
Review the Workup for Primary Biliary Cirrhosis and Understand its Complications
By John Joseph Pack MD
Published on 06/08/2026
Primary Biliary Cirrhosis is a slowly progressive autoimmune disease involving the destruction of small intrahepatic bile ducts. It most commonly affects middle-aged women. Symptoms usually include fatigue and unexplained pruritis, that is poorly responsive to anti-pruritics. Alkaline phosphatase is usually very elevated, especially early on. Anti-mitochondrial antibody is positive, along with Serum IgM. Patients may have deficiencies of Vitamins A, D, E, and K, the fat soluble vitamins as bile duct destruction causes decreased elimination of bile salts necessary to absorb fat and absorb the fat soluble vitamins. Likewise, cholesterol is commonly excreted in bile. Therefore, less bile excretion, less cholesterol excretion with the result being hypercholesterolemia. Curiously, this cohort does not seem to have increased cardiovascular risk. Screening for lipids, and fat soluble vitamins should be done, as well as Dexa scan for osteoporosis. Ursodeoxycholic Acid is generally considered first line treatment.
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