自身免疫性肝病重叠综合征的诊断与治疗

近年来,自身免疫性肝病及其重叠综合征受到越来越多的关注。由于重叠综合征的发病率低,难以进行大规模的随机对照研究,导致该类疾病的诊断以及治疗方案没有统一标准,本文就该病的诊断及治疗做一综述     

自身免疫性肝病重叠综合征的现状

自身免疫性肝病(autoimmune liver disease,A I L D)包括自身免疫性肝炎(a u t o i m m u n e hepatitis,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).AILD重叠综合征(overlap syndrome,OS)是指患者同时兼具AIH和PBC或PSC两种疾病的临床表现、生物化学、血清学、组织学以及影像学特征的一种罕见疾病状态.AILD OS主要分为AIH-PBC OS和AIH-PSC OS.前者多见于成人,后者多见于儿童.OS若无治疗,最终可致肝硬化或肝衰竭.高剂量熊去氧胆酸(ursodeoxycholic acid)与免疫抑制剂类固醇和/或硫唑嘌呤(azathioprine)联合使用通常被用于治疗AIH-PBC OS和AIH-PSC OS.目前,肝移植仍是治疗终末期OS患者的唯一有效方法.     

自身免疫性肝病重叠综合征的诊断和治疗

自身免疫性肝病（AILD）是一组以肝脏病理损害和肝功能异常为主要表现的自身免疫性疾病,可分为自身免疫性肝炎（AIH）、原发性胆汁性肝硬化（PBC）和原发性硬化性胆管炎（PSC）,重叠综合征指同时具有其中两种疾病的临床和病理表现。重叠综合征相对少见,主要包括AIH—PBC和AIH—PSC。由于重叠综合征在临床表现、血清学和组织学方面综合了两种AILD的特点,其诊断和治疗有一定难度并存在争议。对其临床表现以及诊断和治疗方案进行深入研究有助于对该病的认识和防治。     

自身免疫性肝病与其重叠综合征

自身免疫性肝病(autoimmune liver disease,AILD)是一组由异常自身免疫介导的肝、胆损伤性疾病。根据其临床表现、生化、免疫学、影像学和组织病理学特点,可分为以肝炎为主型,即自身免疫性肝炎(AIH)和以胆系损害及胆汁     

自身免疫性肝病重叠综合征的诊断和治疗

自身免疫性肝病重叠综合征是肝脏自身免疫性疾病的一种,肝硬化相关并发症发生率高、预后差。本文从流行病学、临床特点、诊断、治疗和预后等方面阐述了自身免疫性肝病重叠综合征的最新研究进展,重点总结了如何诊断并使用有效的方案治疗不同的重叠综合征,以期提高临床工作者对该类疾病的认识,及早的诊断和治疗该疾病。     

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.     

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。     

自身免疫性肝病重叠综合征的诊断及治疗现状

自身免疫性肝病重叠综合征是一种特殊类型的自身免疫性疾病,其病因机制及诊疗尚无统一意见。回顾了重叠综合征的病因、发病机理、诊断及诊疗的研究发展进程,分析了本病的定义、诊断及治疗近况。指出本病的发病机制目前仍未完全阐明,诊疗规范仍需进一步探讨。此外,分析表明重叠综合征的表现形式多种多样,如何早期诊断及鉴别并合理的治疗仍是目前研究的重点。     

原发性干燥综合征的肝损害与自身免疫性肝病

原发性干燥综合征 (ｐＳＳ)是一系统性自身免疫性疾病 ,除口、眼腺体受累外 ,内脏器官如肾、肺、肝、胰等也可受到侵犯 ,尤其是肝损害越来越受到重视 ,它与自身免疫性肝病之间的关系已成为探讨的热点。ｐＳＳ肝损害并不少见 ,有时甚至十分严重 ,危及生命。因此 ,早期发现、及时治疗对遏制肝损害的进一步发展及改善预后有重要意义。一、ｐＳＳ肝损害的原因、发生机制及临床表现多种原因可以导致ｐＳＳ患者出现肝损害 ,如各型肝炎病毒感染、长期饮酒、服用药物等。ｐＳＳ患者长期服用非甾体抗炎药和慢作用抗风湿药 ,这些药物有一定的肝毒性 ,…     

自身免疫性肝病的诊断和治疗现状

自身免疫性肝病(autoimmune liver diseases,AILD)是一组由异常自身免疫介导的肝、胆损伤,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     

Overlap syndromes with autoimmune hepatitis in chronic cholestatic liver diseases

