肺部受累嗜酸性肉芽肿性多血管炎的临床特点

目的分析肺部受累嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)患者的临床特点。方法回顾性分析北京协和医院2014年1月至2017年1月住院的12例EGPA肺部受累患者的临床资料,同时分检北京协和医院2000年1月至2017年1月住院的无肺部受累的EGPA患者17例作为对照。结果 12例肺部受累EGPA患者中男性6例,女性6例;年龄25~73岁,中位年龄46.5岁。100%以哮喘起病,到明确诊断EGPA的中位时间为31(14,51)个月。25%抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibody,ANCA)阳性,83.3%出现全身消耗症状,其余各系统受累情况为耳鼻喉83.3%、皮肤75.0%、神经系统75.0%、消化系统41.7%、心脏33.3%、肾脏8.3%。EGPA肺部受累患者出现全身消耗症状(83.3%vs.29.4%,P=0.004)及耳鼻喉受累(83.3%vs.35.3%,P=0.012)的发生率显著高于对照组,出现外周血管受累(0 vs.52.9%,P=0.003)尤其是肢端坏疽(0 vs.47.1%,P=0.009)的发生率显著低于对照组。EGPA肺部受累患者常见的呼吸系统症状为呼吸困难100%、咳嗽咳痰100%、鼻塞流涕66.7%、咯血16.7%、胸痛8.3%。肺功能表现为阻塞性通气功能障碍55.6%、限制性通气功能障碍11.1%和混合性通气功能障碍11.1%,肺通气功能正常占22.2%。肺部CT显示,83.3%有细支气管炎表现,75.0%有片状磨玻璃改变,8.3%有实变,16.7%有间质网格改变。结论 EGPA哮喘前驱期长,ANCA阳性率低,不易早期诊断。肺部受累与无肺部受累EGPA其他各系统受累情况相比,更多见全身消耗症状及耳鼻喉受累,更少出现外周血管受累。了解肺部受累EGPA患者各阶段临床特征,尤其是影像特征对提高早期诊断率非常重要。     

嗜酸性肉芽肿性多血管炎并发肾脏受累的临床特征

目的探究嗜酸性肉芽肿性多血管炎(eosinophilic granulomatous with polyangiitis,EGPA)肾脏受累患者的临床特征和实验室检查特点。方法回顾性收集北京协和医院2000至2017年EGPA患者住院病例,对并发肾脏受累患者的临床表现、实验室检查、治疗进行分析。结果 73例EGPA患者中17例(23.3%)出现不同程度的肾脏受累。2例患者以肾脏受累为首发表现。EGPA肾脏受累患者中5例为女性,12例为男性;平均年龄(43.5±14.1)岁;病程平均(51.5±92.9)个月。17例EGPA并发肾脏受累患者中鼻部受累15例,哮喘13例,外周神经受累13例,肺内病变12例,皮肤受累7例,心脏受累4例,消化道受累3例,中枢神经系统受累2例。17例肾脏受累患者中,14例ANCA阳性,比无肾脏受累患者阳性率显著増高(82.4%vs.20.8%,P0.001)。肾脏受累方面:15例患者出现血尿,14例出现蛋白尿,8例患者肌酐升高,3例患者出现少尿或无尿。2例患者进行了肾脏活检,病理均为局灶节段性肾小球坏死,伴新月体形成。15例患者应用大剂量激素联合环磷酰胺治疗,治疗效果好。结论 EGPA患者可出现肾脏受累,肾脏受累常表现为镜下血尿和蛋白尿,少数出现急进性肾小球肾炎。EGPA出现肾脏受累的患者ANCA阳性多见。应用激素和环磷酰胺治疗效果好。     

嗜酸细胞肉芽肿性血管炎抗中性粒细胞胞浆抗体阳性与阴性患者临床特征

嗜酸细胞肉芽肿性血管炎(EGPA)是以外周血和受累脏器组织中嗜酸性粒细胞增高并伴有坏死性肉芽肿为特征的系统性血管炎,依据血清抗中性粒细胞胞浆抗体(ANCA)检测结果可分为ANCA阳性及ANCA阴性表型,ANCA阳性患者更易出现神经系统、肾脏、皮肤紫癜和肺泡出血;心脏和肺受累在ANCA阴性患者中更常见。本文就ANCA阳性与阴性患者的临床表现及受累脏器特征进行文献复习,并综述如下。     

