qzmjef 发表于 2024-10-10 07:04:56

关于自己免疫性肝病，你应该晓得这些 | 临床必须


    <div style="color: black; text-align: left; margin-bottom: 10px;"><img src="https://p3-sign.toutiaoimg.com/tos-cn-i-axegupay5k/bfcffb6e5ca24643a672116f9d9e7bc0~noop.image?_iz=58558&amp;from=article.pc_detail&amp;lk3s=953192f4&amp;x-expires=1728785888&amp;x-signature=5F%2F44PEGgdZwc%2BAscMC62eXfnCc%3D" style="width: 50%; margin-bottom: 20px;"></div>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;"><span style="color: black;">导读</span></strong></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;"><span style="color: black;"><span style="color: black;">自己</span>免疫性肝病是临床上<span style="color: black;">平常</span>的<span style="color: black;">疾患</span>类型，<span style="color: black;">重点</span><span style="color: black;">指的是</span><span style="color: black;">因为</span><span style="color: black;">自己</span>免疫介导的肝脏炎症性病变，<span style="color: black;">包含</span><span style="color: black;">自己</span>免疫性肝炎（autoimmune hepatitis，AIH）、原发性胆汁性胆管炎（primary biliary cholangitis，PBC）和原发性硬化性胆管炎（primary sclerosing cholangitis，PSC）。</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;"><span style="color: black;">本文<span style="color: black;">重点</span>围绕<span style="color: black;">自己</span>免疫性肝病的临床表现、诊断、并发症、治疗<span style="color: black;">办法</span>进行简要介绍。</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><strong style="color: blue;"><span style="color: black;"><span style="color: black;"><span style="color: black;">自己</span>免疫性肝病的临床表现</span></span></strong></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;"><span style="color: black;">►AIH</span></strong></span><span style="color: black;"><span style="color: black;"><span style="color: black;">：临床表现多样，<span style="color: black;">通常</span>表现为慢性、隐匿起病，但<span style="color: black;">亦</span>可表现为急性<span style="color: black;">爆发</span>，<span style="color: black;">乃至</span><span style="color: black;">导致</span>急性肝功能衰竭。部分AIH<span style="color: black;">病人</span><span style="color: black;">无</span>任何症状，多因体检<span style="color: black;">发掘</span>转氨酶水平<span style="color: black;">上升</span>而就诊。部分<span style="color: black;">病人</span>可能<span style="color: black;">显现</span>疲劳、关节<span style="color: black;">病痛</span>、恶心、腹泻、食欲不振等症状，体检可<span style="color: black;">发掘</span>肝大、脾大、腹水等体征，偶见<span style="color: black;">周边</span>性水肿。急性起病者可<span style="color: black;">显现</span>急性肝衰竭、重度黄疸和凝血酶原时间延长。</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;"><span style="color: black;">►PBC</span></strong></span><span style="color: black;"><span style="color: black;"><span style="color: black;">：<span style="color: black;">初期</span><span style="color: black;">病人</span>大多无<span style="color: black;">显著</span>临床症状，<span style="color: black;">然则</span>大<span style="color: black;">都数</span>无症状<span style="color: black;">病人</span>会在５年内<span style="color: black;">显现</span>症状。最<span style="color: black;">平常</span>的临床表现为乏力和皮肤瘙痒。PBC<span style="color: black;">病人</span>还可能<span style="color: black;">显现</span>胆汁淤积症相关表现，如骨代谢<span style="color: black;">反常</span>、脂溶性维生素缺乏、高脂血症等。<span style="color: black;">疾患</span>后期，<span style="color: black;">病人</span>可<span style="color: black;">出现</span>肝硬化和门静脉高压的一系列并发症。</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;"><span style="color: black;">►PSC</span></strong></span><span style="color: black;"><span style="color: black;"><span style="color: black;">：临床表现多样，15%-55%的<span style="color: black;">病人</span>诊断时无症状。<span style="color: black;">显现</span>症状的<span style="color: black;">病人</span>最<span style="color: black;">平常</span>的表现可能为乏力，但无特异性。其他可能<span style="color: black;">显现</span>的症状及体征<span style="color: black;">包含</span>体质量减轻、瘙痒、黄疸和肝脾肿大等。<span style="color: black;">病人</span>还可伴有反复<span style="color: black;">爆发</span>的右上腹痛，酷似胆石症和胆道感染。