Peri-nuclear antibodies correlate with survival in Greek primary biliary cirrhosis patients

AIM:To investigate possible associations of anti-nuclear envelope antibody(ANEA)with disease severity and survival in Greek primary biliary cirrhosis(PBC)patients.METHODS:Serum samples were collected at diagnosis from 147 PBC patients(85%female),who were followed-up for a median 89.5 mo(range 1-240).ANEA were detected with indirect immunofluorescence on 1% formaldehyde fixed Hep2 cells,and anti-gp210 antibodies were detected using an enzyme linked immunosorbent assay.Findings were correlated with clinical data,histology,and survival.RESULTS:ANEA were detected in 69/147(46.9%) patients and 31/147(21%)were also anti-gp210 positive.The ANEA positive patients were at a more advanced histological stage(Ⅰ-Ⅱ/Ⅲ-Ⅳ56.5%/43.5% vs 74.4%/25.6%,P=0.005)compared to the ANEA negative ones.They had a higher antimitochondrial antibodies(AMA)titer(≤1:160/>1:160 50.7%/49.3%vs 71.8%/28.2%,P=0.001)and a lower survival time(91.7 ±50.7 mo vs 101.8±55 mo,P=0.043).Moreover,they had more advanced fibrosis,portal inflammation,interface hepatitis,and proliferation of bile ductules(P =0.008,P=0.008,P=0.019,and P=0.027,respectively).They also died more frequently of hepatic failure and/or hepatocellular carcinoma(P=0.016).ANEA positive,anti-gp210 positive patients had a difference in stage(Ⅰ-Ⅱ/Ⅲ-Ⅳ54.8%/45.2%vs 74.4%/25.6%,P= 0.006),AMA titer(≤1:160/>1:160 51.6%/48.4%vs 71.8%/28.2%,P=0.009),survival(91.1±52.9 mo vs 101.8±55 mo,P=0.009),and Mayo risk score(5.5 ±1.9 vs 5.04±1.3,P=0.04)compared to the ANEA negative patients.ANEA positive,anti-gp210 negative patients had a difference in AMA titer(≤1:160/>1:160 50%/50%vs 71.8%/28.2%,P=0.002),stage(Ⅰ-Ⅱ/Ⅲ -Ⅳ57.9%/42.1%vs 74.4%/25.6%,P=0.033),fibrosis(P=0.009),portal inflammation(P=0.018),interface hepatitis(P=0.032),and proliferation of bile ductules(P=0.031).Anti-gp210 positive patients had a worse Mayo risk score(5.5±1.9 vs 4.9±1.7,P=0.038)than the anti-gp210 negative ones.CONCLUSION:The presence of ANEA and anti-gp210 identifies a subgroup of PBC pati     

Anti-neutrophil cytoplasm antibodies (ANCA) in rheumatoid arthritis: relationship to HLA-DR phenotypes,rheumatoid factor,anti-nuclear antibodies and disease severity

To investigate a possible relationship between the presence of anti-neutrophil cytoplasm antibodies (ANCA), rheumatoid factors (RF), anti-nuclear antibodies (ANA), disease severity and HLA-DR phenotypes, 46 consecutive ANCA⁺ and 48 ANCA^-, clinically well-documented RA patients were studied for RF, ANA and HLA-DR phenotypes. The 46 ANCA⁺ patients showed predominantly an atypical perinuclear staining pattern (89%). ANCA positivity was associated with higher RF titres (P<0.005) and advanced functional Steinbrocker grades III/IV (P<0.015). ANCA⁺ patients were also more often positive for ANA than ANCA^- patients (P<0.008). There was no correlation between ANCA positivity and certain HLA-DR phenotypes although the frequency of DR4⁺ (67% vs 52%) and, in particular, of DR4⁺ blanks (phenotypically homozygous) was increased in ANCA⁺ as compared to ANCA^- patients (20% vs 8%). DR4^-DR1^- RA patients were twice as frequent in the ANCA^- than in the ANCA⁺ group (22.9% vs 8.7%). Correspondingly, the DR4⁺DR1^- phenotype was increased among ANCA⁺ RA patients. Regarding functional Steinbrocker grades, the DR4⁺ phenotypes were slightly but not significantly increased in grades III and IV whereas ANCA positivity was significantly associated with severe functional Steinbrocker grades III/IV (66% ANCA⁺ vs 39% ANCA^-,P<0.015). ANCA positivity identified a population of RA patients with a long-standing and severe clinical course of the disease. There was no correlation between ANCA positivity and certain HLA-DR phenotypes.     