强直性脊柱炎伴发炎症性肠病的临床资料分析

目的分析强直性脊柱炎(AS)伴发炎症性肠病(IBD)的临床特征及功能状态,探讨AS合并与未合并IBD者间的差异,提高对其的认识,为临床诊疗提供依据。方法从脊柱关节炎智能管理系统中选2016年4月至2017年6月连续就诊于解放军总医院第一医学中心风湿科门诊就诊的AS患者,通过消化内镜或病理检查确诊(包括溃疡性结肠炎、克罗恩病)IBD。分析其临床资料,包括一般资料、病情评估、实验室检查、疾病严重程度评价。结果共纳入893例AS患者,男739例,女154例,年龄(30.8±8.8)岁。AS合并IBD者64例,未合并IBD者829例。合并IBD者年龄高于未合并IBD者[(34.5±7.5)岁比(30.5±8.8)岁,P<0.001],病程长于未合并IBD者[(10.8±6.9)年比(8.1±5.9)年,P=0.001]。合并IBD者更易出现颈椎[21.9%(14/64)比10.5%(87/829),P=0.006]、胸椎[29.7%(19/64)比12.3%(102/829),P<0.001]受累,且易合并葡萄膜炎[28.1%(18/64)比16.4%(136/829),P=0.017]和银屑病[7.8%(5/64)比2.3%(19/829),P=0.009]。合并IBD者Bath AS疾病活动度(BASDAI)、Bath AS功能指数(BASFI)、国际脊柱关节炎评价协会健康指数(ASAS HI)均高于未合并IBD者[BASDAI:3.3±2.1比2.4±1.8,P<0.001;BASFI:2.2(1.0,3.3)比1.1(0.2,2.4),P<0.001;ASAS HI:7.1±4.3比5.3±3.7,P=0.001]。结论合并IBD的AS患者疾病活动度更高,机体功能和总体健康指数更差,发生银屑病和葡萄膜炎的机会增加。     

嗜酸性肉芽肿性多血管炎并发中枢神经系统受累八例

目的分析嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)并发中枢神经系统受累患者的临床特点。方法回顾性分析2000年1月至2017年1月北京协和医院诊治的EGPA并发中枢神经系统受累患者的临床、实验室检查资料以及治疗和预后。结果 73例EGPA患者中,66例资料齐全;8例(11%)并发中枢神经系统受累,男∶女=3∶1,其中4例脑梗死或缺血、2例脑出血、1例蛛网膜下腔出血、1例颈髓受累。与未并发中枢神经系统受累EGPA患者相比,中枢神经系统受累患者更容易出现消化道受累(6/8 vs.10/58,P=0.002)。8例中枢神经系统受累患者中,4例抗中性粒细胞胞浆抗体阳性;6例行腰椎穿刺术检查,其中4例脑脊液压力升高,1例蛛网膜下腔出血患者表现为血性脑脊液,4例脑脊液蛋白升高;4例接受糖皮质激素冲击治疗,2例接受大剂量糖皮质激素治疗,另有2例接受中等剂量糖皮质激素治疗,免疫抑制剂首选环磷酰胺;5例经长期随访病情稳定,1例因消化道穿孔死亡,1例因脑出血死亡,1例失访。结论中枢神经系统受累为EGPA的少见并发症,EGPA患者出现中枢神经系统症状时应警惕原发病累及中枢神经系统的可能,积极控制原发病为治疗的基础。     

