Answer 3. D
This patient most likely has type II AIH, which is characterized by positive anti-LKM-1 antibody and/or anti-liver cytosol-1 antibody. Compared with patients with type I AIH, patients with type II AIH tend to present at a younger age and to have a higher incidence of partial IgA deficiency and acute liver failure at presentation (which is suggested by this patient’s coagulopathy).
Type I AIH is more common than type II AIH and is characterized by positivity for antinuclear antibody (ANA), anti–smooth muscle antibody (SMA), or anti–F-actin antibodies. Primary sclerosing cholangitis is an autoimmune inflammatory condition targeting the extrahepatic bile ducts that may be associated with other autoimmune disorders such as AIH, inflammatory bowel disease, or thyroiditis. Patients with primary sclerosing cholangitis are usually ANA, SMA, and LKM-1 negative, although these values are occasionally positive in patients with overlap syndrome, in which AIH and PSC occur together. Patients with lupus may have an elevated ANA titer, which is also seen in patients with AIH, but typically do not present with positive LKM-1 and acute liver failure. Caroli disease is a disease that presents with hyperbilirubinemia that is not associated with positive LKM-1 or IgA deficiency.
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