Masuda J, Omagari K, Matsuo I, Kinoshita H, Sakimura K, Hazama H, Ohba K, Isomoto H, Murase K, Murata I, Kohno S
Second Department of Internal Medicine, Nagasaki University School of Medicine, Japan.
J Gastroenterol Hepatol. 2001 Feb;16(2):239-43. doi: 10.1046/j.1440-1746.2001.02377.x.
A case of primary biliary cirrhosis (PBC) in whom a complete biochemical (serum bilirubin, transaminases and alkaline phosphatase) remission was noted after combination treatment with ursodeoxycholic acid (UDCA) and corticosteroid is reported. The antimitochondrial antibody (AMA) detected by indirect immunofluorescence was initially positive, and the antinuclear antibody (ANA) was negative, but these two antibodies subsequently fluctuated independently (AMA-positive/ANA-negative, AMA-negative/ANA-negative, AMA-negative/ANA-positive, AMA-positive/ANA-positive, and again AMA-negative/ANA-positive) in spite of a lack of histopathological improvement in the liver after treatment. The clinical presentation in our case suggests that in some cases the diagnosis of PBC or so-called autoimmune cholangitis (AIC) might depend on the 'phase' of the same disease. Our results also suggest that detailed immunoreactive profiles against 2-oxo-acid dehydrogenase complex (2-OADC) enzymes by using immunoblotting, together with a serial histological examination, should provide more precise information for a diagnosis of PBC.
报告了一例原发性胆汁性肝硬化（PBC）患者，该患者在接受熊去氧胆酸（UDCA）和皮质类固醇联合治疗后，生化指标（血清胆红素、转氨酶和碱性磷酸酶）完全缓解。通过间接免疫荧光检测的抗线粒体抗体（AMA）最初呈阳性，抗核抗体（ANA）呈阴性，但尽管治疗后肝脏组织病理学无改善，这两种抗体随后独立波动（AMA阳性/ANA阴性、AMA阴性/ANA阴性、AMA阴性/ANA阳性、AMA阳性/ANA阳性，再次AMA阴性/ANA阳性）。我们病例的临床表现表明，在某些情况下，PBC或所谓的自身免疫性胆管炎（AIC）的诊断可能取决于同一疾病的“阶段”。我们的结果还表明，通过免疫印迹法对2-氧代酸脱氢酶复合物（2-OADC）酶进行详细的免疫反应谱分析，以及连续的组织学检查，应为PBC的诊断提供更精确的信息。
