Division of Gastroenterology and Hepatology, Mayo Clinic, 5777 East Mayo Boulevard, Phoenix, AZ 85054, USA.
Division of Gastroenterology and Hepatology, Mayo Clinic, 5777 East Mayo Boulevard, Phoenix, AZ 85054, USA; Office of the Provost, Arizona State University, 550 North 3rd Street, Phoenix, AZ 85004, USA.
Clin Liver Dis. 2018 Aug;22(3):589-601. doi: 10.1016/j.cld.2018.03.009.
Antimitochondrial antibody (AMA)-negative primary biliary cholangitis (PBC) is a term reserved for patients with clinical and histopathological findings consistent with PBC but without positive AMA. There does not seem to be a natural progression from AMA negativity to positivity. Antinuclear and antismooth muscle antibodies are frequently found in the absence of histologic autoimmune hepatitis features. The disease course may be more severe than AMA-positive. Response to standard therapy for PBC and autoimmune hepatitis varies. Nevertheless, there is insufficient evidence to suggest AMA-negative PBC is different enough to warrant classification as a separate disease from AMA-positive PBC.
抗线粒体抗体（AMA）阴性原发性胆汁性胆管炎（PBC）是一个术语，用于描述具有与 PBC 一致的临床和组织病理学发现但 AMA 阴性的患者。似乎没有从 AMA 阴性到阳性的自然进展。在没有组织学自身免疫性肝炎特征的情况下，常发现抗核抗体和抗平滑肌抗体。疾病过程可能比 AMA 阳性更严重。对 PBC 和自身免疫性肝炎的标准治疗的反应各不相同。然而，目前尚无足够的证据表明 AMA 阴性 PBC 有足够的差异，需要将其与 AMA 阳性 PBC 区分开来作为一种单独的疾病。
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