循环CD133+/KDR+内皮祖细胞水平与急性缺血性卒中患者转归的相关性

目的 探讨急性缺血性卒中患者循环CD133+/KDR+内皮祖细胞(endothelial progenitor cells,EPCs)水平与转归的关系.方法 纳入发病24 h内的首次急性缺血性卒中住院患者以及年龄和性别相匹配的健康体检者.收集患者人口统计学和临床资料.采用流式细胞术检测CD133+/KDR+EPCs水平.在发病后90 d时对所有患者进行随访,采用改良Rankin量表评价临床转归,0~2分定义为转归良好,>2分定义为转归不良.结果 共纳入连续126例缺血性卒中患者以及60例年龄和性别相匹配的健康体检者.在缺血性卒中患者中,大动脉粥样硬化(large artery atherosclerosis,LAA) 33例(26.19%),小动脉闭塞(small artery occlusion,SAO)74例(58.73%),心源性栓塞(cardioembolism,CE)19例(15.08%);82例(65.08%)转归良好,44例(34.92%)转归不良.LAA型(0.071%±0.018%)、CE型(0.068%±0.016%)和SAO型(0.118%±0.012%)患者基线循环EPCs数量均显著低于对照组(0.246%±0.052%;P均<0.05);CE型(P=0.028)和LAA型(P=0.037)均显著低于SAO型;CE型低于LAA型,但差异无统计学意义(P=0.762).转归不良组LAA型(40.91%对18.29%;χ2=7.577,P=0.006)和CE型(29.55%对7.32%;χ2=11.049,P=0.001)和心房颤动(29.55%对10.98%;χ2=6.582,P=0.009)患者的构成比以及年龄[(69.64±9.62)岁对 (61.12±7.31)岁;t=5.570,P<0.001]、基线NIHSS评分[(14.16±4.22)分对 (6.96±2.04)分;t=12.919,P<0.001]、基线收缩压[(176.06±13.42)mmHg对 (164.12±11.69)mmHg,1 mmHg=0.133 kPa;t=5.187,P<0.001]、低密度脂蛋白胆固醇[(2.92±0.52)mmol/L对 (2.49±0.36)mmol/L;t=5.447,P<0.001]、空腹血糖[(8.76±2.88)mmol/L对 (6.82±2.24)mmol/L;t=4.185,P<0.001]、C反应蛋白[(7.62±1.82)mg/L对 (4.57±1.58)mg/L;t=9.790,P<0.001]和D-二聚体[(1.14±0.08)mg/L对 (0.97±0.22)mg/L;t=4.946,P<0.001]水平均显著高于转归良好组,而SAO型患者构成比(29.55%对74.39%;χ2=23.759,P<0.001)以及高密度脂蛋白胆固醇[(0.94±0.68)mmol/L对 (1.16±0.14)mmol/L;t=2.829,P=0.005]和基线EPCs(0.069%±0.018%对0.098%±0.021%;t=7.755,P<0.001)水平显著低于转归良好组.多变量logistic回归分析显示,基线NIHSS评分较高(优势比1.242,95%可信区间1.126~1.372;P<0.001)、CE型(优势比3.460,95%可信区间1.312~5.146;P=0.016)和基线EPCs数量较低(优势比1.632,95%可信区间1.006~3.024;P<0.001)是急性缺血性卒中患者转归不良的独立危险因素.结论 急性缺血性卒中患者循环EPCs水平显著降低,基线EPCs水平较低是缺血性卒中患者90 d时转归不良的独立预测因素.     