原发性胆汁性肝硬化临床特征分析

目的探讨原发性胆汁性肝硬化(primarybiliarycirrhosis,PBC)的临床特点,提高认识,寻找合理的治疗方法。方法对46例PBC患者的临床、病理、实验室检查资料及治疗进行回顾性分析。结果46例中男性3例,女性43例,平均发病年龄51±4岁。主要症状有乏力、腹胀、食欲不振、皮肤瘙痒等,发生率分别为87%、83%、76%和56%;主要体征包括黄疸(78%)、尿色加深(74%)、肝脾肿大(65%)等。实验室检查显示胆汁淤积和IgM增高,ALP、GGT和IgM分别为460.6±251.5U/L、644.1±246.3U/L和5.9±3.7g/L,多数患者的ALT、AST和TBIL升高。46例中有43例(93%)患者血清线粒体抗体或线粒体抗体M2亚型阳性。主要的组织学变化为小叶间胆管变性、减少伴有淋巴细胞和浆细胞浸润、细小胆管增生、胆栓形成等,但出现典型组织学变化的阳性率仅有33%。39例(84.8%)患者对以熊去氧胆酸(UDCA)治疗为主的综合治疗出现了满意的疗效。结论原发性胆汁性肝硬化主要发生于中年女性,临床症状和体征无特异性,肝功能异常以胆汁淤积为主,典型组织学变化的阳性率不髙,诊断需综合生化、免疫、病理和临床,UDCA可有效改善患者的症状和肝功能异常。     

Antineutrophil cytoplasmic antibodies (ANCA) in rheumatoid arthritis: a prospective study

To investigate a possible relationship between the presence of antineutrophil cytoplasmic antibodies (ANCA), rheumatoid factor (RF), antinuclear antibodies (ANA), complement, disease activity and disease severity, 111 clinically well-documented RA patients were studied prospectively for ANCA, RF, ANA, C-reactive protein (CRP), total haemolytic complement (CH50) and complement split product C3d. Disease activity and severity were also assessed clinically, as well as anamnestically, using the Hannover Activity of Daily Living Questionnaire, the functional Steinbrocker grades, and numeric and verbal rating scales. At a serum dilution of 1:50, 20% of the 111 sera showed predominantly an atypical perinuclear staining pattern. There was no correlation between ANCA positivity and serological markers, disease activity and discase severity. Regarding previous therapies with disease-modifying antirheumatic drugs, ANCA⁺ patients took sulphasalazine significantly more often than ANCA^- patients.     

Primary biliary cirrhosis-specific autoantibodies in first degree relatives of Greek primary biliary cirrhosis patients

AIM: To determine the prevalence and significance of primary biliary cirrhosis (PBC)-specific autoantibodies in first-degree relatives (FDRs) of Greek PBC patients.METHODS: The presence of antimitochondrial antibodies (AMA) and PBC-specific antinuclear antibodies (ANA) were determined using indirect immunofluorescence assays, dot-blot assays, and molecularly based enzyme-linked immunosorbent assays in 101 asymptomatic for liver-related symptoms FDRs of 44 PBC patients. In order to specify our results, the same investigation was performed in 40 healthy controls and in a disease control group consisting of 40 asymptomatic for liver-related symptoms FDRs of patients with other autoimmune liver diseases namely, autoimmune hepatitis-1 or primary sclerosing cholangitis (AIH-1/PSC).RESULTS: AMA positivity was observed in 19 (only 4 with abnormal liver function tests) FDRs of PBC patients and none of the healthy controls. The prevalence of AMA was significantly higher in FDRs of PBC patients than in AIH-1/PSC FDRs and healthy controls [18.8%, 95% confidence interval (CI): 12%-28.1% vs 2.5%, 95% CI: 0.1%-14.7%, P = 0.01; 18.8%, 95% CI: 12%-28.1% vs 0%, 95% CI: 0%-10.9%, P = 0.003, respectively]. PBC-specific ANA positivity was observed in only one FDR from a PSC patient. Multivariate analysis showed that having a proband with PBC independently associated with AMA positivity (odds ratio: 11.24, 95% CI: 1.27-25.34, P = 0.03) whereas among the investigated comorbidities and risk factors, a positive past history for urinary tract infections (UTI) was also independently associated with AMA detection in FDRs of PBC patients (odds ratio: 3.92, 95% CI: 1.25-12.35, P = 0.02).CONCLUSION: In FDRs of Greek PBC patients, AMA prevalence is significantly increased and independently associated with past UTI. PBC-specific ANA were not detected in anyone of PBC FDRs.     

