以黄疸为首发表现的自身免疫性肝炎患者的临床特征

目的分析以黄疸为首发表现的自身免疫性肝炎(AIH)患者临床特点。方法选取AIH患者63例,其中以黄疸为首发表现的患者28例和非黄疸患者35例。比较两组患者临床症状体征、血清生物化学指标、免疫学指标及肝活组织病理。结果黄疸组与非黄疸组AIH患者相比,在免疫学指标、自身抗体阳性率、典型组织病理学特征比较,差异均无统计学意义(Z=-0.224、0.318,χ2=0.053、0.237、0.129、0.051,P均0.05),而黄疸组AIH患者的肝肾功能指标、肝组织炎症分级和纤维化分期、胆管反应发生率、肝小叶中央区坏死程度明显高于非黄疸组(χ2=12.504、0.026、0.009,Z=-2.213,P均0.05)。对本次试验中接受免疫抑制剂的AIH患者分别进行随访,达到生物化学缓解的患者有43例,用Kaplan-Meier法对生物化学缓解率进行分析,黄疸组患者的累积生物化学缓解率为80.5%,中位缓解时间为25个月(95%CI:22.921~29.349);非黄疸组患者的累积生物化学缓解率为93.2%,中位缓解时间为8个月(95%CI:4.150~9.940),差异具有统计学意义(χ2=7.139,P=0.008)。结论以黄疸为首发症状的急性AIH患者发生率较低,但肝组织损伤较为严重,治疗后缓解率较低。     

自身免疫性肝炎和原发性胆汁性肝硬化患者检测可溶性细胞间黏附分子1的临床价值

目的探讨原发性胆汁性肝硬化(PBC)和自身免疫性肝炎(AIH)患者中检测可溶性细胞间黏附分子1(sICAM-1)的临床价值。方法选择2012年6月-2013年9月住院及门诊就诊的PBC患者61例,其中初发患者29例,缓解期患者21例,复发患者11例;AIH患者共59例,其中初发患者26例,缓解期患者20例,复发患者13例。健康对照50例。血清sICAM-1测定方法为双抗体夹心酶联免疫吸附法,采用生物化学酶法测定血清ALT、TBil。各组间比较采用方差分析,相关性采用Pearson相关分析。结果PBC患者中,初发组和复发组sICAM-1水平均明显高于缓解组和对照组(P均=0.000);初发组和复发组之间sICAM-1水平差异无统计学意义(P=0.484);缓解组sICAM-1水平高于对照组(P=0.000);AIH患者中,初发组和复发组sICAM-1水平均明显高于缓解组和对照组(P均=0.000);初发组和复发组之间sICAM-1水平差异无统计学意义(P=0.802);缓解组和对照组之间sICAM-1水平差异无统计学意义(P=0.281);PBC、AIH患者血清sICAM-1与ALT呈正相关(r=0.664,P=0.000;r=0.784,P=0.000);PBC、AIH患者血清sICAM-1与TBil呈正相关(r=0.715,P=0.000;r=0.580,P=0.000)。结论 sICAM-1可能参与PBC、AIH的免疫损伤机制,PBC、AIH患者血清sICAM-1水平升高程度与肝损害严重程度有密切关系,临床上动态观察其血清水平的变化,可望在病情活动评估、判断预后、指导治疗中起重要作用。     

自身抗体阴性自身免疫性肝炎患者临床病理学特点

目的:分析自身抗体阴性自身免疫性肝炎(AIH)患者临床特点,并总结诊疗经验。方法:对167例AIH患者的临床和病理特点进行分析,将自身抗体阴性AIH患者定义为:符合国际自身免疫性肝炎小组(IAIHG)1999年描述性诊断标准且抗核抗体、抗平滑肌抗体、抗肝肾微粒体1型抗体和抗线粒体抗体均为阴性者。结果:167例中17例自身抗体阴性,占10.2%。自身抗体阳性和自身抗体阴性AIH患者在一般情况(诊断时年龄除外)、生化指标方面均无明显差异(P0.05)。自身抗体阴性组血清IgG水平低于自身抗体阳性组,差异有统计学意义(P=0.004)。两组肝组织学炎症分级无明显差异,而自身抗体阴性组中存在更多纤维化进展期患者(P0.001)。采用1999年诊断积分系统评估时,自身抗体阴性组中11例(64.7%)为可能性诊断,其余6例(35.3%)为确定性诊断;采用简化诊断积分系统重新评估后,仅3例(17.6%)达到可能性诊断,且无1例达到确定性诊断标准。与自身抗体阳性组相比,自身抗体阴性AIH患者接受24个月的免疫抑制治疗后累积完全生化缓解率为86%,两组患者无明显差异(P=0.658)。结论:自身抗体阴性AIH在国人中并非罕见,在应用免疫抑制治疗方面与自身抗体阳性AIH患者类似,能取得满意疗效。     