抗中性粒细胞胞浆抗体阳性与阴性微型多血管炎的比较

目的比较抗中性粒细胞胞浆抗体(ANCA)阳性(ANCA+ve)与ANCA阴性(ANCA-ve)微型多血管炎(MPA)临床与病理的差异,探讨ANCA与MPA病情及预后的关系.方法31例微型多血管炎(MPA),其中男16例,女15例,同时采用标准间接免疫荧光(IF)和ELISA法检测血清ANCA,根据血清ANCA结果分为ANCA+ve组与ANCA-ve组,比较两组肾脏及肾外脏器损害、肾脏病理及预后.两组患者血管炎活动分数(BVAS1)无差异.结果①血清ANCA31例MPA中19例ANCA阳性(61.3%),其中IF-ANCA阳性者8例,ELISA法ANCA阳性者19例.血清抗-GBM抗体均阴性.②发病年龄及病程ANCA+ve组大于ANCA-ve组,分别为(56±11)岁vs(48.5±16.8)岁(P＜0.05)和(30.2±47.7)月vs(6.5±8.8)月(P＜0.01).ANCA+ve组女性多于男性(男/女8/11),而ANCA-ve组男性多见(男/女8/4).③ANCA+ve组需透析病例数及活检时SCr水平(588±334)μmol/L高于ANCA-ve(487±284)μmol/L(P＜0.05),贫血程度更为严重,Hb(78.9±16.2)g/Lvs(95.5±22.0)g/L(P＜0.01).肾活检显示ANCA+ve组新月体比例显著高于ANCA-ve组(61.7±33.2)%vs(33.4±32.8)%(P＜0.01),ANCA-ve组细胞性新月体所占比例高于ANCA+ve组.两组袢坏死、全球硬化、间质血管炎及酸性粒细胞浸润程度相似.④ANCA+ve组伴有2个以上肾外脏器受累比例高于ANCA-ve组(63.2%vs31.3%),肺损害较重,咯血(42.1%)及严重肺出血(15.8%)患者均见于ANCA+ve组.ANCA+ve组消化道出血及眼部受累多见,而皮肤损害ANCA-ve组发生率高于ANCA+ve组(41.7%vs15.8%).⑤ANCA-veMPA预后差.两组治疗方法相似,但治疗后ANCA+ve组仅1例肾功能恢复正常,绝大多数患者需维持透析或处于CRF,5例死亡,而ANCA-ve组3例需要维持性透析,4例肾功能恢复正常,无一例死亡,其余病例肾功能均显著改善.结论血清ANCA阳性与ANCA阴性MPA无论在临床表现、肾脏病理及预后方面均存在一定差异,临床应分别对待.造成这种差异的机制有待进一步研究.     

肾脏受累为主的抗中性粒细胞胞质抗体相关性血管炎患者的预后及其相关危险因素分析

目的 探讨肾脏受累为主的抗中性粒细胞胞质抗体(ANCA)相关性血管炎患者的预后及其影响因素.方法 对2006-2008年我院诊断的64例肾脏受累为主的ANCA相关性血管炎患者进行回顾性分析,并对其进行前瞻性随访,使用Logistic回归、Cox回归等分析与患者肾脏预后有关的危险因素.结果 ①共入选64例患者,其中男性40例,女性24例,平均年龄(59.9±2.0)岁.随访21～55个月,平均(38±16)个月,期间34例(53％)患者出现死亡或进入长期肾脏替代治疗.②预后不同的2组患者起病时血肌酐[分别为(624±246)、(245±127) μmol/L,t=7.17,P=0.005]、红细胞沉降率(ESR)[分别为(112±24)、(76±48) mm/l h,t＝3.74,P＜0.01]、血浆白蛋白[分别为(294±31)、(316±42) g/L,t=-2.27,P=0.01]、血红蛋白[分别为(79±13)、(99±33) g/L,t=-3.23,P＜0.01 ]差异均有统计学意义.Logistic回归及Cox回归分析证实起病时血肌酐[β=1.004,95％可信区间(CI) 1.002～1.006,P＜0.01]及ESR[β=1.018,95％CI1.000～1.037,P=0.046]是预测患者预后的独立危险因素.③ROC曲线结果显示起病时血肌酐和ESR曲线下面积分别为0.95,0.80;敏感性均为94％,特异性分别为93％和70％.结论 以肾脏受累为主的ANCA相关性血管炎患者预后差,起病时血肌酐水平和ESR可能是判断患者预后的重要影响因素.     

