Farias Alberto Queiroz, Gonçalves Luciana Lofego, Bittencourt Paulo Lisboa, De Melo Evandro Sobroza, Abrantes-Lemos Clarice Pires, Porta Gilda, Nakhle Maria Cristina, Carrilho Flair Jose, Cancado Eduardo Luiz Rachid
Department of Gastroenterology, Institute of Tropical Medicine, Division of Pathology, University of Sao Paulo School of Medicine, Sao Paulo, Brazil.
J Gastroenterol Hepatol. 2006 May;21(5):887-93. doi: 10.1111/j.1440-1746.2006.04130.x.
According to the International Autoimmune Hepatitis Group (IAIHG) criteria, circulating antimitochondrial antibodies (AMA) do not support the diagnosis of autoimmune hepatitis (AIH). The aims of this study were to characterize a subset of patients with AIH who have AMA and antiM2 seropositivity, and to assess the applicability of the revised scoring system of the IAIHG in the diagnosis of this variant form of AIH.
Eighteen patients with AMA-AIH were enrolled and compared with 206 classical AIH and 85 primary biliary cirrhosis (PBC) controls. Human leukocyte antigen (HLA) class II alleles were determined by polymerase chain reaction (PCR) amplification with sequence-specific primers, and biopsies were blindly reevaluated.
The patients with AMA-AIH were, on average, older than patients with classical AIH and had an hepatocellular pattern of elevated liver enzymes, hypergammaglobulinemia and lower levels of cholesterol, when compared with PBC controls. There were no histological signs of PBC or overlapping forms in any AMA-AIH biopsies. The majority of patients with AMA-AIH carried HLA antigens associated with classical AIH (DRB103, n = 5; DRB104, n = 7, and DRB1*13, n = 6). Pretreatment scores classified all AMA-AIH patients with probable (n = 17) or improbable (n = 1) AIH. After treatment, only 28% of AMA-AIH patients reached scores for definite diagnosis, compared with 90.1% of AIH-1 and 96.4 AIH-2. In the AMA-AIH group, only patients who relapsed after immunosuppressive drug withdrawal could be classified with definite AIH.
AMA-AIH shares common features with classical AIH. The diagnosis of AMA-AIH may be swayed by the IAIHG criteria, rendering questionable the applicability of the revised scoring system to this variant form of AIH.
根据国际自身免疫性肝炎小组（IAIHG）的标准，循环抗线粒体抗体（AMA）不支持自身免疫性肝炎（AIH）的诊断。本研究的目的是对AMA和抗M2血清学阳性的AIH患者亚组进行特征分析，并评估IAIHG修订评分系统在诊断这种AIH变异形式中的适用性。
纳入18例AMA-AIH患者，并与206例经典AIH患者和85例原发性胆汁性肝硬化（PBC）对照进行比较。通过序列特异性引物的聚合酶链反应（PCR）扩增测定人类白细胞抗原（HLA）II类等位基因，并对活检标本进行盲法重新评估。
与PBC对照相比，AMA-AIH患者的平均年龄大于经典AIH患者，具有肝细胞型肝酶升高、高球蛋白血症和较低的胆固醇水平。在任何AMA-AIH活检标本中均无PBC或重叠形式的组织学征象。大多数AMA-AIH患者携带与经典AIH相关的HLA抗原（DRB103，n = 5；DRB104，n = 7，DRB1*13，n = 6）。治疗前评分将所有AMA-AIH患者分类为可能（n = 17）或不太可能（n = 1）的AIH。治疗后，只有28%的AMA-AIH患者达到明确诊断的评分，而AIH-1患者为90.1%，AIH-2患者为96.4%。在AMA-AIH组中，只有在停用免疫抑制药物后复发的患者才能被分类为明确的AIH。
AMA-AIH与经典AIH具有共同特征。IAIHG标准可能会影响AMA-AIH的诊断，使得修订评分系统在这种AIH变异形式中的适用性受到质疑。
