AIH-PBC重叠综合征的诊断及治疗

自身免疫性肝病是一组与自身免疫异常有关的肝胆疾病,其包括自身免疫性肝炎（AIH）、原发性胆汁性肝硬化（PBC）和原发性硬化性胆管炎（PSC）以及这三种疾病中任两者之间的综合征。AIH-PBC重叠综合征（AIHPBCOS）在PBC患者中的发生率为2％～20％．其定义为一个患者同时具有这二种疾病的主要特征。     

自身免疫性肝病自身抗体研究进展

自身免疫性肝病（ALD）是一组免疫介导的肝脏损伤,根据其临床表现、生化、免疫学、影像学和组织病理学特点,可简单分为以肝炎为主型,即自身免疫性肝炎（AIH）和以胆系损害及胆汁淤积为主型,即原发性胆汁性肝硬化（PBC）、原发性硬化性胆管炎（PSC）。此外,还有这三种疾病中任意两者之间的重叠综合征（overlap syndromes）,主要以AIH—PBC重叠综合征多见。由于我国肝炎和肝硬化多因肝炎病毒,尤其是乙型肝炎病毒引起,故过去认为ALD较少见。     

原发性胆汁性肝硬化-自身免疫性肝炎重叠综合征临床与病理分析

目的总结原发性胆汁性肝硬化-自身免疫性肝炎重叠综合征患者的临床及病理组织学特点。方法对10例原发性胆汁性肝硬化一自身免疫性肝炎重叠综合征患者的临床及病理资料进行回顾性分析。结果本组患者均有肝功能酶谱的不同程度升高，血清IgG和IgM升高，4例血清抗线粒体抗体（antimitochondrial antibody-2，AMA-2）阳性，其中3例抗核抗体（antinuelear antibodY，ANA）阳性，1例核心蛋白gp210抗体（gp210）阳性。10例肝穿刺活检示均有小胆管损害，肝细胞炎症活动度从中度到重度，纤维化程度从S2到S4。结论自身免疫性肝病重叠综合征患者中女性多见。在临床及病理组织学上兼有原发性胆汁性肝硬化和自身免疫性肝炎的双重特点，临床工作中需结合临床、生物化学、免疫学及病理情况及时作出准确诊断。     

重叠综合征：国际自身免疫性肝炎小组（IAIHG）就争议问题的立场声明

自身免疫性肝病中,部分患者具有胆汁淤积性肝病[原发性胆汁性肝硬化（PBC）或原发性硬化性胆管炎（PSC）]和自身免疫性肝炎（AIH）两种疾病特征,这些患者通常诊断为＂重叠综合征＂,目前尚缺乏定义＂重叠＂的国际一致性标准。     

自身免疫性肝病自身抗体检测的临床意义

自身免疫性肝病(autoimmuneliverdisease,ALD)是指一组病因尚不明确,但普遍认为与自身免疫有关的肝脏疾病。通常包括自身免疫性肝炎(autoimmunehepatitis,AIH)、原发性胆汁性肝硬化(primarybiliarycirrhosis,PBC)及原发性硬化性胆管炎(primarysclerosingcholangi-tis,PSC)。AIH     

2012年自身免疫性肝脏疾病基础和临床进展

自身免疫性肝病是一组由自身免疫反应介导的慢性肝胆系统炎症性疾病,主要包括自身免疫性肝炎（AIH ）、原发性胆汁性肝硬化（PBC ）、原发性硬化性胆管炎（PSC ）。本文对于自身免疫性肝病的发病机制、诊断、治疗等方面的进展做一简要综述。     

原发性干燥综合征伴AMA阴性的原发性胆汁性肝硬化1例

原发性干燥综合征（PSS）是一种常见的结缔组织病,而原发性胆汁性肝硬化（PBC）是一种慢性肝内胆汁淤积性疾病,血清线粒体抗体M2（AMA—M2）诊断PBC的敏感性和特异性均超过9（1％～95％,为本病最突出的免疫学指标异常。     

原发性硬化性胆管炎合并自身免疫性胰腺炎一例

自身免疫性胰腺炎（autoimmune pancreatitis，AIP）是与自身免疫有关的慢性胰腺炎症，患者常有高γ球蛋白血症，偶伴有其他自身免疫性疾病如Sjogren综合征，原发性硬化性胆管炎，原发性胆汁性肝硬化等，国内有关病例报道甚少，国外亦不多见。我院于2005年3月收治一例原发性硬化性胆管炎合并自身免疫性胰腺炎患者，现报道如下。     

自身免疫性肝病重叠综合征的临床诊治进展

自身免疫性肝病是血清中出现相关的自身抗体和免疫球蛋白,并由此引起相应的肝脏病理损伤和肝功能生物化学异常的一组自身免疫性疾病,包括自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)和原发性硬化性胆管炎(primary sclerosing cholangitis,PSC).这3种疾病都有其各自典型的临床表现和血清生物化学、免疫学及病理学特征,然而,在许多情况下,患者的临床特征往往不那么典型,而表现为兼有以上3种中2种疾病的特征,我们称之为重叠综合征.     

