Lecture 4

Immunology
16-10-2022
Autoimmune liver diseases:
PBC - primary biliary cirrhosis
And
PSC - primary sclerosing cholangitis
•Cholestasis: decrease in bile flow due to
decrease in production by hepatocytes or
obstruction in bile ducts; the obstruction is either
in intrahepatic bile ducts or extrahepatic bile
ducts.
•Primary biliary cirrhosis and primary sclerosing
cholangitis are two examples of cholestatic liver
diseases.
•In both diseases, there is inflammation of bile
ducts (also called cholangitis), causes obstruction
of bile ducts, then accumulation of bile in liver
and bile ducts casing destruction of the
hepatocytes, then liver inflammation, then liver
fibrosis and liver cirrhosis.
 Comparison between PSC and PBC

Primary sclerosing
Feature Primary biliary cirrhosis
cholangitis
Chronic inflammatory, Chronic inflammatory,
organ specific organ specific
Definition
autoimmune, cholestatic autoimmune, cholestatic
liver disease liver disease
Comparison between PSC and PBC
Primary sclerosing
Feature Primary biliary cirrhosis
cholangitis

Unknown, mostly Unknown, mostly

Causes
autoimmune components autoimmune components
 Comparison between PSC and PBC
Feature Primary sclerosing cholangitis Primary biliary cirrhosis

Inflammation and sclerosis of bile

ducts inside and outside the
liver;
Inflammation of Small bile ducts
Site of lesion however in small duct PSC
inside the liver only
(10-15%) of patients only the
small ducts inside the liver are
affected
 Comparison between PSC and PBC
Feature Primary sclerosing cholangitis Primary biliary cirrhosis

1. Elevation of alkaline phosphatase

and elevation of transaminases 1. Elevation of alkaline
(ALT, AST). phosphatase while ALT, AST are
2. Elevated IgM in 50% may be seen. normal or slightly elevated.
3. Anti-nuclear antibodies may be 2. Elevated serum IgM
Mode of
positive. 3. Anti-nuclear antibodies may be
diagnosis
4. Cholangiography is needed, positive.
cholangiogram typically shows 4. Positive disease specific
multiple strictures of the intrahepatic antibodies (anti-mitochondrial
and extrahepatic biliary tree. antibodies AMA).
 Comparison between PSC and PBC

Primary sclerosing Primary biliary

Feature
cholangitis cirrhosis

Associated with
increased risk of bile Yes No
duct and colon cancer
 Comparison between PSC and PBC

Primary sclerosing Primary biliary

Feature
cholangitis cirrhosis

Co-existing Around 80% of patients Very rare and not

inflammatory bowel have IBD characteristic;
disease (IBD) - mostly ulcerative colitis around 1%
 Comparison between PSC and PBC

Primary sclerosing Primary biliary

Feature
cholangitis cirrhosis

Common symptoms Itching, jaundice, Itching, jaundice,

in early disease fatigue, fatigue,
abdominal pain abdominal pain
 Comparison between PSC and PBC

Primary sclerosing Primary biliary

Feature
cholangitis cirrhosis

Gender 60% men, 90% women,

predominance 40% women 10% men
 Comparison between PSC and PBC

Primary sclerosing Primary biliary

Feature
cholangitis cirrhosis

Average age
Mostly around 40 years Mostly over 40 years
at diagnosis
Comparison between PSC and PBC

Primary sclerosing Primary biliary

Feature
cholangitis cirrhosis

Associated
with excess
No Yes
alcohol
consumption?
Comparison between PSC and PBC

Primary sclerosing Primary biliary

Feature
cholangitis cirrhosis

Associated
Associated with a
with Most often non-smokers
history of smoking
smoking
 Comparison between PSC and PBC
Feature Primary sclerosing cholangitis Primary biliary cirrhosis

1. Patients who are asymptomatic at

the time of diagnosis are better than 1. Asymptomatic patients have
those who are symptomatic, but the a higher rate of survival
disease tends to progress (bad than symptomatic patients.
Prognosis
prognosis) in either case. 2. Some end with liver
2. The average overall survival time is cirrhosis after 15-20 years
approximately 10 years from the and then death.
date of diagnosis.