Brenard R, Geubel A P
Service de Gastro-entérologie, Hospital St-Joseph, Gilly.
Acta Clin Belg. 1991;46(5):305-12. doi: 10.1080/17843286.1991.11718181.
Antimitochondrial antibodies are found in 85 to 95% of patients with primary biliary cirrhosis. Nine different patterns have been identified but only four are associated with primary biliary cirrhosis. Anti-M2 antibodies are specific for the disease. The M2 antigen was found to be composed of five antigen determinants and related to the E2 component of three multienzyme complexes located within mitochondria. Anti-M4 and M8 antibodies appear invariably associated with anti-M2 and are markers for the "overlap syndrome" between primary biliary cirrhosis and chronic active hepatitis as well as for poor prognosis. Anti-M9 antibodies are preferentially associated with early and/or asymptomatic disease. Antinuclear antibodies are found in 24 to 58% of patients with primary biliary cirrhosis. Six various patterns have been reported. Antibodies directed to the 200 kD polypeptide of the nuclear pore and giving a perinuclear fluorescence are specific for primary biliary cirrhosis. Patients with such antibodies exhibit a less symptomatic disease and lower titers of anti-M2 than those without.
85%至95%的原发性胆汁性肝硬化患者体内可检测到抗线粒体抗体。已识别出九种不同模式，但只有四种与原发性胆汁性肝硬化相关。抗M2抗体是该疾病的特异性抗体。发现M2抗原由五个抗原决定簇组成，与位于线粒体内的三种多酶复合物的E2成分相关。抗M4和M8抗体总是与抗M2同时出现，是原发性胆汁性肝硬化与慢性活动性肝炎“重叠综合征”以及预后不良的标志物。抗M9抗体优先与早期和/或无症状疾病相关。24%至58%的原发性胆汁性肝硬化患者体内可检测到抗核抗体。已报道六种不同模式。针对核孔200 kD多肽并产生核周荧光的抗体是原发性胆汁性肝硬化的特异性抗体。与没有此类抗体的患者相比，有此类抗体的患者症状较轻，抗M2抗体滴度较低。