成年女性急性心肌炎的临床特点分析

目的:探讨成人女性急性心肌炎的临床特点,为临床诊治提供参考。方法:回顾性分析北京安贞医院和拉萨市人民医院2010年1月至2015年12月,诊断为急性心肌炎的年龄超过18周岁的患者共108例,分为两组,一组为女性急性心肌炎组,另一组为男性急性心肌炎组,比较两组的临床特点和转归等。结果:女性急性心肌炎共有35例(32.4%),男性共有73例(67.6%),女性与男性比例为1∶2.1。女性平均年龄(31.5±12.6)岁,男性平均年龄(26.3±8.4)岁,两组间差异有统计学意义(P=0.011)。女性急性心肌炎患者入院时收缩压[(101±19)vs.(114±16)mm Hg,1mm Hg=0.133k Pa,P=0.000]和舒张压为[(64±11)vs.(72±12)mm Hg,P=0.001]与男性组相比差异有统计学意义。女性表现为急性暴发性心肌炎的患者有15例(42.9%),男性有13例(17.8%),两组相比差异有统计学意义(P=0.009)。女性急性心肌炎患者使用有创呼吸机、持续肾脏替代治疗和ECMO辅助比例明显比男性增多,两组差异均有统计学意义。住院期间并发心源性休克在女性急性心肌炎组为8例(22.9%),男性急性心肌炎组为2例(2.7%),两组间差异有统计学意义(P=0.002)。住院期间死亡女性急性心肌炎组为4例(11.4%),男性急性心肌炎组为2例(2.7%),两组间差异有统计学意义(P=0.037)。女性急性急性心肌炎组住院时间为13.0(5.0,17.0)d,男性急性心肌炎组住院时间为9.0(7.0,12.5)d,两组间差异有统计学意义(P=0.036)结论:成人女性与男性相比不易感染急性心肌炎。女性急性心肌炎起病急,病情重,住院期间心源性休克和死亡的发生率较男性相对较高,心脏机械辅助有效,预后良好。     

阿德福韦酯联合安络化纤丸治疗慢性乙型肝炎的疗效

目的 观察阿德福韦酯(ADV)联合安络化纤丸治疗慢性乙型肝炎患者的临床疗效.方法 治疗组36例采用ADV联合安络化纤丸治疗,对照组36例单用ADV治疗,治疗48周后,观察治疗前后的肝功能、肝纤维化指标、HBV DNA及肝组织学.统计学分析采用t检验或x2检验.结果 治疗48周后,治疗组和对照组患者在肝功能、血清纤维化标志物及肝脏组织学均有改善,治疗后两组患者在ALT、AST、总胆红素、白蛋白、肝组织炎症活动度积分和HBV DNA阴转率方面比较,差异无统计学意义.治疗组和对照组患者治疗后血清肝纤维化标志物透明质酸(HA)值分别为(101.58±30.11)μg/L、(182.25±117.59)μg/L;层黏连蛋白(LN)值分别为(147.89±41.72)μg/L、(181.50±56.96)μg/L;Ⅳ型胶原蛋白(CⅣ)值分别为(38.75±9.50)μg/L、(74.92±31.14)μg/L,治疗组患者的HA、LN、CIV值与对照组比较,t值分别为3.987、2.856、6.666,P值均＜0.05,差异均有统计学意义.治疗组治疗前后肝组织纤维化积分分别为12.28±3.16、10.61±2.37,治疗前后比较,t=2.532,P＜0.05,差异有统计学意义;对照组治疗前后肝组织纤维化积分分别为12.17±3.01、11.36±2.93,治疗前后比较,t=1.151,P＞0.05,差异无统计学意义.结论 ADV联合安络化纤丸治疗慢性乙型肝炎患者具有良好的抗肝纤维化作用,患者获得明显肝组织学改善.     

