A small percent (5-10%) of patients with primary biliary cirrhosis (PBC) may be negative for antimitochondrial antibodies (AMA). This condition may be referred to as autoimmune cholangitis.
Features:
(1) clinical signs and symptoms consistent with PBC
(2) histologic changes and progression consistent with PBC
(3) persistent absence of AMA in the patient's serum
(4) response to therapy similar to patients who are AMA-positive
Some patients show antinuclear antibodies, anti-gp210, anti-sp100, anti-p62 or other autoantibodies.
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