新疆地区维吾尔族及汉族溃疡性结肠炎患者人类白细胞抗原-DRB1等位基因与抗中性粒细胞胞质抗体的相关性

目的 研究人类白细胞抗原(HLA)-DRB1基因多态性与新疆地区维吾尔族及汉族溃疡性结肠炎(UC)患者抗中性粒细胞胞质抗体(ANCA)的关联性.方法 用间接免疫荧光法分别对62例维吾尔族UC患者、58例汉族UC患者、188名维吾尔族健康对照者、184名汉族健康对照者进行血清ANCA检测,采用聚合酶链反应直接测序分型法(PCR-SBT)进行HLA DRB1基因分型,分别在维吾尔族和汉族内比较ANCA阳性者、ANCA阴性者及健康对照者的HLA-DRB1等位基因频率,并按UC临床类型、严重程度、受累范围进行分层分析.应用SPSS 17.0统计软件进行x2检验,当P＜0.05时,计算比值比(OR)和95％可信区间(95％ CI).结果 维吾尔族UC患者ANCA阳性率[53.2％ (33/62)]高于汉族UC患者[34.5％ (20/58)],差异有统计学意义(x2=4.269,P＝0.045).在维吾尔族中,ANCA阳性UC患者HLA-DRB1* 13的基因频率(0.202)显著高于ANCA阴性患者(0.017,x2＝10.092,P＝0.016,OR＝16.000,95％CI:2.892～88.524)和健康对照组(0.075,x2=9.351,P＝0.040,OR=3.407,95％CI:1.666～6.971).ANCA阳性的全结肠炎型UC患者HLA-DRB1* 13基因频率(9/15)高于ANCA阴性的全结肠炎型UC患者(1/14),差异有统计学意义(x2=8.955,P=0.040,OR＝19.500,95％CI:2.787～136.461).在汉族中,HLA-DRB1 各等位基因在ANCA阳性者、ANCA阴性者和健康对照者间差异均无统计学意义(P均＞0.05),分层分析所获结果亦然.结论 在新疆地区维吾尔族UC患者中,HLA-DRB1* 13可能与ANCA有关,还可能与全结肠炎型患者的ANCA有关.在新疆地区汉族患者中则可能无此关联性.     

426例抗中性粒细胞胞浆抗体相关性小血管炎患者多系统临床表现和肾脏病理分析

目的 分析426例抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎患者多系统的临床和病理表现。方法 回顾性分析我院1997年-2004年6月检测并明确诊断的426例ANCA相关性小血管炎患者的临床病理资料。结果 426例患者中,70例胞浆型ANCA（cANCA）阳性,均识别蛋白酶3（PR3）;354例环核型ANCA（pANCA）阳性,均识别髓过氧化物酶（MPO）。201例（47．2％,201／426）患者是在发病后3个月内确诊。临床表现呈多器官受累,其中cANCA阳性者皮疹、关节痛、眼、鼻受累的发生率显著高于pANCA阳性者,而pANCA阳性者。肾脏受累和乏力的发生率显著高于cANCA阳性者。多数患者有贫血,血沉增快,C反应蛋白增高。采用糖皮质激素联合环磷酰胺进行强化免疫抑制治疗,诱导缓解期的缓解率为88．5％。结论 ANCA相关性小血管炎在我国并非少见,临床表现呈多器官受累,ANCA检测有助于早期诊断。     

抗Ro52抗体阳性的抗合成酶综合征患者的临床特征分析

分析抗Ro52抗体阳性的抗合成酶综合征（ASS）患者的临床特征。收集2017年至2020年郑州大学第一附属医院风湿免疫科和呼吸科住院确诊的203例ASS患者,回顾性分析其一般资料、临床表现、实验室检查等。203例ASS患者中男55例,女148例,ASS发病年龄（51.9±13.3）岁。抗Ro52抗体阳性者163例,抗R...     

老年嗜酸性肉芽肿性多血管炎患者临床特点及预后

目的 年龄是影响抗中性粒细胞胞浆抗体相关性血管炎预后的重要因素,但针对老年嗜酸性肉芽肿性多血管炎(EGPA)患者的研究较为缺乏,本研究旨在探索老年EGPA患者的临床特点及预后影响因素.方法 回顾性分析2000年1月至2015年12月于北京协和医院确诊的EGPA患者,将患者分为老年组(年龄≥60岁)及非老年组(年龄<60...     

