Berg P A, Wiedmann K H, Sayers T, Klöppel G, Lindner H
Lancet. 1980;2(8208-8209):1329-32. doi: 10.1016/s0140-6736(80)92397-1.
Clinical and morphological features in fifteen patients with chronic cholestatic liver disease (mixed form) who had two different types of complement-fixing antimitochondrial antibodies were compared with those found in twenty-five patients with classic primary biliary cirrhosis (PBC). PBC-specific antimitochondrial antibody (M2) directed against an antigen of the inner mitochondrial membrane was always present in both groups. However, mixed-form cases had a second antimitochondrial antibody (M4) which fixed complement with a trypsin insensitive antigen probably located on the outer membrane. Histological lesions typical of chronic active hepatitis, often associated with granulomata formation and bileduct proliferation, and simultaneous increases in IgM and IgG were predominant features, indicating that these mixed-form cases may represent a separate clinical entity.
对15例患有慢性胆汁淤积性肝病（混合型）且有两种不同类型补体结合抗线粒体抗体的患者的临床和形态学特征，与25例经典原发性胆汁性肝硬化（PBC）患者的特征进行了比较。两组患者均始终存在针对线粒体内膜抗原的PBC特异性抗线粒体抗体（M2）。然而，混合型病例还有第二种抗线粒体抗体（M4），它能与可能位于外膜的胰蛋白酶不敏感抗原结合补体。慢性活动性肝炎典型的组织学病变，常伴有肉芽肿形成和胆管增生，以及IgM和IgG同时升高是主要特征，表明这些混合型病例可能代表一种独立的临床实体。
