Primary sclerosing cholangitis is a disease that causes inflammation and scarring of the bile ducts, resulting in liver damage over time. It has no known cause but may be triggered by an immune reaction in genetically predisposed individuals. Symptoms include fatigue and itching initially, with potential later complications of liver failure, infection, and cancer. Diagnosis involves blood tests, MRI or other imaging of the bile ducts, and potentially a liver biopsy. While there is no cure, treatments focus on managing complications through antibiotics, procedures to open blocked ducts, and potentially liver transplantation.

1. Primary sclerosing cholangitis
Justin V Sebastian

2. • Primary sclerosing cholangitis is a disease of
the bile ducts. Bile ducts carry the digestive
liquid bile from liver to small intestine.
• In primary sclerosing cholangitis,
in
fl
ammation causes scars within the bile
ducts. These scars make the ducts hard and
narrow and gradually cause serious liver
damage
.
• In most people with primary sclerosing
cholangitis, the disease progresses slowly. It
can eventually lead to liver failure, repeated
infections, and tumors of the bile duct or liver.

3. Cause
s
• It's not clear what causes primary sclerosing cholangitis.
An immune system reaction to an infection or toxin may
trigger the disease in people who are genetically
predisposed to it
.
• A large proportion of people with primary sclerosing
cholangitis also have in
fl
ammatory bowel disease, an
umbrella term that includes ulcerative colitis and Crohn's
disease.

4. Risk factor
s
Factors that may increase the risk of primary sclerosing
cholangitis include
:
•
In
fl
ammatory bowel disease. A large proportion of
people with primary sclerosing cholangitis also have
in
fl
ammatory bowel disease
.
•
Geographical location. People with Northern
European heritage have a higher risk of primary
sclerosing cholangitis.

5. Symptom
s
Early symptoms often include
:
•
Fatigue
•
Itchin
g
Signs and symptoms that may appear as the disease
progresses include
:
•
Pain in the upper right part of the abdome
n
•
Feve
r
•
Chill
s
•
Enlarged live
r
•
Enlarged splee
n
•
Weight los
s
•
Yellow eyes and skin (jaundice)

6. Complication
s
Complications of primary sclerosing cholangitis
may include
:
•
Liver disease and failure. Chronic in
fl
ammation
of the bile ducts throughout liver can lead to tissue
scarring (cirrhosis), liver cell death and,
eventually, loss of liver function
.
•
Repeated infections. If scarring of the bile ducts
slows or stops the
fl
ow of bile out of the liver, may
experience frequent infections in the bile ducts
.
•
Portal hypertension. It can also divert blood
from the portal vein to other veins, causing these
veins to become swollen (varices).

7. •
Thinning bones. People with primary sclerosing
cholangitis may experience thinning bones
(osteoporosis)
.
•
Bile duct cancer. If there primary sclerosing
cholangitis, have an increased risk of developing
cancer in the bile ducts
.
•
Colon cancer. People with primary sclerosing
cholangitis associated with in
fl
ammatory bowel
disease have an increased risk of colon cancer. If
patient have been diagnosed with primary
sclerosing cholangitis, doctor may recommend
testing for in
fl
ammatory bowel disease, even if
patient have no signs or symptoms.

8. Diagnosi
s
•
Liver function blood tests. A blood test to
check liver function, including levels of liver
enzymes
.
•
MRI of bile ducts. Magnetic resonance
cholangiopancreatography uses magnetic
resonance imaging (MRI) to make images of
liver and bile ducts and is the test of choice to
diagnose primary sclerosing cholangitis
.
•
X-rays of bile ducts. A type of bile duct X-ray
c a l l e d e n d o s c o p i c r e t r o g r a d e
cholangiopancreatography (ERCP) in addition
to, or instead of, an MRI may be needed. To
make bile ducts visible on an X-ray, doctor uses
a
fl
exible tube passed down throat to inject dye
into the area of small intestine where bile ducts
empty
.
• Testing a sample of liver tissue. A liver biopsy
is a procedure to remove a piece of liver tissue
for laboratory testing.

9. Treatmen
t
Treatments for primary sclerosing cholangitis focus on managing
complications and monitoring liver damage. Many medications have been
studied in people with primary sclerosing cholangitis, but so far none have
been found to slow or reverse the liver damage associated with this disease
.
Treatment for itchin
g
•
Bile acid sequestrants. Medications that bind to bile acids — the
substances thought to cause itching in liver disease — are the
fi
rst line
treatment for itching in primary sclerosing cholangitis. Eg
cholestyramine
.
•
Antibiotics. If there trouble tolerating a bile acid-binding drug or if it
doesn't help, doctor may prescribe rifampin (Rifadin, Rimactane, others),
an antibacterial drug. Exactly how rifampin reduces itching is unknown,
but it may block the brain's response to itch-inducing chemicals in
circulation
.
•
Opioid antagonists. Itching related to liver disease may also respond to
opioid antagonist drugs, such as naltrexone. Like rifampin, these drugs
seem to reduce the itch sensation by acting on brain.

10. Treatment for infection
s
Bile that backs up in narrowed or blocked ducts causes frequent bacterial
infections. To prevent and treat these infections, people with primary sclerosing
cholangitis may take repeated courses of antibiotics or continue taking
antibiotics for long periods
.
Treatment for bile duct blockage
s
Blockages that occur in bile ducts may be due to disease progression but can be
a sign of cancer of the bile duct. Endoscopic retrograde
cholangiopancreatography (ERCP) can help determine the cause, and bile duct
blockage can be treated with
:
•
Balloon dilation. In balloon dilation, doctor runs a slender tube with an
in
fl
atable balloon at its tip (balloon catheter) through an endoscope and into a
blocked bile duct. Once the balloon catheter is in place, the balloon is in
fl
ated
.
•
Stent placement. In this procedure, doctor uses an endoscope and attached
instruments to place a small plastic tube called a stent in a blocked bile duct to
hold the duct open
.
Liver transplan
t
A liver transplant is the only treatment known to cure primary sclerosing
cholangitis. During a liver transplant, surgeons remove diseased liver and
replace it with a healthy liver from a donor.