Toll样受体7基因拷贝数变异与汉族人系统性红斑狼疮的相关性研究

目的 探讨Toll样受体7(TLR7)基因拷贝数变异和汉族人系统性红斑狼疮(SLE)的发病及临床表型的相关性.方法 用AccuCopyTM多重基因拷贝数检测方法在337例汉族SLE患者及338名健康对照中测定TLR7基因拷贝数.按临床表型分层,分为狼疮肾炎及非狼疮肾炎组,血液系统受累组及非血液系统受累组,抗Sm抗体阳性组及阴性组.采用非参数秩和检验进行统计学分析.结果 按性别分组,女性组和男性组的TLR7基因拷贝数在SLE组和对照组的分布差异均无统计学意义(Z=-1.175,-1.085;P=0.240,0.278).按临床表型分层分析,狼疮肾炎组和非狼疮肾炎组TLR7基因拷贝数在女性和男性组的分布差异无统计学意义(Z=-0.888,-0.460;P=0.375,0.646),狼疮血液系统受累和非血液系统受累组TLR7基因拷贝数在女性和男性组的分布差异无统计学意义(Z=-1.085,-0.340;P=0.278,0.733),抗Sm抗体阳性组和阴性组TLR7基因拷贝数在女性和男性组的分布差异无统计学意义(Z=-0.529,-0.158;P=0.597,0.874).结论 TLR7基因拷贝数变异与汉族SLE患者及其临床表型无关.     

慢性乙型肝炎合并自身免疫性肝炎的临床诊治分析

目的总结慢性乙型肝炎(CHB)合并自身免疫性肝炎(AIH)的临床诊断和治疗方法。方法回顾性分析2013年12月至2018年6月上海交通大学医学院附属仁济医院消化内科门诊随访的19例诊断为CHB合并AIH患者的临床表现、实验室检查、影像学、病理组织学特点、治疗及转归情况,正态分布计量资料治疗前后比较采用配对样本t检验;不符合正态分布的计量资料以中位数(四分位数间距)表示,治疗前后比较采用配对样本的Wilcoxon符号秩和检验。结果19例患者中男性5例、女性14例,发病年龄35~63(47.10±8.76)岁。CHB先于AIH诊断有12例,AIH先于CHB诊断有5例,AIH和CHB同时诊断有2例。明确诊断CHB合并AIH后,予以核苷(酸)类似物抗乙型肝炎病毒联合糖皮质激素治疗,并根据肝内炎症(炎症分级在G3及以上)及白细胞情况加用免疫抑制剂硫唑嘌呤或吗替麦考酚酯。治疗2周至16周不等(治疗中位时间6周),除1例刚诊断治疗随访中外,其余18例患者治疗前后生物化学指标、免疫球蛋白均明显下降,治疗前后指标差异均有统计学意义(P值均<0.05),HBV DNA均<20拷贝/ml。结论CHB合并AIH容易漏诊,需要结合临床特点、自身抗体、免疫球蛋白水平,尤其重视肝组织病理学特点进行综合诊断。对抗-HBc阳性使用免疫抑制剂治疗的患者,应加强对HBV DNA的监测,必要时需抗乙型肝炎病毒治疗。     

血流储备分数指导女性冠状动脉临界病变的治疗及预后研究

目的研究血流储备分数(FFR)指导女性冠状动脉临界病变的治疗及预后。方法纳入我院行冠状动脉造影为临界病变的患者270例,其中男性160例,女性110例,根据FFR值决定治疗方案,比较男性与女性临床资料特征、FFR值、PCI比例及随访期间主要不良心血管事件(MACE)发生率。结果女性患者年龄、合并高血压及高脂血症比例高于男性,既往心肌梗死、吸烟和饮酒史比例低于男性,差异有统计学意义(P<0.05,P<0.01)。女性FFR值明显高于男性,差异有统计学意义(0.85±0.08 vs 0.83±0.08,P=0.036)。男性与女性行PCI比例比较,差异无统计学意义(29.4%vs 24.5%,P=0.382)。完成临床随访患者262例中,男性156例,发生MACE 7例;女性106例,发生MACE 8例,男性与女性MACE发生率比较,差异无统计学意义(4.5%vs 7.5%,P=0.295)。Kaplan-Meier生存曲线显示,男性与女性生存率比较,差异无统计学意义(95.5%vs 92.5%,P=0.366)。结论临界病变的FFR值有性别差异,女性的FFR值较男性高;FFR指导临界病变治疗的预后无性别差异。     