原发性胆汁性肝硬化患者自身抗体测定的意义

目的:探讨自身抗体测定对诊断原发性胆汁性肝硬化(PBC)的临床意义.方法:PBC患者52例和非PBC患者202例,其中包括自身免疫性肝炎(AIH)41例,原发性硬化型胆管炎(PSC)18例,乙型肝炎(HBV)89例,丙型肝炎(HCV)54例以及健康体检者40例,采用间接免疫荧光法(IIF)检测抗核抗体(ANA)、抗平滑肌抗体(SMA)、抗线粒体抗体(AMA)、抗心肌抗体(HRA)、抗骨骼肌抗体(ASA)、抗胃壁细胞抗体(PCA)、抗双链DNA(ds-DNA)抗体、抗肝肾微粒体抗体(LKM)、抗可溶性肝抗原(SLA)抗体和抗中性粒细胞胞质抗体(ANCA)等自身抗体,ELISA法检测抗髓过氧化酶抗体(MPO),并对其结果进行回顾性分析.结果:PBC患者中AMA阳性例数最高为46例(88.5%),ANA阳性率为71.2%(37/52);非PBC患者中阳性例数则分别为20例(9.9%)和51例(25.2%),两组比较,有非常显著性意义(P<0.01).AMA、SMA抗体检测在PBC与AIH患者中,均有非常显著性意义(P<0.01).但两组之间的ANA阳性率无显著性意义(P>0.05).PSC患者18例ANA阳性6例,AMA阳性7例均低于PBC患者.HBV,HCV感染患者检测ANA阳性率分别只有9.0%和22.2%;AMA阳性率也只有7.9%和3.7%,与PBC患者比较均有显著性差异(P<0.01).PBC患者及对照组检测ds-DNA,ANCA,LKM,SLA和MPO抗体结果显示PBC患者检测最高的ANCA阳性率为26.9%(14/52),其次是MPO阳性率为25.0%(13/52),与AIH比较,均有非常显著性意义(P<0.01).结论:血清自身抗体的检测对诊断、治疗和阻止原发性胆汁性肝硬化的发展有着十分重要作用.对提高PBC同其他疾病鉴别诊断和治疗有着非常重要的意义.     

22例原发性胆汁性肝硬化的临床分析

目的 研究原发性胆汁性肝硬化（PBC）的临床特点、实验室检查、治疗转归，提高对PBC的认识。方法 分析22例PBC的临床表现、实验室检查及治疗转归。结果 22例PBC中女性20例，发病平均年龄51岁，主要症状包括皮肤瘙痒、乏力、纳差、腹痛，主要体征包括黄疸、肝大、脾大、腹水，实验室检查以ALP、高GGT、高胆红素血症、高球蛋白血症、存在多种自身抗体如抗线粒体抗体（AMA）、AMA－M2及抗核抗体（ANA）等，多数患者血ALT、AST升高，所有患者血清AST高于ALT。出现症状至临床确诊时间为2月－5年，平均8个月。治疗采用以熊去氧胆酸（UCDA）为主的综合方法，治疗3个月后ALP及TBil下降达50％以上者有12例，72．7％症状改善，死亡2例。结论 PBC以中年女性多，以肝脾肿大、黄疸、瘙痒、乏力为主要临床表现，肝功能异常以胆汁淤积为主，伴有高球蛋白血症及自身抗体；UCDA能够改善患者的症状和部分肝功能异常。     

