Lacerda M A, Ludwig J, Dickson E R, Jorgensen R A, Lindor K D
Division of Gastroenterology, Mayo Clinic, Rochester, Minnesota.
Am J Gastroenterol. 1995 Feb;90(2):247-9.
We reviewed our experience with patients who had biochemical and histological features of primary biliary cirrhosis in the absence of antimitochondrial antibodies (AMA) to better understand this variant of the syndrome.
During the period between 1976 to 1992, 597 patients with clinical and histological features of primary biliary cirrhosis were seen at the Mayo Clinic. Thirty-five (5.8%) of these patients were negative for antimitochondrial antibody and had normal cholangiographic studies. The records of these patients were reviewed for this study.
No difference was found between the two groups with respect to age, gender, or biochemical features. IgM and gamma-globulin levels were higher in the antimitochondrial antibody-positive than the antimitochondrial antibody-negative patients. What is more important, 96% of the AMA-negative patients who could be tested were positive for antinuclear antibody or anti-smooth-muscle antibodies. These tests were positive in only 56% of the antimitochondrial antibody-positive group (p < 0.05). The response of five of these patients to ursodeoxycholic acid appeared comparable to the response seen in antimitochondrial antibody-positive patients.
Patients with histological features of primary biliary cirrhosis, whether antimitochondrial antibody positive or negative, are quite comparable with respect to clinical and biochemical features. Other autoantibodies, such as antinuclear or anti-smooth-muscle antibodies, are more common in the antimitochondrial antibody-negative group. These two conditions might be part of a spectrum that has been termed "autoimmune cholangitis" and that is characterized by chronic cholestasis, histological features of chronic nonsuppurative destructive cholangitis, and the presence of any of a variety of serum autoantibodies.
我们回顾了原发性胆汁性肝硬化患者在缺乏抗线粒体抗体（AMA）时的生化和组织学特征，以更好地理解该综合征的这一变异型。
在1976年至1992年期间，梅奥诊所诊治了597例具有原发性胆汁性肝硬化临床和组织学特征的患者。其中35例（5.8%）患者抗线粒体抗体阴性且胆管造影检查正常。本研究对这些患者的病历进行了回顾。
两组在年龄、性别或生化特征方面未发现差异。抗线粒体抗体阳性患者的IgM和γ球蛋白水平高于抗线粒体抗体阴性患者。更重要的是，96%可进行检测的AMA阴性患者抗核抗体或抗平滑肌抗体呈阳性。这些检测在抗线粒体抗体阳性组中仅56%呈阳性（p<0.05）。其中5例患者对熊去氧胆酸的反应与抗线粒体抗体阳性患者的反应相当。
原发性胆汁性肝硬化组织学特征的患者，无论抗线粒体抗体阳性或阴性，在临床和生化特征方面相当。其他自身抗体，如抗核抗体或抗平滑肌抗体，在抗线粒体抗体阴性组中更为常见。这两种情况可能是被称为“自身免疫性胆管炎”的一系列病症的一部分，其特征为慢性胆汁淤积、慢性非化脓性破坏性胆管炎的组织学特征以及多种血清自身抗体中的任何一种的存在。