自身免疫性肝炎-原发性胆汁性肝硬化重叠综合征的诊治

自身免疫性肝炎（AI H）和原发性胆汁性肝硬化（PBC）是影响肝脏的两种自身免疫性疾病,每种疾病均具有各自的临床表现,免疫性和组织学特征,然而,某些患者同时具有两种疾病的特点,这种在同一时段或病程中具备PBC和AI H两种疾病特征的综合征称为AI H-PBC重叠综合征（AI H-PBC OS）。     

Autoimmune liver diseases

The liver was one of the earliest recognized sites among autoimmune diseases yet autoimmune hepatitis,primary biliary cirrhosis,primary sclerosing cholangitis,and their overlap forms,are still problematic in diagnosis and causation.The contributions herein comprise ＇pairs of articles＇ on clinical characteristics,and concepts of etiopathogenesis,for each of the above diseases,together with childhood autoimmune liver disease,overlaps,interpretations of diagnostic serology,and liver transplantation.This issue is timely,since we are witnessing an ever increasing applicability of immunology to a wide variety of chronic diseases,hepatic and non-hepatic,in both developed and developing countries.The 11 invited expert review articles capture the changing features over recent years of the autoimmune liver diseases,the underlying immunomolecular mechanisms of development,the potent albeit still unexplained genetic influences,the expanding repertoire of immunoserological diagnostic markers,and the increasingly effective therapeutic possibilities.     

继发性嗜血细胞综合征15例临床分析

目的 探讨自身免疫病相关性与非自身免疫病相关性嗜血细胞综合征(HPS)两者的临床特征、治疗及预后.方法 回顾性收集15例2008年1月至2010年6月我院风湿科及血液科住院的继发性嗜血细胞综合征患者,分析临床及实验室特征、治疗以及预后的关系.并比较自身免疫病相关性嗜血细胞综合征(A组)与非自身免疫病相关性嗜血细胞综合征(B组)之间的异同点.采用Fisher精确概率法、t检验和秩和检验.结果 2组均表现出发热、出血、黄疸、肝脾肿大,A组特异性表现出关节痛、皮疹、自身抗体阳性,而B组中黄疸更常见(38%和100%,P=0.018).但比较2组实验室指标及预后差异均无统计学意义(P>0.05).激素联合免疫抑制剂和丙种球蛋白治疗预后更好(P<0.05).结论除黄疸外,自身免疫病相关性HPS与其他继发性HPS的临床表现及实验室指标无明显差异,激素联合免疫抑制剂及丙种球蛋白治疗有效.

Abstract：

Objecfive To investigate the clinical characteristics,treatment and prognosis of autoimmune diseases associated and non-autoimmune diseases associated hemophagocytic syndrome.Methotis Clinical records of 15 cases witll secondary hemophagocytic syndrome'were collected and the relations with treatment and prognosis was analyze.The similarities and differences between autoimmune disease associated bemophagocytic syndrome (group A)and non-autoimmune disease associated hemophagocytic syndrome (group B)were compared.Fisher exact test,t test and Willcoxen test were used for statistical analysis.Results Both groups had fever,bleeding,jaundice,hepatosplenomegaly,and arthralgia,skin rash and positive of autoantibodies in group A were discovered specifically.But in group B,the patients with icterus were mo common(38% vs 100%,p=0.018).There was no significant difference in their laboratory data and prognosis when compared between the two groups(P>0.05).The patients who received corticosteroids and IVIG and/or immunosuppressive agents had better prognosis(P<0.05).Conclusion Except for icterus there is no significant difference in clinical features and laboratory data among autoimmune disease associated hemophagocytic syndrome and other secondary hemophagocytic syndrome.And the therapy with corticosteroids combined with IVIG and/or immunosupprcssive agents is effective.     

