Autoimmune and Metabolic Liver Diseases MCQs

Type 1, more common in middle-age and older individuals, is characterized by the presence of antinuclear (ANA), anti-smooth muscle actin (SMA), anti-mitochondrial (AMA), and anti-soluble liver antigen/ liver-pancreas antigen (anti-SLA/LP) antibodies. Type 2 auto immune hepatitis, is more common in children and is characterized by anti- liver kidney microsome-1 (lkm-1)antibodies, which are directed against cyp2d6, and anti-liver cytosol-1 (acl-1) anti bodies. Reference: Robbins Basic Pathology, 10 th ed, Pg-651

The signs, symptoms, and history, combined with the results of the ERCP, are most consistent with primary sclerosing cholangitis. Primary sclerosing cholangitis (psc) is a chronic liver disease in which the bile ducts inside and outside the liver become inflamed and scarred, and eventually narrowed or blocked. When this happens, bile builds up in the liver and causes further liver damage. Reference: Robbins Basic Pathology, 10 th ed, Pg-664

Primary biliary cirrhosis is most common in middle aged females, and patients will often present with pruritus due to cholestasis, which can evolve to cirrhosis of the liver (D). Jaundice is a sign of liver failure, and thus, among the choices, α-1-antitrypsin and Galactosemia could cause fatigue, but they are not commonly associated with pruritus; also, Galactosemia would affect younger ages (A, B). Primary sclerosing cholangitis is associated with ulcerative colitis (C). Primary biliary cholangitis, previously called primary biliary cirrhosis, is a chronic disease in which the bile ducts in your liver are slowly destroyed. Bile is a fluid made in your liver. It aids with digestion and helps you absorb certain vitamins. Reference: Robbins Basic Pathology, 10 th ed, Pg-664