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Autoimmune hepatitis in children: what is different from adult AIH?

机译:儿童自身免疫性肝炎:与成人AIH有何不同?

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Autoimmune hepatitis (AIH) is characterized by inflammatory liver histology, circulating non-organ-specific autoantibodies, and increased levels of immunoglobulin (Ig) G in the absence of a known etiology. Two types of childhood AIH are recognized according to seropositivity: smooth muscle antibody (SMA) and/or antinuclear antibody (ANA), which is AIH type 1; and antibodies to liver-kidney microsome type 1 (anti-LKM1), which is AIH type 2. There is a female predominance in both. Autoimmune hepatitis type 2 presents more acutely, at a younger age, and commonly with IgA deficiency; however, duration of symptoms before diagnosis, clinical signs, family history of autoimmunity, presence of associated autoimmune disorders, response to treatment, and long-term prognosis are similar in the two groups. Immunosuppressive treatment with steroids and azathioprine, which should be instituted promptly to avoid progression to cirrhosis, induces remission in 80% of cases. Relapses are common, often due to nonadherence. Drugs effective in refractory cases include cyclosporine and mycophenolate mofetil. Long-term treatment is usually required, with only some 20% of AIH type 1 patients able to discontinue therapy successfully. In childhood, sclerosing cholangitis with strong autoimmune features, including interface hepatitis and serological features identical to AIH type 1, is as prevalent as AIH, but it affects boys and girls equally. The differential diagnosis relies on cholangiographic studies. In autoimmune sclerosing cholangitis, liver parenchymal damage responds satisfactorily to immunosuppressive treatment, whereas bile duct disease tends to progress.
机译:自身免疫性肝炎(AIH)的特征是炎症性肝组织学,循环的非器官特异性自身抗体以及在缺乏已知病因的情况下增加的免疫球蛋白(Ig)G水平。根据血清阳性,可识别两种类型的儿童期AIH:平滑肌抗体(SMA)和/或抗核抗体(ANA),即AIH类型1;以及针对1型肝肾微粒体(抗LKM1)(即AIH 2型)的抗体。两者均以女性为主。 2型自身免疫性肝炎发病较年轻,年龄较小,通常患有IgA缺乏症。然而,两组的诊断前症状持续时间,临床体征,自身免疫家族史,相关的自身免疫性疾病的存在,对治疗的反应以及长期预后相似。应立即采用类固醇和硫唑嘌呤进行免疫抑制治疗,以避免进展为肝硬化,在80%的病例中可缓解。复发是常见的,通常是由于不坚持所致。在难治性病例中有效的药物包括环孢霉素和霉酚酸酯。通常需要长期治疗,只有约20%的AIH 1型患者能够成功终止治疗。在儿童时期,硬化性胆管炎具有很强的自身免疫特征,包括与AIH 1型相同的界面肝炎和血清学特征,与AIH一样普遍,但对男孩和女孩的影响均相同。鉴别诊断依赖于胆道造影研究。在自身免疫性硬化性胆管炎中,肝实质损害对免疫抑制治疗的反应令人满意,而胆管疾病则倾向于发展。

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