自身免疫性肝炎和原发性胆汗性肝硬化患者检测可溶性细胞间黏附分子1的临床价值

目的：探讨原发性胆汁性肝硬化（PBC）和自身免疫性肝炎（AIH）患者中检测可溶性细胞间黏附分子1（sICAM-1）的临床价值。方法选择2012年6月-2013年9月住院及门诊就诊的PBC患者61例，其中初发患者29例，缓解期患者21例，复发患者11例；AIH患者共59例，其中初发患者26例，缓解期患者20例，复发患者13例。健康对照50例。血清sICAM-1测定方法为双抗体夹心酶联免疫吸附法，采用生物化学酶法测定血清ALT、TBil。各组间比较采用方差分析，相关性采用Pearson相关分析。结果 PBC患者中，初发组和复发组sICAM-1水平均明显高于缓解组和对照组（P均＝0．000）；初发组和复发组之间sICAM-1水平差异无统计学意义（P＝0．484）；缓解组sICAM-1水平高于对照组（P＝0．000）；AIH患者中，初发组和复发组sICAM-1水平均明显高于缓解组和对照组（P均＝0．000）；初发组和复发组之间sICAM-1水平差异无统计学意义（P＝0．802）；缓解组和对照组之间sICAM-1水平差异无统计学意义（P＝0．281）；PBC、AIH患者血清sICAM-1与ALT呈正相关（r＝0．664，P＝0．000；r＝0．784，P＝0．000）；PBC、AIH患者血清sICAM-1与TBil呈正相关（r＝0．715，P＝0．000；r＝0．580，P＝0．000）。结论 sICAM-1可能参与PBC、AIH的免疫损伤机制，PBC、AIH患者血清sICAM-1水平升高程度与肝损害严重程度有密切关系，临床上动态观察其血清水平的变化，可望在病情活动评估、判断预后、指导治疗中起重要作用。     

45岁以下行冠状动脉介入治疗女性冠心病患者的临床特点

目的 对照评价45岁以下行冠状动脉介入治疗的女性和男性冠心病患者的临床特点及近远期临床疗效.方法 选择2004年4月至2008年2月在阜外心血管病医院择期接受冠状动脉介入治疗的45岁以下所有未绝经的124例女性患者作为女性组,采取整群抽样的方法选择阜外心血管病医院2006至2007年所有择期行冠状动脉介入治疗的45岁以下男性患者430例作为对照(男性组).两组均在术后6个月行临床冠状动脉造影随访,所有患者均临床随访1年.结果 共入选124例女性患者160处病变,430例男性患者665处病变,两组皆完成随访.女性组的血脂异常、陈旧性心肌梗死及吸烟史比例均低于男性组(均P<0.01).左主干病变比例女性组高于男性组(11.2%比2.9%,P<0.01).左前降支比例女性组高于男性组(61.3%比46.9%P=0.016).右冠状动脉病变比例女性组低于男性组(15.6%比30.2%,P<0.01).C型病变比例女性组低于男性组(36.2%比48.9%,P=0.004).分叉病变比例女性组高于男性组(26.9%比11.1%,P<0.01).靶病变长度女性组短于男性组[(20.36±13.37)mm比(23.04±13.86)mm,P=0.027].住院期及1年随访期间,女性组与男性组主要不良心血管事件、血栓发生率及再狭窄率差异无统计学意义.结论 病变位于左主干、前降支和分叉病变的比例45岁以下女性冠心病患者高于45岁以下男性冠心病患者,但是男性患者的冠心病危险因素较多、冠状动脉病变较严重.45岁以下男、女冠心病患者冠状动脉介入治疗的近远期疗效相似.     

