Primary Biliary Cholangitis

Pathogenesis

100

Primary biliary cholangitis is inflammation of the _____ bile ducts

small

200

What type of cells drive the immune mediated attack in primary biliary cholangitis?

T-lymphocytes

300

How might hepatocytes become affected in PBC?

Destruction of bile ducts → cholestasis → increased concentration of bile acids destroys hepatocytes

400

What are the four histopathological findings consistent with PBC? (biopsy slide)

1. florid bile duct 

2. destruction of bile duct

3. inflammatory infiltrate

4. granuloma

500

______ antibodies target the ______ subunit of the ______ to kickstart the pathogenesis of PBC

Antimitochondrial antibodies (AMAs) target the E2 subunit of the pyruvate dehydrogenase complex

Diagnosis

100

What are three findings needed to diagnose PBC?

Elevated AP, Positive AMA, Histopathological findings

200

 Common symptoms of PBC

Pruritus (due to buildup of bile), fatigue, over 50% asymptomatic

300

Name two auto-immune diseases associated with PBC

Sjogren’s and Thyroid Disease

400

Which IBD (UC or Crohn’s) usually presents with PBC?

Neither, UC typically presents with PSC (trick question)

500

All serological elevations seen in PBC

Elevated AP, bilirubin, and transaminases

With corresponding positive AMA and ANA. May also see elevated LDL and HDL

Treatment

100

What is the cure for PBC?

There is no cure

200

First-line medical therapy for PBC?

Ursodeoxycholic acid 13-15 mg/kg per day

300

What is the only definitive treatment for PBC?

Liver Transplantation

400

What is the active ingredient in Ursodeoxycholic acid?

Hydrophilic bile acid

500

Fibrates may be used for management of PBC. What is the mechanism of action of this drug class?

Activation of the peroxisome proliferator-activated receptor alpha (PPAR–α) → ↑ lipoprotein lipase activity → more rapid degradation of LDL and triglycerides and induction of HDL synthesis → ↓ LDL, ↑ HDL, ↓↓↓ triglyceride