</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><strong style="color: blue;"><span style="color: black;"><span style="color: black;"><span style="color: black;">自己</span>免疫性肝病的诊断</span></span></strong></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;"><span style="color: black;">►AIH</span></strong></span><span style="color: black;"><span style="color: black;"><span style="color: black;">：诊断<span style="color: black;">重点</span>基于一系列典型的临床特征。AIH的一个诊断标志是存在<span style="color: black;">自己</span>抗体，<span style="color: black;">按照</span><span style="color: black;">自己</span>抗体还可将AIH分为两型：1型呈抗核抗体（antinuclear antibody，ANA）、抗平滑肌抗体（anti-smooth muscle antibody，ASMA）或抗可溶性肝抗原（soluble liver antigen，SLA）阳性，2型呈肝肾微粒体抗体１型（liver-kidney-microsomal 1，LKM1）和/或肝细胞质抗体１型（liver cytosol 1，LC1）阳性。后者较少见，<span style="color: black;">重点</span>见于儿童人群。</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;"><strong style="color: blue;"><span style="color: black;">AIH诊断流程及<span style="color: black;">关联</span>内容见链接：</span>关于<span style="color: black;">自己</span>免疫性肝炎，你需要<span style="color: black;">把握</span>这些 | 临床<span style="color: black;">必须</span></strong></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;"><span style="color: black;">►PBC</span></strong></span><span style="color: black;"><span style="color: black;"><span style="color: black;">：当碱性磷酸酶（alkaline phosphate，ALP）<span style="color: black;">上升</span><span style="color: black;">最少</span>为正常值上限的1.5倍，抗线粒体抗体（anti-mitochondrial antibody，AMA）阳性时，可诊断为PBC。AMA对PBC<span style="color: black;">拥有</span>较高的诊断准确性。抗gp210和抗sp100在PBC诊断中<span style="color: black;">拥有</span>高度特异性，但灵敏度较低。5%-10%的PBC<span style="color: black;">病人</span>PBC特异性抗体检测呈阴性或滴度较低，需要进行肝活检。</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;"><span style="color: black;">►PSC</span></strong></span><span style="color: black;"><span style="color: black;"><span style="color: black;">：在排除继发性硬化性胆管炎证据后，PSC的诊断<span style="color: black;">一般</span>基于肝功能慢性胆汁淤积指标（ALP）<span style="color: black;">反常</span>，伴有典型的胆管造影结果（内镜下逆行胆管造影或磁共振胆管造影<span style="color: black;">表示</span>多灶性狭窄<span style="color: black;">导致</span>的肝内和/或肝外胆管“串珠样”改变）。肝穿刺活检病理的典型特征为“洋葱皮样”纤维化，<span style="color: black;">然则</span>较少<span style="color: black;">显现</span>。</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><strong style="color: blue;"><span style="color: black;"><span style="color: black;"><span style="color: black;">自己</span>免疫性肝病的<span style="color: black;">重点</span>并发症</span></span></strong></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;"><span style="color: black;">急性AIH<span style="color: black;">发展</span>为<span style="color: black;">爆发</span>性肝衰竭是一种不<span style="color: black;">平常</span>但预后不良的并发症。慢性AIH、PBC和PSC可在数年内<span style="color: black;">发展</span>为肝硬化，肝硬化可进一步并发门静脉高压和肝细胞癌。在罕见<span style="color: black;">状况</span>下，<span style="color: black;">因为</span>炎性浸润压迫肝小静脉，PBC可在肝硬化前<span style="color: black;">出现</span>门静脉高压。PSC<span style="color: black;">亦</span>可<span style="color: black;">显现</span>独特的并发症，<span style="color: black;">包含</span>细菌性胆管炎、胆管癌、胆囊癌和炎症性肠病<span style="color: black;">关联</span>性结直肠癌。</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><strong style="color: blue;"><span style="color: black;"><span style="color: black;"><span style="color: black;">自己</span>免疫性肝病的治疗<span style="color: black;">办法</span></span></span></strong></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;"><span style="color: black;">►AIH</span></strong></span><span style="color: black;"><span style="color: black;"><span style="color: black;">：治疗的总体<span style="color: black;">目的</span>是<span style="color: black;">得到</span>肝组织学缓解、防止肝纤维化的发展和肝功能衰竭的<span style="color: black;">出现</span>，<span style="color: black;">加强</span><span style="color: black;">病人</span>的<span style="color: black;">存活</span>期和<span style="color: black;">存活</span>质量。治疗<span style="color: black;">重点</span>是<span style="color: black;">经过</span>糖皮质类固醇激素（最常用的是泼尼松）诱导缓解，以及<span style="color: black;">经过</span>免疫调节剂[如硫唑嘌呤（一线<span style="color: black;">药品</span>）或吗替麦考酚酯]维持缓解。类固醇在<span style="color: black;">几周</span>至数月内停药。临床缓解后<span style="color: black;">针对</span>免疫调节剂的停用需要极其<span style="color: black;">小心</span>。