Primary Biliary Cirrhosis: Symptoms, Laboratory Findings, Diagnosis, and Treatment

What Is Primary Biliary Cirrhosis?

Primary Biliary Cirrhosis is a slowly progressive autoimmune disease involving the destruction of small intrahepatic bile ducts. It most commonly affects middle-aged women.

Common Symptoms of Primary Biliary Cirrhosis

Symptoms usually include fatigue and unexplained pruritis, that is poorly responsive to anti-pruritics.

Laboratory Findings in Primary Biliary Cirrhosis

1. Alkaline phosphatase is usually very elevated, especially early on.
2. Anti-mitochondrial antibody is positive, along with Serum IgM.

Fat-Soluble Vitamin Deficiencies in Primary Biliary Cirrhosis

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.

Hypercholesterolemia and Cardiovascular Risk in Primary Biliary Cirrhosis

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.

Recommended Screening for Primary Biliary Cirrhosis Complications

Screening for:

1. Lipids
2. Fat soluble vitamins
3. Dexa scan for osteoporosis

First-Line Treatment for Primary Biliary Cirrhosis

Ursodeoxycholic Acid is generally considered first line treatment.