Variant forms of autoimmune hepatitis.
Variant forms of autoimmune hepatitis.
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DOI：
10.1007/s11894-999-0089-0
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发表时间：
1999-02-01
影响因子：
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通讯作者：
Czaja, A J
中科院分区：
文献类型：
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作者：
Czaja, A J
自身免疫性肝炎的变异形式具有与另一种疾病（重叠综合征）混合的特征，或者与经典疾病（离群综合征）的可靠诊断不一致或不充分的发现。诊断标准尚未编纂，但修改后的评分系统的应用提供了一个模板，可以结合临床结果，以确保统一的评价和分类。变异形式经常被同化到常规的诊断范畴中。它们可能会扭曲自然历史和结果的分析。管理策略是经验性的，基于对最合适的药物方案的主要特征和机构的评估。对皮质类固醇有反应的变异综合征患者，其血清碱性磷酸酶水平通常低于正常上限的两倍。通过区分这些综合征，可以进一步了解自然史，遗传易感性和免疫机制。
Variant forms of autoimmune hepatitis have features that are intermixed with another disorder (overlap syndrome) or findings that are inconsistent with or insufficient for a confident diagnosis of classic disease (outlier syndrome). Diagnostic criteria have not been codified, but application of a modified scoring system provides a template that can be combined with clinical findings to ensure uniform evaluation and classification. Variant forms are frequently assimilated into conventional diagnostic categories. and they may distort analyses of natural history and outcome. Management strategies are empiric, based on an assessment of the predominant characteristics and institution of the most appropriate drug regimen. Patients with variant syndromes who respond to corticosteroids typically have serum alkaline phosphatase levels of less than twofold the upper limit of normal at presentation. By distinguishing between these syndromes, further insights into natural history, genetic predisposition, and immunopathic mechanisms are possible.