Frequency and predictive factors for overlap syndrome between autoimmune hepatitis and primary cholestatic liver disease

OBJECTIVES: To evaluate the frequency of cholestatic pattern in patients with autoimmune hepatitis (AIH) and to identify predictive factors associated with the development of the overlap syndrome. METHODS: Eighty-two consecutive patients diagnosed with AIH at the referral centre between January 1998 and June 2002 were included in the study. The new scoring system modified by the International Autoimmune Hepatitis Group was used to classify patients as definite/probable. Overlap syndrome was considered when the patient had clinical, serological and histological characteristics of two conditions: AIH and primary biliary cirrhosis (PBC) or AIH and primary sclerosing cholangitis (PSC). RESULTS: From the 82 AIH patients (76 female and six male), 84.1% presented definite AIH (> 15 points) and 15.9% probable AIH (10 - 15 points). The frequency of the overlap syndrome was 20%: 13% with PBC and 7% with PSC. In the univariate analysis the overlap syndrome was associated with male gender (P = 0.01), age < 35 years (P < 0.0001), histopathological aspect of cholestasis (P < 0.0001), suboptimal response to treatment (P < 0.0001) and probable AIH (P < 0.0001). Age < 35 years, probable AIH and the absence of anti-nuclear antibody (ANA) have been identified as independent indicators of the overlap diagnosis by the logistic regression analysis. CONCLUSION: Patients with overlap syndrome between AIH and primary cholestatic liver disease are frequently diagnosed in clinical practice, representing 20% of AIH cases in our study. The independent predictive factors associated with the diagnosis of overlap syndrome are young age, ANA(-) profile, and probable diagnosis according with the scoring system for AIH.     

Budesonide induces complete remission in autoimmune hepatitis

AIM: Prednisone and azathioprine represent the standard treatment for autoimmune hepatitis (AIH). However, only 65% of the patients enter complete histological remission. Recently, budesonide (BUD) was reported to be a promising alternative. In this study we assessed the efficacy and safety of BUD in AIH. METHODS: Eighteen patients (12 women, 6 men; mean age 45.4±21 years) with AIH were treated with BUD (Budenofalk(?)) 3 mg thrice daily and followed up for at least 24 wk. Seven patients also had features of primary biliary cirrhosis (n=5) or primary sclerosing cholangitis (n=2). Advanced liver fibrosis or cirrhosis was present in 6 patients. RESULTS: Fifteen (83%) patients had a complete clinical and biochemical remission. Ten patients, including five with acute hepatitis, were given BUD as first-line therapy, of which seven enter remission. Three patients, two with liver cirrhosis, did not improve. All patients with second-line therapy experienced long-term remission. A histological remission was also seen in three patients. Clinically relevant BUD-induced side effects were recorded only in patients with liver cirrhosis (n=4). CONCLUSION: BUD is effective in remission induction in the majority of our patients with AIH. Side effects and treatment failure was mainly observed in patients with liver cirrhosis.     

Diagnostic difficulties,therapeutic strategies,and performance of scoring systems in patients with autoimmune hepatitis and concurrent hepatitis B/C

Abstract

Background. The diagnosis of autoimmune hepatitis (AIH) is already difficult, and that of AIH with chronic viral hepatitis including hepatitis B (HBV) or hepatitis C (HCV) is even more challenging. To date, only a few case-based studies have described this association. Aim. The aim was to retrospectively assess diagnostic difficulties, therapeutic approaches, and performance of the scoring systems in AIH patients with concurrent HBV and HCV. Methods. A total of 25 patients from United States, Sweden, Italy, and Turkey were retrospectively evaluated. Both revised and simplified criteria suggested by the International Autoimmune Hepatitis Group were applied for each patient. All study data were obtained from medical records. Results. Of the 25 patients, 20 (80%) had concomitant HCV and 5 (20%) had HBV. Based on the revised scoring system and simplified criteria, 18 (72%) and 12 (48%) patients were diagnosed as “probable” AIH. None of the patients were diagnosed as “definite” AIH according to both scoring systems. Patients with HCV initially were treated with immunosuppressive agents, and antiviral therapy was commenced when biochemical remission occurred. AIH patients with HBV were first treated with antiviral and thereafter, immunosuppressive therapy was started. Conclusions. This large case series describes concurrent AIH and chronic viral hepatitis. The revised scoring system for AIH had a better performance than the simplified scoring system. However, neither scoring system is optimal for diagnosing AIH alone. In these patients, a definitive diagnosis of AIH should be based on a combination of serological profiles, histological findings, scoring systems, treatment response, and outcomes.     