Prevalence and spectrum of rheumatic diseases associated with proteinase 3-antineutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA

OBJECTIVES: To evaluate the prevalence and association of antineutrophil cytoplasmic antibodies (ANCA) and their subtypes [proteinase 3 (PR3)-ANCA, myeloperoxidase (MPO)-ANCA] with distinct clinical features in various clinicopathological syndromes. METHODS: All consecutive ANCA-positive patients seen at the combined unit for rheumatology for Bad Bramstedt and the University of Lübeck between 1989 and 1999 were analysed. ANCA were detected by an immunofluorescence technique and ANCA subspecificities were determined by ELISA. Clinical features at presentation and diagnoses were recorded according to standardized procedures. RESULTS: Among 4620 patients tested, 333 were cytoplasmic ANCA-positive and 291 were perinuclear ANCA-positive. cANCA/PR3-ANCA were strongly associated with Wegener's granulomatosis (WG), whereas pANCA/MPO-ANCA were associated with a diverse disease spectrum. Further investigation of PR3-ANCA-positive (n=80) and MPO-ANCA-positive patients (n=40) revealed a greater extent of disease [disease extent index (DEI); median 8 vs 5, P<0.01] and more frequent involvement of the upper/lower respiratory tract and the eyes in PR3-ANCA-positive than in MPO-ANCA-positive patients. Fewer than 5% of WG patients were MPO-ANCA-positive. Compared with matched PR3-ANCA-positive WG patients, the MPO-ANCA-positive WG patients had a lower DEI (median 5 vs 8) and had a lower frequency of peripheral neuropathy. CONCLUSIONS: ANCA testing is useful due to its high sensitivity and specificity, especially for cANCA/PR3-ANCA in WG. We found a divergence in the disease spectrum between PR3- and MPO-ANCA-positive patients, characterized by higher DEI and extrarenal manifestations in the PR3-ANCA group. MPO-ANCA was rarely found in WG and was associated with less organ involvement.     

银屑病关节炎患者中代谢综合征的患病率及其各组分与临床特征的关联研究

目的:研究PsA患者中代谢综合征（MS）的患病率及MS各组分与PsA临床特征的关联。方法:纳入自2017年1月至2019年9月就诊于我科的PsA患者,收集其人口学及临床资料、实验室检查、MS相关指标（身高、体质量、腰围、血压、空腹血糖、血脂谱）及有无高血压、糖尿病、动脉粥样硬化、冠状动脉粥样硬化性心脏病（冠心病）、脑血...     

Renal Involvement as Rare Acute Tubulointerstitial Nephritis in a Patient with Eosinophilic Disorder Treated with Early Add-on Administration of Mepolizumab

A 39-year-old man presented with peripheral eosinophilia, pulmonary eosinophilic infiltrate, and renal failure due to acute tubulointerstitial nephritis (TIN). He had experienced childhood asthma and was negative for anti-neutrophil cytoplasmic antibody (ANCA). He was tentatively diagnosed with ANCA-negative eosinophilic granulomatous polyangiitis (EGPA) or idiopathic hypereosinophilic syndrome (HES). Renal involvement of isolated TIN with eosinophil infiltration is rare in EGPA and HES and does not seem to have a good prognosis in the literature. However, his condition improved well with corticosteroids and mepolizumab. The revised classification of EGPA based on the etiology should dictate the proper treatment in suspected EGPA patients with nonsystemic vasculitis.     

