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自身免疫性肝病指南解读与临床实践
$52.25
● 本书的亮点是:1.内容实用,全书对自身免疫性肝病的诊治指南进行了详细解读,并配以彩色图表以进一步精细讲解,图文并茂,提高阅读者的理解力,方便读者学习和使用。● 本书彩色印刷,内容价值和写作水平都上乘,作者为复旦大学附属中山医院专家团队。
本书约30万字(含图表60幅),不同的自身免疫性肝病其临床生化表现、血清自身抗体谱、组织病理特征、有效治疗药物又有所不同,本书介绍各种自身免疫性肝病的国内外诊治指南的共识意见,并结合临床实例阐述其规范化诊疗方法,对该类疾病的诊疗具有指导意义。
主创团队为复旦大学附属中山医院的专家、教授,主编郭津生,主任医师,硕士研究生导师,上海市中西医结合器官纤维化学组顾问委员,美国西奈山肝病研究中心访问学者。荣获中山医院十大优秀青年、复旦大学世纪之星等荣誉。从事肝病临床医教研工作24年。
篇 自身免疫性肝炎的诊断与治疗章 自身免疫性肝炎指南解读2第二章 自身免疫性肝炎病例37第二篇 原发性胆汁性肝硬化的诊断与治疗章 原发性胆汁性肝硬化指南解读52第二章 原发性胆汁性肝硬化病例74第三篇 原发性硬化性胆管炎的诊断与治疗章 原发性硬化性胆管炎指南解读108第二章 原发性硬化性胆管炎病例128第四篇 IgG4相关硬化性胆管炎的诊断与治疗章 IgG4相关硬化性胆管炎指南解读140第二章 IgG4相关硬化性胆管炎病例170第五篇 胆汁淤积性肝病的诊断与治疗附录一 临床实践指南的产生和应用215附录二 临床检测正常参考值217 附录三 英文缩略词表220
自身免疫性肝病(autoimmune liver diseases,AILD)是一类病因不明确,但具有自身免疫基础的非化脓性炎症性肝病。AILD 表现为机体对自身肝组织失去免疫耐受,肝脏出现病理性炎症性损伤,同时血清中出现与肝病有关的循环自身抗体。AILD 根据主要受累的肝细胞类型不同可分为两大类:肝细胞受累的自身免疫性肝炎(autoimmune hepatitis,AIH)、胆管细胞受累的自身免疫性胆管病。后者有胆汁淤积的表现,包括原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)、原发性硬化性胆管炎(primary sclerosing cholangitis, PSC)和 IgG4 相关硬化性胆管炎(IgG4 relatedsclerosing cholangitis,IgG4-SC)。这些疾病有时又以兼具2种损伤特征的重叠综合征形式出现,如AIH-PBC重叠综合征;或在疾病发展的不同阶段序贯表现出不同细胞类型的损伤。AILD易患人群也有一定的分布规律。如PBC主要发生在40~60岁的中年女性,女性与男性比约为9∶1;PSC主要累及年轻人,平均诊断年龄40岁,70%的患者是男性,10%~30%的患者会发展成胆管癌。 近 30年来,国内外关于自身免疫性肝病(AILD)的报告均显著增加,究其原因,除发病率可能升高外,人们对这类疾病的认识的提高、检测方法的不断普及等因素也起到重要作用。如体检项目中纳入对胆汁淤积为敏感的谷氨酰转肽酶和为特异的生化指标碱性磷酸酶检查可识别和筛选出生化检查异常的可疑 PBC 患者。对早期未发生显著肝纤维化和肝硬化的患者,进一步作特异性自身抗体如抗线粒体M2抗体(AMA-M2)的筛查和(或)肝穿刺病理检查(非化脓性小胆管炎)可获得PBC的诊断;而对氨基转移酶(转氨酶)升高为主的肝损伤,患者通过血清自身抗体、免疫球蛋白 IgG 的检查和(或)病理检查(界面肝炎),同时结合对其他肝病的排查,通过AIH诊断积分标准可获得 AIH的诊断。IgG4-SC常累及肝外胆管,常伴发自身免疫性胰腺炎以及其他纤维化性疾病、血清IgG4水平升高和胆管内及肝组织IgG4阳性浆细胞浸润是其特征性表现。与所有其他慢性肝病一样,AILD 如不及时发现和治疗终可进展为肝硬化和肝衰竭,是肝移植的主要适应证之一,而在纤维化发生早期就明确诊断和及时治疗是阻断疾病进展为失代偿期肝硬化的关键。治疗的总体目标是获得肝组织学缓解、防止肝纤维化进展和发生肝衰竭,延长患者生存期,提高患者的生存质量。在治疗方面,这类疾病有其独特的有效方法。已知 AIH对激素等免疫抑制药物治疗敏感,对严重、快速进展的AIH使用免疫抑制药物治疗,可使大部分患者的症状缓解,肝功能和组织学异常改善。对于尚不满足指征的患者的治疗应基于临床判断并给予个体化治疗方案。类似地,IgG4-SC患者使用免疫抑制治疗有效者的长期预后良好。PBC 的治疗主要为熊去氧胆酸(ursodeoxycholic acid,UDCA)。部分患者对 UD?CA 治疗有完全反应,能延长生存期,减少食管静脉曲张及失代偿肝硬化的发生。已知AILD的诊治具有特殊性,临床上应注意诊断和鉴别诊断,并及时治疗。国内外肝病学会均制定了各类AILD的诊治指南,对这类疾病的诊疗提出了共识性、规范化的意见。本书介绍了各种AILD的指南,并通过临床实例解读这些指南的应用。本书读者主要是在内科、消化科工作和培训的内科医生,内科各个专科的临床医生、医学生。自身免疫性肝病患者也可参阅。参加本指南翻译、提供病例和讨论者由复旦大学附属中山医院消化科医生及研究生、病理科和肝外科医师组成,并由富有临床经验的专科主任医师或副主任医师点评、审校。他们在繁忙工作中辛勤笔耕,在此一并表示感谢。本书难免有疏漏和不妥,谨请读者指正。
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