Clinical usefulness of ursodeoxycholic acid for Japanese patients with autoimmune hepatitis

AIM To evaluate the therapeutic effects of ursodeoxycholic acid(UDCA) on autoimmune hepatitis(AIH).METHODS A total 136 patients who were diagnosed with AIH were included in our study. All of the patients underwent a liver biopsy, and had at least a probable diagnosis on the basis of either the revised scoring system or the simplified scores. Initial treatment included UDCA monotherapy(Group U, n = 48) and prednisolone(PSL) monotherapy(Group P, n = 88). Group U was further classified into two subgroups according to the effect of UDCA: Patients who had achieved remission induction with UDCA monotherapy and showed no sign of relapse(Subgroup U1, n = 34) and patients who additionally received PSL during follow-up(Subgroup U2, n = 14). We compared the clinical and histological findings between each groups, and investigated factorscontributing to the response to UDCA monotherapy.RESULTS In Group U, 34 patients(71%) achieved and maintained remission over 49(range: 8-90) mo(Subgroup U1) and 14 patients(29%) additionally received PSL(Subgroup U2) during follow-up. Two patients in Subgroup U2 achieved remission induction once but additionally required PSL administration because of relapse(15 and 35 mo after the start of treatment). The remaining 12 patients in Subgroup U2 failed to achieve remission induction during follow-up, and PSL was added during 7(range: 2-18) mo. Compared with Subgroup U2, Subgroup U1 had significantly lower alanine aminotransferase(ALT) levels at onset(124 IU/L vs 262 IU/L, P = 0.023) and a significantly higher proportion of patients with mild inflammation(A1) on histological examination(70.6% vs 35.7%, P = 0.025). When multivariate analysis was performed to identify factors contributing to the response to UDCA monotherapy, only a serum ALT level of 200 IU/L or lower was found to be associated with a significant difference(P = 0.013).CONCLUSION To prevent adverse events related to corticosteroids, UDCA monotherapy for AIH needs to be considered in patients with a serum ALT level of 200 IU/L or lower.     

Primary biliary cirrhosis - Autoimmune hepatitis overlap syndrome: A rationale for corticosteroids use based on a nation-wide retrospective study in Japan

Aims: Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) may simultaneously coexist in some patients, designated as PBC‐AIH overlap syndrome. Previous studies suggest that combination therapy of ursodeoxycholic acid (UDCA) and corticosteroids may be effective. In the current study, we aimed to describe clinical features of these cases and to propose a rationale for combination treatment in PBC‐AIH overlap. Methods: We enrolled patients with PBC‐AIH overlap from eight referral centers for liver diseases in Japan, and clinical, biochemical and immunological features were examined. Liver histology of all patients at diagnosis were analyzed altogether in detail. Eighty‐nine and 44 patients with PBC and AIH alone were included and served as controls. Results: We identified 33 patients with PBC‐AIH overlap. The mean follow‐up period was 6.1 years. On liver histology, the HA (hepatitis activity) score was significantly higher than the CA (cholangitis activity) score (P < 0.001). At the end of the follow‐up period, corticosteroids were used in 23 patients (72%), and neither liver‐related death nor liver transplantation had been noted. The sensitivity and specificity of the simplified AIH scoring system for prediction of patients who required corticosteroids during clinical course was 92% and 75% in the training set (n = 17), and 91% and 80% in the validation set (n = 16) of overlap. Only 3% of PBC patients were diagnosed as having indication for corticosteroid use. Conclusion: In PBC‐AIH overlap, AIH‐like features are dominant in liver histology. The simplified AIH scoring system could predict patients who needed corticosteroids with a higher specificity.     

Role of international criteria in the diagnosis of autoimmune hepatitis

AIM: To study the clinical and laboratory characteristics of autoimmune hepatitis (AIH), and compare them with International Autoimmune Hepatitis Group (IAHG) criteria. METHODS: Sixty consecutive patients with AIH attended the University Clinic at Tabriz University of Medical Sciences, Iran for a 12mo period and were assessed in a case series study. Serological and biochemical evaluations were carried out in all patients. Autoantibodies, such as antinuclear antibody (ANA), anti-smooth muscle antibody (ASMA), anti-liver-kidney microsomal antibody (ALKM-1) type 1, and perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA) were evaluated in these patients. A liver biopsy was performed after diagnosis of the disease. Patients were evaluated in terms of their signs and symptoms, and laboratory results and the degree to which they corresponded with the diagnostic criteria of IAHG. In this study, both a comprehensive diagnostic scoring system and a simplified diagnostic scoring system were employed for AIH.RESULTS: Sixty patients, 2-0 male, 40 female, mean age 39.45 ± 17.50 years, participated in the study. Treatment began immediately after enrolment into the study. The percent distribution of the study population into definite and probable did not change after the treatment. The most common symptoms in descending order were fatigue (100%), icter (66.7%), abdominal discomfort (33.3%), abdominal distension (2-8.3%), dark urine (2-3.3%), edema (2-3.3%), hematemesis (2-0.0%), pruritus (2-0.0%), melena (11.7%) and pale stool (10.0%). At the physical examination, splenomegaly, ascites, hepatomegaly, epigastric tenderness and an abdominal mass were found in 50.0%, 16.7%, 13.3%, 5.0% and 3.3% of patients, respectively. Hypergammaglobulinemia was detected in 95.0% of cases. ALKM-1, P-ANCA, ANA and ASMA were positive in 71.4%, 66.7%, 42-.4% and 19.4% of cases, respectively. Portal hypertensive gastropathy (45.0%), esophageal varices (41.7%) and cirrhosis (40.0%) were the most prevalent complications of AIH, and there was     