Undifferentiated connective tissue diseases (UCTD): a new frontier for rheumatology

Patients with signs and symptoms suggestive of a systemic autoimmune disease but not fulfilling the classification criteria for defined diseases are common in clinical practice. Such conditions have been defined as undifferentiated connective tissue diseases (UCTDs). Since the 1980s, many studies have analyzed different aspects of the UCTDs -- their frequency and epidemiological characteristics, the rate of evolution to defined CTD, and their clinical and serological characteristics. It is agreed that UCTDs represent around 60% of diseases with an undifferentiated onset, that they are systemic autoimmune diseases characterized by simplified clinical and serological profiles, and that they have a good prognosis. Although many aspects of these conditions have been studied and clarified, there is still no agreement on how best to identify UCTD patients after the onset of their disease. However, such identification is of paramount importance, and further analysis is necessary to improve the sensitivity and specificity of the proposed classification criteria.     

Thyroid dysfunction in primary biliary cirrhosis,primary sclerosing cholangitis and non‐alcoholic fatty liver disease

Background/Aims: Primary biliary cirrhosis (PBC) is frequently associated with autoimmune diseases, including thyroid disease, although it is uncertain that this association is higher than in other liver diseases. Methods: We compared the prevalence and incidence of thyroid dysfunction (TD) in a series of patients with PBC (n=67) with patients with primary sclerosing cholangitis (PSC) (n=79) and non‐alcoholic fatty liver disease (NAFLD) (n=97) seen in a tertiary referral centre who had previously participated in clinical trials. Results: At initial evaluation, prevalence of TD in PBC was 13% compared with 11% in PSC (P=0.71) and 25% in NAFLD (P=0.08). Incidence of TD was 2.9 patients per 100 person‐years in PBC compared with 2.1 patients per 100 person‐years in PSC (P=0.57) and 1.8 patients per 100 person‐years in non‐alcoholic liver disease (P=0.45). Older age, female gender, biochemical abnormalities and concurrent autoimmune disorders were not predictive of the development of TD. Conclusions: TD was unexpectedly as common in patients with PBC as in patients with PSC and NAFLD, yet significantly more common than expected in the general population. Further investigation of thyroid disease in PSC and NAFLD is warranted.     

Celiac disease in a rheumatology unit: a case study

The objective of the study is to present a series of 20 patients who have been attending a rheumatology unit and were diagnosed with celiac disease in adult life. The record-charts of 20 Italian not consanguineous patients affected by celiac disease (1 man and 19 women, mean age of 46.7), diagnosed at >16 years of age, followed by a rheumatology unit were reviewed (group 1). Any other autoimmune disease diagnosed in the patients were given was recorded; moreover, the reason for rheumatologist evaluation was registered as well as the presence of symptoms suggestive of celiac disease and the obstetric history. The clinical features were compared with those of a group of 40 celiac patients (8 men and 32 women, mean age of 43.1) followed by a medicine department (group 2); even in these cases the diagnosis of celiac disease was performed in adult life. Sixteen out of 20 patients in Group 1 were diagnosed as suffering from celiac disease by the rheumatologist. Seventeen concomitant autoimmune disorders among which nine were connective tissue diseases were found in 15 patients. The main reason for rheumatologist evaluation was arthromyalgias. Ten patients showed one or more clinical features suggestive of celiac disease; moreover, eight patients had a history of sideropenic anemia. After the adoption of a gluten-free diet there were three pregnancies that all ended with alive newborns, differently from the obstetric history before celiac disease diagnosis, characterized by a relevant number of miscarriages and foetus deaths. In Group 2, a total of ten autoimmune diseases concomitant with celiac disease were found in eight patients; autoimmune thyroid disorders represented the most frequent cases. No connective tissue diseases were recognized. Celiac disease may coexist with connective tissue diseases; the recognition of this association is difficult because celiac disease may present with atypical or even symptomless forms or in some cases may resemble a multisystem disorder or may mimic a rheumatologic condition; on the other hand, the variety of symptoms of rheumatic disorders may make difficult the diagnosis of celiac disease in association with a systemic autoimmune disease. These confounding factors often lead to a delay in performing the right diagnostic formulation.     

重视肝活组织病理检查在临床肝病诊疗中的应用

目的探讨超声引导下经皮肝穿刺活组织病理检查在临床肝病诊疗中的意义。方法回顾性分析了吉林大学第一医院157例超声引导下经皮肝穿刺活组织病理检查病例,所有病例均为肝损害经常规检查方法未能明确诊断或临床诊断分期不明确。所有患者经临床生化学检查及影像学检查均未提示肝硬化。分为I组(HBV感染组)和II组(非HBV感染组)。结果 157例病例中,I组81例,其中IA组(ALT<2×ULN)43例,IB组(ALT≥2×ULN)38例。II组76例,其中自身免疫性肝病30例,药物性肝损害21例,脂肪性变11例,罕见病例8例,隐源性肝炎6例。结论超声引导下经皮肝穿刺活组织检查安全性较高,肝活组织病理检查在不明原因的肝损害或临床诊断分期不明确的肝损害诊断中有非常重要的价值,可减少临床漏诊及误诊,有利于明确肝损害的病因及程度。     

