患者共191例,对其中合并PAH的12例患者的临床表现、实验窒检查及治疗、预后进行回顾性分析.结果 12例患者中女性10例,男性2例.年龄14～47岁,平均(27±10)岁.11例有肺血管受累的临床表现和(或)体征,7例以肺血管受累表现(活动后气短、咯血)为首发症状,其他首发表现分别为发热4例,乏力4例,四肢缺血表现(下肢间歇性跛行、上肢痛、肢体麻木)4例,头晕3例.12例患者中7例为Ⅰ+Ⅳ型、1例为Ⅱ+Ⅳ型、3例为Ⅲ+Ⅳ型、1例为Ⅳ型.10例患者有炎性指标[红细胞沉降率(ESR)、C反应蛋白(CRP)]明显升高,ESR(66±56)mm/1 h.所有的患者均接受激素及免疫抑制剂治疗,1例行支架植入术,1例接受手术治疗.除1例因术后低心排出量死亡外,其余患者症状均好转.结论 PAH为大动脉炎累及肺血管的晚期及严重并发症,常合并有全身其他部位血管受累.早期因起病隐匿不易引起重视,应行肺血管造影、CTA或心脏超声进行早期筛查,早期治疗.治疗上除激素、免疫抑制剂治疗原发病外,当血管重度狭窄时可行支架植入或血管扩张术.     

白塞病伴发骨髓增生异常综合征的临床分析(附八例报告)

白塞病(BD)是一种原因不明的以血管炎为病理基础的疾病,以口腔和(或)外阴溃疡、皮肤及眼病为最常见,但胃肠道、心血管、神经系统、肺、肾、关节等多系统均可受累.     

间充质干细胞在系统性硬化的应用前景

间充质干细胞(mesenchymal stem cells,MSCs)多存在于骨髓中,是一类具有多向分化能力及免疫调节作用的细胞.近年来,MSCs在自身免疫性疾病中的应用受到很大关注.研究表明,MSCs分化为血管内皮细胞的能力受损在系统性硬化(systemic sclerosis,SSc)的病理改变中起重要作用.同时,...     

呕吐、腹泻、神志异常

病例报告患者男性,35岁,因呕吐、腹泻3个月于2009年7月6日入住北京协和医院消化科。2009年4月,进食不新鲜食物后出现恶心、呕吐,腹泻每天10次以上,为褐色稀糊便,可见脓,里急后重,     

类风湿关节炎合并持久性隆起性红斑一例

患者男,56岁,因多关节肿痛3年,四肢伸侧红斑、结节5个月于2010年5月来北京协和医院门诊就诊.患者3年前无明显诱因逐渐出现双手第2掌指关节、第3近端指间关节、双膝及双踝关节肿痛,伴晨僵约1 h;同时间断干咳,无活动后憋气、呼吸困难.     

复发性多软骨炎合并中枢神经系统病变两例

复发性多软骨炎(relapsing polychondritis,RP)是一种以间歇性或进行性加重的全身多发软骨破坏性炎症为特征的结缔组织病,主要累及耳软骨、呼吸道、关节软骨、眼部等[1].RP合并中枢神经系统病变(central nervous system,CNS)较为罕见,现将北京协和医院收治的2例RP合并CNS (RP-CNS)病例报道如下.     

系统性硬化病治疗进展

系统性硬化病(systemic sclerosis,SSc)是一种全身性自身免疾病,以皮肤纤维化及内脏受累(肺、消化系统、肾、心脏等)为特征.SSc的发病机制复杂,目前尚未阐明.因此SSc一直缺乏有效的治疗方法.现就SSc的免疫调节治疗、血管病治疗、抗纤维化治疗综述如下.     

系统性硬化病患者心室功能评价:超声心动图研究

目的 采用超声心动图技术对系统性硬化病(SSc)患者的心室功能进行评价.方法 对30例确诊为SSc的女性患者和15名年龄、性别匹配的健康人群进行超声心动图检查,同时采用组织多普勒(TDI)技术测量左、右心室心肌的运动速度和收缩期应变、应变率峰值;应用t检验进行统计分析,相关性分析采用Pearson法.结果 SSc患者和对照组相比,肺动脉收缩压增加[(38±16)和(24±5)mm Hg,t=2.79,P=0.008],而左心房室内径、左心室射血分数、右心房室内径和右心室面积改变分数差异无统计学意义;二尖瓣、三尖瓣血流舒张早期和晚期速度峰值比值差异无统计学意义.TDI分析显示与对照组相 比,SSc患者右心室心肌舒张和收缩功能均有受损,即三尖瓣环舒张早期峰值速度(Em)[(10.7±4.8)和(15.0±2.1)cm/s,t=-2.72,P=0.01]、右心室侧壁的应变收缩峰值[(-16.3±3.9)%和(-20.9±2.0)%,t=-3.62,,P=0.001]和应变率峰值[(-1.4±0.4)和(-2.4±0.5)/s,t=-6.15,P<0.01]均有明显减低.2组之间二尖瓣环速度峰值和左心室侧壁的收缩应变和应变率差异无统计学意义.结论 TDI技术无创、简便,可以早期发现ssc患者的心脏特别是有心室的功能损害.

Abstract：

objecfive To analyze ventricular myocardial function in patients with systemic sclerosis (SSc).Methods Thirtv female SSc patients and fifteen age-and sex-comparable healthy subjects underwent standard echo and tissue Doppler imaging(TDI)examination.T-test and Pearson's analysis were used.Results Both left and right atrial and ventricular diameters,left ventricular eiection fraction and right ventricular fraction of area change were comparable between the two groups.Mitral and tricuspid inflow E/A ratio was almost the same in both groups,while systolic pulmoHary pressure was increased in SSc [(38±16)vs (23.9±4.8)mm Hg,P=0.008].TDI detected impaired right ventricular myocardial function in SSc:eartydiastolic peak velocity Em [(10.7±4.8)vs(15.0±2.1)cm/s,P=0.01],peak systolic strain[(-16.3±3.9)%vs (-20.9±2.0)%,P=0.001]and strain rate[(-1.4±0.4) vs(-2.4±0.5)/s,P<0.01] were reduced significantly.Left ventricular myocardial function was not impaired in SSc patients.Conclusion TDI iS a valuable noninvasive tool for early detecting of ventricular myocardial involvement caused by SSc.