老年抗中性粒细胞胞浆抗体相关性小血管炎肺部损害29例临床分析

目的 探讨老年抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎肺脏损害的临床特征.方法 分析2002年9月至2011年9月住院的29例60岁以上的ANCA相关性小血管炎肺损害患者的临床资料.结果 29例ANCA相关性小血管炎肺脏损害患者.常见症状有咳嗽、咯痰(19/29,65.5%);呼吸困难(14/29,48.2%);咯血(7/29,24.1%)和发热(16/29,55.1%).所有患者ANCA均阳性,其中髓过氧化物酶(MPO)-ANCA阳性率89.7%(26/29),蛋白酶3(PR3)-ANCA阳性率10.3%(3/29).胸部影像学表现为肺间质纤维化16例,磨玻璃影者6例,浸润影5例,结节性病变1例,胸腔积液1例.24例(82.8%)合并肾脏受累表现.经激素和免疫抑制剂治疗后短期预后较好,9例(31%)于初诊及随访期间因肺部感染呼吸衰竭等死亡.结论 老年原发性ANCA相关性小血管炎患者常有肺脏损害,其临床表现无特异性,肺间质纤维化、磨玻璃影为常见影像学表现,且多合并肾脏受累.     

抗线粒体抗体M2亚型IgG和IgA在原发性胆汁性肝硬化诊断中的作用

目的 评价以新型天然M2抗原和BPO融合蛋白M2-3E(BPO)为靶抗原的酶联免疫吸附(ELISA)法(抗-M2-3E ELISA)检测抗线粒体抗体M2亚型(AMA-M2)IgG和IgA抗体在原发性胆汁性肝硬化(PBC)诊断中的敏感性、特异性以及其临床意义.方法 分别用间接免疫荧光法(IFL)、以丙酮酸脱氢酶复合体为靶抗原的ELISA法(抗-PDC ELlSA),抗-M2-3E ELISA法检测107例PBC患者,87例疾病对照患者和26名健康体检者的AMA-M2 IgG和(或)IgA抗体.检测ALT,AST,总胆红素、白蛋白、Y-谷氨酰转肽酶、碱性磷酸酶、血清肌酐、IgG、IgM及IgA.对于符合正态分布的计量数据采用t检验,不符合正态分布者采用秩和检验. 结果 107例PBC患者抗-M2-3E ELISA法AMA-M2 IgG的检出率(90.6%)高于IFL法(81.3%)和抗-PDCELISA法(72.9%),t值分别为4.32和6.03,P值均<0.05.抗M2-3E ELISA法检测AMA-M2 IgA的检出率为55.1%,特异性为97.2%,而总体AMA-M2 IgG和(或)IgA的检出率为92.5%,特异性为95.2%.20例IFL检测AMA-M2 IgG为阴性的PBC患者中,抗-M2-3E ELISA法检出率为45%,AMA-M2 IgG和(或)IgA的检出率为55%,抗-M2-3E ELISA可以在超过一半的IFL阴性的患者中检出AMA-M2 IgG和(或)IgA.AMA-M2 IgG呈阳性的患者(97例)比呈阴性的患者(10例)有更加严重的组织学变化和更高的IgG,IgM和碱性磷酸酶水平.结论 抗-M2-3E ELISA法具有比IFL和抗-PDC ELISA法更高的敏感性和特异性,可作为第一轮AMA-M2的筛查.AMA-M2 IgG阳性可能说明该PBC患者有更加严重的疾病,但是AMA-M2 IgG和IgA都不能单独用于诊断PBC.     

Update on Anti-Saccharomyces cerevisiae antibodies, anti-nuclear associated anti-neutrophil antibodies and antibodies to exocrine pancreas detected by indirect immunofluorescence as biomarkers in chronic inflammatory bowel diseases： Results of a multicenter study