Polyautoimmunity in rheumatological conditions

Co‐occurrence of autoimmune diseases (ADs) within an individual is postulated to be a frequent phenomenon in rheumatic diseases. Similar clinical signs and symptoms, pathophysiological mechanisms, genetic factors within autoimmune diseases and aggregation of diverse ADs within families sustain the theory of shared pathogenesis of several ADs (autoimmune tautology). Polyautoimmunity (PA) is defined as the presence of more than one autoimmune disease in a single patient. When three or more autoimmune diseases coexist, this condition is called multiple autoimmune syndrome (MAS). This analysis summarizes an estimated prevalence of PA in the most common rheumatic diseases, the presumable risk factors for PA and influence of concomitant diseases on the course of disease.     

弥漫性结缔组织病伴皮肤软组织结核六例临床分析并文献复习

目的 分析弥漫性结缔组织病(DCTD)伴皮肤软组织结核(CSTB)的临床特点.方法 对我院2003年7月至2008年10月收治的6例DCTD伴CSTB感染患者进行回顾性分析并复习相关文献.结果 6例患者在应用糖皮质激素和免疫抑制剂12～19个月内患CSTB.CSTB的发生部位分别为大腿、臀部、小腿、前臂、颈部和上臂.其中4例形成窦道同时合并普通细菌感染.从CSTB发病到确诊的时间为1～11个月.经抗结核治疗6个月后,1例痊愈,3例好转.2例未愈.结论 CSTB感染是DCTD伴结核感染的少见类型,容易误诊和延迟诊断.当DCTD在治疗过程中出现皮肤软组织的炎症表现时,应警惕结核感染,尽早完善结核的相关检查,必要时可试行诊断性抗结核治疗.     

Epidemiology of autoimmune liver disease

Primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are chronic liver diseases that likely have an autoimmune basis to their pathogenesis. Although significant strides have been made in the clinical management of these conditions, their pathogenesis remains obscure. Understanding of various epidemiological factors may shed light on predisposing or causative factors for these diseases. Most is known about the epidemiology of PBC, with only minimal information on that of PSC and AIH. In this review, the current data on the epidemiology of PBC, AIH and PSC are summarized and suggestions are made for future work in this important area.     

Clinical features of liver disturbance in rheumatoid diseases: clinicopathological study with special reference to the cause of liver disturbance

Background: Background: Liver disturbance in rheumatoid diseases results not only from liver disease associated with the rheumatoid diseases themselves but also from various other causes. This study aimed to elucidate the clinical features of liver disturbance in rheumatoid diseases, focusing on the cause of this disturbance. Methods: A clinicopathological study was performed in 306 patients (106 with systemic lupus erythematosus, 71 with Sj?gren's syndrome, 59 with rheumatoid arthritis, 27 with scleroderma, 30 with polymyositis, and 13 with polyarteritis nodosa). Results: Liver disturbance occurred in 43% of these patients and resulted from various causes. Its degree and duration varied from one cause to another. Liver disease associated with rheumatoid diseases was the leading cause of the liver disturbance in these patients and was characterized by mild and transient liver disturbance (maximum alanine aminotransferase [ALT] level during the study period, 68 ± 8 IU/ml; maximum alkaline phosphatase [ALP] level, 410 ± 31 IU/ml; duration of liver disturbance, 6 ± 2 months). Most patients with this type of liver disease showed minimal change in liver histology, although two-thirds of those evaluated by the international scoring system for autoimmune hepatitis (AIH) were classified as “probable” or “definite”. Eight of 14 patients with histologically proven chronic hepatitis or cirrhosis were infected with hepatotropic virus (7 with hepatitis C virus [HCV] and 1 with hepatitis B virus [HBV]). Five of 9 patients in whom the hepatic lesion progressed had hepatotropic virus infection (4 with HCV and 1 with HBV), and the other 4 patients suffered from autoimmune liver diseases. Conclusions: Liver disease associated with rheumatoid diseases was the leading cause of liver disturbance in these patients and was characterized by mild and transient liver disturbance, whereas progressive liver diseases were often associated with hepatotropic virus, mainly HCV, or autoimmune liver diseases. Liver histology is indispensable for differentiating AIH from liver disease associated with rheumatoid diseases. Received: August 27, 2001 / Accepted: January 7, 2002