Conditions exhibiting features of two different autoimmune liver diseases are commonly designated overlap syndromes, although there is no current agreement on what constitutes an overlap syndrome or specific diagnostic criteria. As in the classic autoimmune liver diseases, such as autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), the etiology is unknown but presumed to be related to alterations of immune regulation. Distinction of these clinical entities is important for management as outcomes may differ from outcomes of patients with diagnosis of classic autoimmune liver diseases. Due to their presumed rarity, no large therapeutic trials are available and treatment of overlap conditions is empirical and based upon extrapolation of data from the primary autoimmune liver diseases. PBC–AIH overlap is the most frequently described overlap syndrome and may be associated with a poor prognosis. This may represent an important and unrecognized cause of resistance to ursodeoxycholic acid in patients with PBC. PSC–AIH overlap is less commonly reported. Prognosis may be better than in patients with PSC alone; however, worse than in patients with AIH alone. Further studies are needed for determining diagnosis, natural history and optimal therapeutic strategies of overlap syndromes of autoimmune liver disease.     

A case of coexisting primary biliary cirrhosis and primary sclerosing cholangitis: a new overlap of autoimmune liver diseases

Although the etiology of AIH, PBC, and PSC remains unknown, it is apparent that these autoimmune liver diseases share many common features and can coexist in the same patient. Our patient had features of PBC and later clearly developed a picture of PSC. This case suggests that PBC, PSC, AIH, and autoimmune cholangitis are part of a spectrum of chronic autoimmune liver disease that develop in response to some yet unidentified antigen.     

Living-donor liver transplantation for autoimmune hepatitis and autoimmune hepatitis-primary biliary cirrhosis overlap syndrome

Aim: Recurrent autoimmune hepatitis (AIH) following liver transplantation has been reported in 20–30% of cases, mainly of Western populations. The aim of this study was to review our experience of living‐donor liver transplantation (LDLT) in Japanese patients with AIH. Methods: Among 375 adult (age ≥18 years) LDLT performed at our center between 1996 and 2010, 16 (4.2%) were for patients with AIH (n = 12) or AIH–primary biliary cirrhosis overlap syndrome (n = 4). The patient and donor characteristics and post‐transplantation course were reviewed. Results: All recipients were female with a median age of 48 years (range, 21–58). Low‐dose methylprednisolone and calcineurin inhibitors were continued in all patients. Acute cellular rejection occurred in 10 (63%), which was more frequent than in our overall series of 28.5% (107/375 cases). Overall survival rate was 81.2% at 5 years. At the end of the follow up (median, 6.0 years [range, 0.1–9.6]), 13 patients were alive with normal liver function tests (aspartate transaminase, 18 ± 5 IU/mL; alanine transaminase, 16 ± 8 IU/mL). None of the survivors exhibited liver function test results suspicious for recurrent AIH, which might indicate liver biopsy. Conclusion: Survival after LDLT for AIH and overlap syndrome was excellent and there was no evidence of clinical recurrence. The recurrence rate of AIH after liver transplantation may differ among countries, and requires further investigation.     

临床病理学在自身免疫性肝病诊治中的重要性

肝脏组织学在自身免疫性肝病的诊断中起着重要作用,尤其是临床表现非特异、自身抗体阴性的患者。介绍了几种自身免疫性肝病在组织学上的特征性表现。自身免疫性肝炎(AIH)的特征性表现包括界面性肝炎伴淋巴-浆细胞浸润、玫瑰花环及穿入现象,而慢性非化脓性胆管炎、上皮样肉芽肿常提示原发性胆汁性肝硬化(PBC)。胆管周围洋葱皮样纤维化是原发性硬化性胆管炎(PSC)特征性表现,席纹状纤维化伴IgG4阳性浆细胞浸润常须考虑为IgG4相关硬化性胆管炎。最后指出,在日常工作中,临床医生和病理医生加强沟通有利于提高自身免疫性肝病的诊治。     

自身免疫性肝病重叠综合征的临床治疗探讨

目的探讨自身免疫性肝病重叠综合征的治疗方法,提高治疗的有效性与安全性。方法 32例自身免疫性肝炎（AIH）-原发性胆汁性肝硬化（PBC）重叠综合征患者,均给予强的松（0.5mg.kg-1.d-1）联合熊去氧胆酸（UDCA,15 mg.kg-1.d-1）治疗,回顾性分析治疗前、后不同时段患者疾病状态的变化,评价临床疗效。结果所有患者症状、体征明显减轻,生化指标、肝脏病理损害均明显改善,与入院前比较差异有统计学意义（P〈0.01或P〈0.05）。IgG、IgM、抗平滑肌抗体（SMA）、抗线粒体抗体（AMA）治疗前后无明显变化,差异无统计学意义（P〉0.05）。结论强的松联合UDCA短期内能明显减轻AIH-PBC患者的临床症状、体征,改善生化学和肝组织学指标,提高患者生存质量,治疗安全有效。