AIM: Anti-Saccharomyces cerevisiae antibodies (ASCA), anti-nuclear associated anti-neutrophil antibodies (NANA) and antibodies to exocrine pancreas (PAB), are serological tools for discriminating Crohn’s disease (CrD) and ulcerative colitis (UC). Like CrD, coeliac disease (CoD) is an inflammatory bowel disease (IBD) associated with (auto) antibodies. Performing a multicenter study we primarily aimed to determine the performance of ASCA, NANA and PAB tests for IBD diagnosis in children and adults, and secondarily to evaluate the prevalence of these markers in CoD. METHODS: Sera of 109 patients with CrD, 78 with UC, 45 with CoD and 50 healthy blood donors were retrospectively included. ASCA, NANA and PAB were detected by indirect immunofluorescence (IIF). RESULTS: ASCA /NANA- profile displayed a positive predictive value of 94.2% for CrD. Detection of ASCA was correlated with a more severe clinical profile of CrD and treatment of the disease did not influence their serum levels. ASCA positivity was found in 37.9% of active CoD.PAB were found in 36.7% CrD and 13.3% CoD patients and were not correlated with clinical features of CrD, except with an early onset of the disease. Fifteen CrD patients were ASCA negative and PAB positive. CONCLUSION: ASCA and PAB detected by IIF are specific markers for CrD although their presence does not rule out a possible active CoD. The combination of ASCA, NANA and PAB tests improves the sensitivity of immunological markers for CrD. Repeating ASCA, NANA, and PAB testing during the course of CrD has no clinical value.     

Antiendothelial cell antibodies in systemic lupus erythematosus

Objective: Anti‐endothelial cell antibodies (AECAs) are a heterogeneous group of antibodies against a variety of antigenic determinants on endothelial cells (EC). AECAs are known to play an immunopathogenic role in triggering EC activation, leading to vascular damage. The purpose of this study was to assess: (i) the incidence of AECAs in systemic lupus erythematosus (SLE) patients with nephritis (LN) and to compare this with SLE patients without clinical evidence of nephritis; and (ii) to understand the association of AECAs with disease severity based on renal histopathological findings. Method: Fifty‐three clinically and histopathologically proven cases of LN were studied along with 20 patients without evidence of nephritis. AECAs were detected by immunofluorescence using cultured human umbilical vein endothelial cells (HUVECs). The titres and immunoglobulin subclass of AECAs were also identified. Other autoantibodies were also detected. Results: In the LN group, 21 (39.6%) were AECA positive and 19 (35.8%) were antineutrophil cytoplasmic antibody (ANCA) positive. Autoantibodies to double‐stranded DNA (anti‐dsDNA) were present in 49 (92.4%) cases. In patients without nephritis, seven (35%) tested positive for AECA, five for ANCA and all were antinuclear antibody (ANA) positive. Anti‐dsDNA was detected in 16 patients (80%), higher incidence of AECAs was noted in diffuse proliferative glomerulonephritis (41.2%) as compared to focal proliferative glomerulonephritis (37.5%) and membranoproliferative glomerulonephritis (33.3%). IgG‐AECA subclass was noted in 85.7% patients, IgM‐AECA and IgG + M AECA subclasses of AECA were detected in 7.1% cases. AECAs were also found to be associated with other autoantibodies such as ANA, anti‐dsDNA and ANCA. Conclusion: No significant differences in AECA positivity was found between SLE with and without nephritis.     

抗嗜中性粒细胞胞浆抗体与狼疮性肾炎的关系

目的探讨抗嗜中性粒细胞胞浆抗体（ANCA）与狼疮性肾炎（LN）临床相关表现和发病机制的关系。方法分别应用间接免疫荧光法和酶联免疫吸附分析的方法,检测81例LN患者血清中的ANCA,并分析ANCA与LN临床表现和其它实验室检查结果之间的关系。结果单用间接免疫荧光法检测时,ANCA在LN中的阳性率是30.9％（25／81）。对间接免疫荧光法检测为阳性的血清,用酶联免疫吸附分析法进行验证,结果仅有72.0％（18／25）仍为阳性,全部是核周型ANCA（p-ANCA）,未见中央型ANCA（c-ANCA）出现。ANCA阳性组LN患者合并浆膜炎、神经系统累及、贫血、抗ds-DNA抗体阳性和低补体的频率均显著高于ANCA阴性组LN患者。结论ANCA在LN中的阳性率为30.9％,并与LN特定的临床表现相关,提示ANCA可能参与了LN的发病过程。     

