Match each clinical and diagnostic presentation to the most likely hepatic or biliary disorder.
- Primary biliary cholangitis (PBC)A 45-year-old woman presents with fatigue and severe pruritus; laboratories reveal a significantly elevated alkaline phosphatase and positive antimitochondrial antibodies (AMA).
- Primary sclerosing cholangitis (PSC)A 30-year-old man with a history of ulcerative colitis presents with jaundice; imaging shows multifocal stricturing and dilation of the intrahepatic and extrahepatic bile ducts.
- Autoimmune hepatitisA 35-year-old woman presents with fatigue and joint pain; laboratory studies show highly elevated aminotransferases, elevated total IgG, and positive anti-smooth muscle antibodies (ASMA).
- Wilson diseaseA 22-year-old man presents with a new-onset resting tremor and dysarthria; laboratory testing reveals elevated aminotransferases and a very low serum ceruloplasmin level.
Answer
Primary biliary cholangitis matches the presentation with positive antimitochondrial antibodies (AMA). Primary sclerosing cholangitis matches the patient with ulcerative colitis and bile duct stricturing. Autoimmune hepatitis matches the patient with elevated IgG and anti-smooth muscle antibodies. Wilson disease matches the young adult with neurologic symptoms and low ceruloplasmin.
Each specific diagnosis has a hallmark clinical vignette. Primary biliary cholangitis is characterized by AMA positivity in a middle-aged woman with pruritus. Primary sclerosing cholangitis is characterized by biliary stricturing and a strong association with inflammatory bowel disease. Autoimmune hepatitis features a hepatocellular injury pattern with ASMA positivity. Wilson disease is defined by impaired copper excretion leading to hepatic and neurologic manifestations alongside low ceruloplasmin.
Step-by-Step Solution
Key Concept
Clinical presentation and specific diagnostic markers of autoimmune, cholestatic, and metabolic liver diseases.
Estimated Time:1m 30s