Review Question - QID 104823

This patient is presenting with primary biliary cirrhosis (PBC). The first line treatment for PBC is ursodeoxycholic acid.

PBC is an autoimmune condition in which the small bile ducts in the liver are destroyed. This causes the liver to be unable to excrete bile, causing a conjugated hyperbilirubinemia and elevated alkaline phosphatase. Patients with PBC tend to be female and present in middle age with an insidious onset of pruritus, jaundice (Figure A), xanthelasma (Figure B), dark urine, light stools, and hepatosplenomegaly. PBC is associated with other autoimmune conditions such as CREST, rheumatoid arthritis, and celiac disease. On labs, elevated antimitochondrial antibodies are observed and liver biopsy will show lymphocytic infiltrate and granulomas. The first line treatment for PBC is ursodeoxycholic acid which decreases symptoms and increase transplant free survival time.

Roche and Kobos review the diagnosis and management of jaundice in the adult patient. They state that jaundice in an adult patient can be caused by a wide variety of benign or life-threatening disorders. Organizing the differential diagnosis by prehepatic, intrahepatic, and posthepatic causes may help make the work-up more manageable. The conjugated (direct) bilirubin level is often elevated by alcohol, infectious hepatitis, drug reactions, and autoimmune disorders such as PBC.

Reamy et al. review the diagnostic approach to pruritus. They state that pruritus can be a symptom of a distinct dermatologic condition or of an occult underlying systemic disease. Of the patients referred to a dermatologist for generalized pruritus with no apparent primary cutaneous cause, 14 to 24 percent have a systemic etiology. Initial evaluation for systemic disease includes complete blood count and measurement of thyroid-stimulating hormone, fasting glucose, alkaline phosphatase, bilirubin, creatinine, and blood urea nitrogen.

Figure A demonstrates scleral icterus. Figure B demonstrates xanthelasmas which are commonly observed in PBC. Figure C is a classic liver biopsy of PBC demonstrating lymphocytic infiltration and periductal inflammation. Illustration A is an immunofluorescent staining of the antimitochondrial antibodies observed in PBC. Illustration B demonstrates the pathophysiology of PBC.

Incorrect answers:
Answer 1: Ledipasvir-sofosbuvir is an example of a curative antiviral medication regimen for Hepatis C genotype 1 infection. It is not a treatment for primary biliary cirrhosis.
Answer 2: While liver transplant is sometimes eventually needed in PBC, it is not first line treatment.
Answer 3: Ribavirin is an adjunctive medication that is sometimes administered alongside antiviral regimens for Hepatitis C treatment. It is not used to treated PBC.
Answer 5: The Kasai procedure is the surgical treatment for primary biliary atresia.