Autoimmune acute liver failure: an emerging etiology for intractable acute liver failure

Diagnosis of acute onset autoimmune hepatitis (AIH) is the most challenging task because of atypical clinicopathological features. We examined the nature of acute onset AIH consisting of nonsevere, severe, and fulminant AIH based on our published data and other published papers, and propose how to diagnose and treat this intractable hepatitis. We analyzed clinical, biochemical, immunological, radiological, and histological features of acute onset AIH. Thirty percent of fulminant hepatitis was due to AIH and autoimmune acute liver failure (ALF) was not rare. The important characteristic of acute onset AIH is its histological, radiological, and clinical heterogeneity. Sometimes acute onset AIH develops into ALF in a sub-acute clinical course without appropriate diagnosis and treatment, and becomes resistant to immunosuppressive therapy and has poor prognosis. Unenhanced computed tomography (CT) often shows heterogeneous hypoattenuation in autoimmune ALF. The revised original scoring system (1999) performed better in patients with acute onset AIH than the simplified scoring system (2008). Liver regeneration from periportal progenitor cells to mature hepatocytes was impaired in ALF, resulting in resistance to immunosuppressive therapy. Precise histological evaluation (the presence of centrilobular necrosis/collapse) along with the revised original scoring system and CT findings of heterogeneous hypoattenuation after systematic exclusion of other causes 36 plays an important role in the diagnosis. The most important strategy for autoimmune ALF is to diagnose and treat acute onset AIH before its development into ALF. Liver transplantation should be considered before the occurrence of infectious complications in the case of fulminant liver failure.     

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: evaluating the role of routine magnetic resonance imaging

Large bile duct injury (that seen on cholangiography) is not usually considered a feature of autoimmune hepatitis (AIH) in adults but is present in up to 50% of children with AIH. The aim of this work was to study the prevalence of large bile duct abnormalities identified by magnetic resonance cholangiography (MRC) in adults given a diagnosis of AIH. Seventy-nine (n = 79) patients given a diagnosis of AIH (mean AIH score: 15.1 +/- 3.4) were screened with MRC for evidence of sclerosing cholangitis (SC). Results were reviewed by two radiologists. Clinical parameters were correlated with MRC findings. A histological review of available liver biopsies (n = 29) was performed. Of the 79 patients surveyed, 8 (10%) had MRC findings consistent with primary sclerosing cholangitis (PSC). The interrater variability was excellent (kappa = 0.87). Younger age at diagnosis (24.3 +/- 11.9), higher baseline alkaline phosphatase (186.4 +/- 98.3), higher bilirubin at time of MRC (45.8 +/- 37.2), and greater lobular activity on initial liver biopsy were significantly associated with the detection of this overlap of SC with AIH (P = 0.024, P = 0.037, P = 0.032, and P = 0.041, respectively), but not alkaline phosphatase/aspartate aminotransferase ratio, time between the initial diagnosis of AIH and the MRC, or the presence of cirrhosis on initial liver histology. Two cases with a normal MRC had histological lesions typical of small duct PSC. CONCLUSION: The presence of SC detected by MRC and from liver histology in adult patients with AIH may not be clinically overt, and thus the prevalence of this AIH/SC overlap may be higher than previously recognized. Our data suggest that routine radiological evaluation of the biliary tree should be performed in adults given a diagnosis of AIH, as in children the presence of this overlap negatively impacts on survival.