36例原发性胆汁性肝硬化的临床特征分析

目的探讨原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)患者的临床特征,以提高对PBC的诊断与治疗水平。方法对具有完整资料的36例PBC的一般资料、从出现症状或肝功改变到确诊的时间、临床表现、血清化学指标、免疫学、影像学、病理学改变及治疗转归等进行回顾性分析。结果本组36例PBC患者中女性33例(91.7%)。从出现症状或肝功改变至确诊的平均时间为18.95个月,最长82个月。临床表现主要为乏力(80.6%)、皮肤瘙痒(69.4%);最主要的体征为黄疸(61.1%)和脾大(61.1%);AMA/AMA-M2阳性率达91.67%;r-谷胺酰转肽酶(GGT)、总胆红素(TBIL)、直接胆红素(DBIL)和血清白蛋白(ALB)在不同的临床分期中差异有统计学意义(P<0.05)。肝硬化前期组经熊去氧胆酸(UDCA)治疗前后肝功的对比差异有统计学意义(P<0.05),而肝硬化期组此项对比差异无统计学意义(P>0.05)。结论 PBC以中年女性多见,高滴度AMA及AMA-M2亚型是诊断PBC的主要指标。肝硬化期GGT、TBIL和DBIL的水平明显高于肝硬化前期,而ALB水平反而明显降低。该病病程较长,在早期主要表现为原发性胆汁性胆管炎,需较长时间才发展为原发性胆汁性肝硬化。以UDCA为主的综合治疗能够明显改善早期患者的症状和血清胆汁淤积指标。因此早期发现、早期治疗效果较好。     

Long-term remission by cyclosporine in a patient with eosinophilic fasciitis associated with primary biliary cirrhosis

A 70-year-old man was admitted to the hospital in June 1994 because of cutaneous induration of the extremities. Eosinophilic fasciitis was diagnosed on the basis of the course and distribution of the cutaneous lesions. Cyclosporine (100 mg/day) was given. After 4 weeks of treatment, cutaneous induration and limited joint mobility improved. Liver dysfunction had been diagnosed 5 years before the onset of eosinophilic fasciitis. Primary biliary cirrhosis (PBC) was diagnosed on the basis of the elevated serum biliary-enzyme levels, strongly positive antimitochondrial antibody titer, and histologic features of the liver-biopsy specimens showed stage-3 PBC. These findings suggested that eosinophilic fasciitis developed in association with PBC. PBC is often accompanied by autoimmune diseases, such as Sjögren’s syndrome and Hashimoto’s disease. To our knowledge, eosinophilic fasciitis associated with PBC has not been reported previously. We believe this is the first time a case of eosinophilic fasciitis occurring in a patient with PBC is documented.     

Predictive role of anti-gp210 and anticentromere antibodies in long-term outcome of primary biliary cirrhosis

Because some of the autoreactive T-cell clones specific for human PDC-E2 cross-react to mimicry peptides having an EIExDK motif derived from nuclear antigens such as human gp210 and sp100, we studied the clinical significance of antinuclear antibodies (ANA) in primary biliary cirrhosis (PBC) patients registered to the National Hospital Organization Study Group for Liver Disease in Japan (NHOSLJ). We found that there are two different types of progression in PBC; one is a hepatic failure-type progression which is represented by positive anti-gp210 antibodies and the other is a portalhypertension-type progression which is represented by positive anticentromere antibodies. We discuss the predictive role of these ANA in the long-term outcome of PBC and the mechanisms by which two different PBC progression types occur based on molecular mimicry and aberrant expression of nuclear antigens.     

Antineutrophil cytoplasmic antibodies in childhood systemic lupus erythematosus

We aimed to evaluate the presence of peripheral antineutrophil cytoplasmic antibodies (p-ANCA) and cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) in children with SLE and to correlate its association of laboratory findings.Twenty-one children with SLE were studied. Serum samples in patients were tested by indirect immuno-fluorescence (IIF) slide kit (INOVA) for c-ANCA and p-ANCA and by ELISA for myeloperoxidase (MPO-ANCA) and proteinase 3 (PR3-ANCA). All the patients but two were quiescent for lupus at the time of samplingSixteen of 21 patients showed positive IIF staining whereas only 5 had MPO-ANCA and 2 of nine PR3-ANCA.The data suggests that SLE may be associated p-ANCA directed against additional target antigens rather than MPO and may be implicated in the pathogenesis of SLE or may be only non-specific antibodies developed in lupus.     