</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;"><span style="color: black;">►PBC</span></strong></span><span style="color: black;"><span style="color: black;"><span style="color: black;">：熊去氧胆酸（ursodeoxycholic acid，UCDA）是PBC公认的一线治疗，可减缓大<span style="color: black;">都数</span><span style="color: black;">病人</span>的<span style="color: black;">疾患</span><span style="color: black;">发展</span>。当UCDA应答<span style="color: black;">不良</span>时，奥贝胆酸是<span style="color: black;">获准</span>的辅助治疗，或当UCDA不耐受时，奥贝胆酸可<span style="color: black;">做为</span>单药治疗。需要<span style="color: black;">重视</span>的是，不<span style="color: black;">举荐</span>将奥贝胆酸用于失代偿性肝病<span style="color: black;">病人</span>，<span style="color: black;">由于</span>不适当给药有肝功能失代偿和死亡的<span style="color: black;">危害</span>。贝特类<span style="color: black;">药品</span>（如非诺贝特、苯扎贝特等）可能是PBC的其他潜在疗法，但在正式<span style="color: black;">运用</span>之前，还需要进行更大规模的<span style="color: black;">实验</span>。抗组胺药、考来烯胺、利福平或舍曲林可用于治疗PBC瘙痒症。</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><strong style="color: blue;"><span style="color: black;">►PSC</span></strong></span><span style="color: black;"><span style="color: black;"><span style="color: black;">：<span style="color: black;">日前</span><span style="color: black;">已然</span>在PSC<span style="color: black;">病人</span>中测试了几种<span style="color: black;">药品</span>治疗（如UCDA、免疫调节剂和抗生素）的效果，尽管其中许多<span style="color: black;">药品</span><span style="color: black;">表示</span>出改善肝酶的能力，但还缺乏这些<span style="color: black;">药品</span>降低死亡率或阻止<span style="color: black;">疾患</span><span style="color: black;">发展</span>的有力证据。<span style="color: black;">日前</span>欧美的PSC指南均不<span style="color: black;">举荐</span><span style="color: black;">运用</span>UCDA治疗PSC。鉴于<span style="color: black;">日前</span>肝移植治疗在我国广泛<span style="color: black;">运用</span>尚存在困难，<span style="color: black;">因此呢</span>《原发性硬化性胆管炎诊断和治疗专家共识（2015）》<span style="color: black;">意见</span><span style="color: black;">能够</span>对PSC<span style="color: black;">病人</span>尝试进行UCDA经验性治疗，但不<span style="color: black;">举荐</span>高剂量。</span></span></span></p>
    <div style="color: black; text-align: left; margin-bottom: 10px;"><img src="https://p3-sign.toutiaoimg.com/tos-cn-i-qvj2lq49k0/c71e47384d114e67a7feb281e6250e2c~noop.image?_iz=58558&amp;from=article.pc_detail&amp;lk3s=953192f4&amp;x-expires=1728785888&amp;x-signature=e6xHr1D14A1bHDDe96bdI6uAbAE%3D" style="width: 50%; margin-bottom: 20px;"></div>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;"><span style="color: black;">参考资料：</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;"><span style="color: black;"> Amir M, Parekh SM. Classic Autoimmune Liver Disorders and Celiac Hepatitis. Clin Liver Dis (Hoboken). 2021 Jun 4;17(5):347-352.</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;"><span style="color: black;"> 中华医学会肝病学分会. <span style="color: black;">自己</span>免疫性肝炎诊断和治疗共识（2015）. 胃肠病学. 2016,21(4):969-982.</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;"><span style="color: black;"> Smith A. What to know about autoimmune hepatitis treatments and their side effects. Medical News Today. 2021 May 27.</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;"><span style="color: black;"> 中华医学会肝病学分会. 原发性胆汁性肝硬化(又名原发性胆汁性胆管炎)诊断和治疗共识（2015）. 临床肝胆病杂志. 2015,31(12):1980-1988.</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;"><span style="color: black;"> 中华医学会肝病学分会. 原发性硬化性胆管炎诊断和治疗专家共识（2015）. 临床肝胆病杂志. 2016,32(1):23-31.</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;"><span style="color: black;"> 唐映梅, 杨晋辉. <span style="color: black;">自己</span>免疫性肝病的诊断与治疗. 现代消化及介入诊疗. 2012,17(06):337-342.</span></span></span></p>
    <p style="font-size: 16px; color: black; line-height: 40px; text-align: left; margin-bottom: 15px;"><span style="color: black;"><span style="color: black;">源自</span>：<span style="color: black;"><span style="color: black;">医脉通肝脏科</span></span></span></p>




7wu1wm0 发表于 2024-10-15 10:17:02

我们有着相似的经历，你的感受我深有体会。
页: [1]
查看完整版本: 关于自己免疫性肝病，你应该晓得这些 | 临床必须