BACKGROUND AND AIMS Anti-mitochondrial antibodies (AMA) are closely linked to primary biliary cholangitis (PBC). The prevalence of AMA in the general population is low, and AMA positivity may precede PBC. We aimed to determine the natural history of subjects with positive AMA. METHODS In total, 302 patients were tested AMA-positive over a ten-year period. Of these, immunoblotting confirmed specific AMA in 184 (29 male, 155 female, age 59.6 ± 14.1 years). These subjects were invited to our liver outpatient clinic for clinical and biochemical re-evaluation. Detailed clinical history data were additionally collected from the hospital computer system and by telephone. The subsequent course with regard to mortality, liver-related morbidity, extrahepatic co-morbidities and effectiveness of PBC treatment was determined in 150 subjects (81.5%). RESULTS After 5.8 ± 5.6 years of follow-up (FU), of 184 AMA-positive subjects, 28 subjects (15.2%; liver-related mortality n = 5) were deceased, and 122 subjects (66.3%) completed FU while 34 subjects (18.5%) were not available for FU. The 122 patients who completed FU were 63 patients with established PBC, six de novo cases of PBC (10.2% of 59 initially at risk), 42 (34.4%) subjects were still AMA-positive without PBC, and 11 (9.0%) subjects were AMA-negative at FU. CONCLUSIONS Anti-mitochondrial antibodies-positive patients without PBC at baseline infrequently developed PBC over six years of FU. AMA positivity represented a transient serological autoimmune phenomenon in a significant proportion of subjects.

中文翻译：

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在过去的六年中，抗线粒体抗体阳性的人群中新发的原发性胆源性胆管炎发生率低。

背景与目的抗线粒体抗体（AMA）与原发性胆源性胆管炎（PBC）紧密相关。普通人群中AMA的患病率较低，AMA阳性可能早于PBC。我们旨在确定AMA阳性受试者的自然史。方法在十年期间内，总共302例患者接受了AMA阳性测试。其中，免疫印迹证实了184名男性（29名男性，155名女性，59.6±14.1岁）的特异性AMA。这些受试者被邀请到我们的肝脏门诊诊所进行临床和生化重新评估。另外，还从医院的计算机系统和通过电话收集了详细的临床病史数据。在150名受试者（81.5％）中确定了有关死亡率，肝脏相关发病率，肝外合并症和PBC治疗有效性的后续过程。结果在5.8±5.6年的随访（FU）中，在184例AMA阳性受试者中，有28名受试者（15.2％；与肝脏相关的死亡率n = 5）死亡，并且122名受试者（66.3％）完成了FU，而34名受试者（18.5％）无法使用FU。122名完成FU的患者为63例已建立PBC的患者，6例从新发病的PBC（最初处于风险的59例中的10.2％），42例（34.4％）仍为无PBC的AMA阳性患者和11例（9.0％）在FU处AMA阴性。结论基线时无PBC的抗线粒体抗体阳性患者在FU的六年内很少发展为PBC。AMA阳性在很大比例的受试者中代表了短暂的血清学自身免疫现象。122名受试者（66.3％）完成了FU，而34名受试者（18.5％）无法获得FU。122名完成FU的患者为63例已建立PBC的患者，6例从新发病的PBC（最初处于风险的59例中的10.2％），42例（34.4％）仍然没有PBC的AMA阳性患者和11例（9.0％）在FU处AMA阴性。结论基线时无PBC的抗线粒体抗体阳性患者在FU的六年内很少发展为PBC。AMA阳性在很大比例的受试者中代表了短暂的血清学自身免疫现象。122名受试者（66.3％）完成了FU，而34名受试者（18.5％）无法获得FU。122名完成FU的患者为63例已建立PBC的患者，6例从新发病的PBC（最初处于风险的59例中的10.2％），42例（34.4％）仍然没有PBC的AMA阳性患者和11例（9.0％）在FU处AMA阴性。结论基线时无PBC的抗线粒体抗体阳性患者在FU的六年内很少发展为PBC。AMA阳性在很大比例的受试者中代表了短暂的血清学自身免疫现象。11名（9.0％）受试者在FU时AMA阴性。结论基线时无PBC的抗线粒体抗体阳性患者在FU的六年内很少发展为PBC。AMA阳性在很大比例的受试者中代表了短暂的血清学自身免疫现象。11名（9.0％）受试者在FU时AMA阴性。结论基线时无PBC的抗线粒体抗体阳性患者在FU的六年内很少发展为PBC。AMA阳性在很大比例的受试者中代表了短暂的血清学自身免疫现象。