Anti-mitochondrial antibody ( AMA) is a typical serum marker for primary biliary cholangitis ( PBC) , and the diagnosis ofAMA-negative PBC may easily be neglected in clinical practice. This article analyzes the differences in epidemiology, clinical and patho-logical features, and treatment outcome between AMA-negative and AMA-positive PBC and points out that there are significant differencesbetween them in the symptom of pruritus, immunoglobulin M, and severity of bile duct injury. For patients with a clinical diagnosis of AMA-negative PBC, immunofluorescence assay combined with immunological detection should be performed to exclude the false-negative resultof AMA, and a confirmed diagnosis can be made with reference to the manifestation of cholestasis, positive results of anti-sp100, anti-gp210, and anti-ANA antibodies, and typical hyperactive cholangitis based on pathological examination. Differential diagnosis of this dis-ease with other types of bile duct injury or absence of bile duct should be taken seriously in clinical practice.
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