中国人原发性胆汁性肝硬化的临床特点:52年文献的系统分析

目的 研究中国人原发性胆汁性肝硬化(PBC)的临床特点.方法 检索所有有关中国人PBC的文献报道并进行系统分析. 结果自1955-2007年共有103篇PBC相关文献,2740例病例报道(剔除重复报道).16篇文献中985例患者纳入研究,其中女:男为6.82:1,平均年龄42.0～56.2岁,发病至确诊的平均时间为12.0～98.4个月.最常见的症状为乏力(72.40%)、黄疸(67.41%)、纳差(68.58%)、瘙痒(45.60%),无症状患者占20%.最常见的体征为脾肿大(57.53%)、肝肿大(43.56%)和腹水(18.45%).肝功能检查见碱性磷酸酶、γ谷氨酰转肽酶显著升高,免疫球蛋白以1gM升高为主.抗线粒体抗体及其M2型阳性率分别为88.98%与82.65%.30.96%患者进行了肝活组织检查.伴发疾病以十燥综合征最常见(9.14%),其次为类风湿性关节炎(3.95%)和2型糖尿病(2.54%).使用熊去氧胆酸治疗者68.05%(345/507)有效.最主要死亡原因为上消化道出血(41.67%)和肝衰竭(41.67%).肝移植仍是终末期肝病惟一有效的治疗方法.结论 中国人PBC的特点同国外报道基本相似.进一步的研究应侧重于流行病学调查、无症状患者的早期诊断及长期随访、免疫学机制、高质量的临床试验以及肝移植作用等方面.     

Primary biliary cirrhosis in female subjects with sicca-associated antibodies

Abstract

The aim of this study is to clarify the time course of primary biliary cirrhosis (PBC) in subjects possessing anticentromere antibodies (ACA), anti-Ro, and/or anti-La antibodies, and who used alkaline phosphatase (ALP) as a serological marker for PBC. Female subjects (n = 165), who had at least one of ACA, anti-Ro, and/or anti-La, were enrolled in this study. Groups A (ACA alone, n = 44), B (anti-Ro alone, n = 54), E (anti-Ro and anti-La, n = 52), and DFG (ACA with anti-Ro and/or anti-La, n = 14) were analyzed. Healthy females (n = 65) were used as a control. The frequencies of the PBC in groups A (13.6%) and DFG (14.3%) were higher than those in groups B (1.9%) and E (0.0%). The ALP levels increased with age in groups A and DFG and slightly increased with age in groups B and C, and the control group. After correcting for age by analysis of covariance, a comparison of ALP levels among the groups not having anti-M₂ was as follows: group A ≒ group DFG > group B ≒ group E ≒ the control group. The subjects with ACA might thus have PBC more frequently than either those with anti-Ro and/or anti-La, or the control subjects.     

Primary biliary cirrhosis in female subjects with sicca-associated antibodies

The aim of this study is to clarify the time course of primary biliary cirrhosis (PBC) in subjects possessing anticentromere antibodies (ACA), anti-Ro, and/or anti-La antibodies, and who used alkaline phosphatase (ALP) as a serological marker for PBC. Female subjects (n = 165), who had at least one of ACA, anti-Ro, and/or anti-La, were enrolled in this study. Groups A (ACA alone, n = 44), B (anti-Ro alone, n = 54), E (anti-Ro and anti-La, n = 52), and DFG (ACA with anti-Ro and/or anti-La, n = 14) were analyzed. Healthy females (n = 65) were used as a control. The frequencies of the PBC in groups A (13.6%) and DFG (14.3%) were higher than those in groups B (1.9%) and E (0.0%). The ALP levels increased with age in groups A and DFG and slightly increased with age in groups B and C, and the control group. After correcting for age by analysis of covariance, a comparison of ALP levels among the groups not having anti-M₂ was as follows: group A ≒ group DFG > group B ≒ group E ≒ the control group. The subjects with ACA might thus have PBC more frequently than either those with anti-Ro and/or anti-La, or the control subjects.