Elsevier, MacSween's Pathology of the Liver (Eighth Edition)
Autoimmune hepatitis (AIH) is an inflammatory liver disease that appears to be caused by liver-directed aberrant autoreactivity. The clinical spectrum is heterogeneous, from subclinical disease, to cirrhosis, to fulminant hepatitis. Serum autoantibodies such as antinuclear and anti-smooth muscle antibodies are common. Histologic findings include a chronic hepatitis pattern of injury with prominent plasma cells, often with brisk neuroinflammatory activity, or, in some cases, centrilobular injury. Overlap syndromes with other autoimmune liver diseases such as primary biliary cholangitis and primary sclerosing cholangitis are relatively rare. AIH generally responds to immunosuppressive therapy.