Update on eosinophilic granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome) is a rare form of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis characterized by eosinophil-rich granulomatous inflammation and small to medium-size vessel vasculitis associated with bronchial asthma and eosinophilia. Its rarity and unique features such as eosinophilic inflammation have delayed progress of research regarding EGPA for several years, compared to other forms of ANCA-associated vasculitis. However, recently, attention to EGPA as a research subject has been gradually increasing. To resolve problems in existing criteria for EGPA, new classification criteria for EGPA generated by a large international cohort will be launched and is being expected to accelerate future studies. Pathogenesis and roles of ANCA in EGPA are still largely unknown; however, it has been reported that glomerulonephritis is more frequent in ANCA-positive patients than in ANCA-negative patients, while heart failure is more frequent in ANCA-negative patients than in ANCA-positive patients. In addition, a recent genome-wide association study has suggested the presence of two genetically distinct subgroups of EGPA, which correspond to ANCA-positive and -negative subgroups. Although responses to glucocorticoids in EGPA are generally good, patients with EGPA often experience a relapse. Currently, there is no standard therapy for EGPA based on accumulation of clinical trial results. Recently, clinical benefits of mepolizumab for EGPA were proved by a randomized controlled trial and mepolizumab was approved for EGPA. In addition, various new drugs are under evaluation. To find optimal use of these drugs and to resolve unmet needs, such as relapse prevention, will be needed in future.     

Chronic but not acute graft-versus-host disease improves outcome in multiple myeloma patients after non-myeloablative allogeneic transplantation

The outcome of 29 multiple myeloma patients receiving fludarabine and melphalan-based non-myeloablative allogeneic transplant (NMT) was evaluated. Event-free survival (EFS) at 24 months was 33%, being significantly higher for patients who developed chronic graft-versus-host disease (cGVHD) when compared with those who did not [51%vs 0% respectively, P = 0.02; hazard rate = 3.16 (95% confidence interval = 1.09-9.15, P = 0.03)] as well as for patients transplanted in complete remission/partial response (CR/PR) or stable disease (SD), compared with those with refractory/progressive disease (43%vs 0% respectively, P = 0.02). Overall survival (OS) at 24 months was 60%[72%vs 42% for patients who did and did not develop cGVHD respectively (P = 0.1); 63%vs 41% for patients in CR/PR or SD vs refractory/progressive disease at transplant respectively (P = 0.013)]. At a median follow-up of 366 d, 13 patients remained in CR/PR (45% overall response rate). Nine patients have died, three of them as a result of disease progression and six (21%) as a result of transplant-related mortality (TRM). Actuarial incidence of TRM was 37% for patients who developed acute GVHD vs 13% for those who did not (log rank, P = 0.04). The present study suggests that graft-versus-myeloma effect is the main weapon for disease control after NMT in MM patients and the efficacy of this immune effect depends on tumour burden before transplant.     

抗着丝点抗体阳性原发性干燥综合征的临床特点

目的 通过分析抗着丝点抗体(ACA)阳性原发性干燥综合征(pSS)患者的临床特点,提高对本病的诊治水平.方法 回顾性分析北京协和医院风湿免疫科收治的60例ACA阳性pSS患者(ACA阳性组)的临床资料,并与同期收治的139例ACA阴性pSS者(ACA阴性组)相比较.结果 (1)ACA阳性组发病年龄晚于ACA阴性组[(48±11)岁比(41±12)岁,P=0.000],而2组在性别构成、口干、眼干、唇腺活检阳性率差异元统计学意义.(2)肝脏受累是ACA阳性组患者最常见的腺外脏器损害,其发生率远高于ACA阴性组(68.3%比37.0%,P=0.000),而肾脏受累(13.3%比30.9%,P=0.009)、神经系统受累(1.7%比11.5%,P=0.025)、高γ球蛋白血症的发生率(20.8%比45.7%,P=0.002)均低于ACA阴性组.(3)2组抗核抗体阳性率无差异,但ACA阳性组以散点型免疫荧光表型最为常见(占61.7%).与ACA阴性组相比,ACA阳性组中抗SSA抗体、抗SSB抗体、抗U1 RNP抗体的阳性率减低(P＜0.05),而抗线粒体抗体及其M2亚型的阳性率增高(P＜0.05).(4)ACA阳性组死亡5例,其中3例死于胃食管静脉曲张破裂出血.结论 对ACA阳性pSS者应警惕肝脏受累.由于ACA阳性pSS在发病年龄、腺外器官受累、免疫指标、自身抗体谱及预后方面均不同于经典pSS,故可能是pSS的一种独特亚型.