Antimitochondrial Antibody-Negative Primary Biliary Cholangitis: A Retrospective Diagnosis.

Primary biliary cholangitis (PBC) is an inflammatory cholestatic disease that tends to worsen, leading to hepatic cirrhosis and portal hypertension. We present a case of a middle-aged female who presented with progressively worsening generalized itch; the examination was significant only for urticarial rash and facial swelling. Investigation revealed direct hyperbilirubinemia, mildly elevated transaminase, and significant elevation of alkaline phosphatase. A differential was performed with labs including antimitochondrial antibodies (AMA) for PBC, hepatitis panel, anti-smooth muscle antibodies for autoimmune hepatitis, and tissue transglutaminase IgA for celiac disease, all of which were unremarkable. The patient was empirically treated with ursodeoxycholic acid (UDCA). Given the excellent clinical response at the three-week follow-up to treatment despite negative AMA, further testing with anti-sp100 and anti-gp210 was pursued, which returned positive for anti-sp100, confirming the diagnosis of PBC.

[Is autoimmune cholangitis-anti-mitochondrial antibody-negative primary biliary cholangitis still an independent disease?]

Autoimmune cholangitis (AIC) was first reported in 1987 as a chronic cholestatic disease that occurs predominantly in middle-aged women and has a common clinical manifestations, biochemical abnormalities and pathological changes with primary biliary cholangitis (PBC). However, serum anti-mitochondrial antibodies (AMA) are negative, and ANA and/or smooth muscle antibody positive rates are higher. The treatment response and prognosis with ursodeoxycholic acid and steroids is poor, thus it needs to be treated with immunosuppressive agents. Presently, the exact pathological mechanism of AIC is still unclear, and there is no unified assertion that classifies it as a new autoimmune liver disease or AMA-negative PBC. This article reviews the worldwide published work on AIC and compares them with PBC.

Is autoimmune cholangitis-anti-mitochondrial antibody-negative primary biliary cholangitis still an independent disease? / 中华肝脏病杂志

Autoimmune cholangitis (AIC) was first reported in 1987 as a chronic cholestatic disease that occurs predominantly in middle-aged women and has a common clinical manifestations, biochemical abnormalities and pathological changes with primary biliary cholangitis (PBC). However, serum anti-mitochondrial antibodies (AMA) are negative, and ANA and/or smooth muscle antibody positive rates are higher. The treatment response and prognosis with ursodeoxycholic acid and steroids is poor, thus it needs to be treated with immunosuppressive agents. Presently, the exact pathological mechanism of AIC is still unclear, and there is no unified assertion that classifies it as a new autoimmune liver disease or AMA-negative PBC. This article reviews the worldwide published work on AIC and compares them with PBC.