GIT Autoimmune cholestatic liver diseases for 4th year medical college.

1. Autoimmune cholestatic liver
diseases:
Dr. Mohammad Shaikhani.
Professor
MBChB- CABM- FRCP-EBGH.

2. Intrahepatic Cholestatic liver
diseases:
• Increased TSB mainly direct.
• Increased SAP.
• Liver enzymes may be elevated.
• No extra-hepatic obstruction.

3. Cholestatic liver diseases:DD
• PBC,PBC-AIH overlap, PSC:
• Cholestatic phase of viral hepatitis.
• Drug or herbals-induced cholestasis.
• Intrahepatic cholestsis of pregnancy.
• Alcoholic hepatitis.
• Intra or extrahepatic biliary obstruction.

5. Features PBC PSC AIH
AGE/Gender PBC occurs primarily
in women between 40-
60 years.
PSC occurs most
often in men
between 20 – 30
Young women
Symptoms The most common
symptom is persistent
fatigue.
80% have an IBD.
The most common
symptoms are
pruritus, jaundice,
abdominal pain,
fatigue.
Mainly Jaundice.
Diagnosis An antimitochondrial
Abs of ≥1:40 is
hallmark for diagnosis
confirmed by liver
biopsy, shows
nonsuppurative
The diagnosis is
confirmed by
MRCP/ERCP :
“string of beads”
pattern of the intra
& extrahepatic
ANF,Anti-sm,Anti
kidney-liver Ags.
Confirmed by liver
biopsy.

6. Features PBC PSC AIH
Complications Patients are at
increased risk for
metabolic bone
diseases.
Patients are at
increased risk for
developing
cholangiocarcinom
a , HCC& CRC if
with IBD.
Risk of Cirrhosis
Treatment: Ursodeoxycholic acid
teatment imroves liver
functions.
Liver
transplantation is
associated with
improved quality
of life& survival.
Steroids &
immunosuppressa
nts.
Features: Either localized or
general pruritus
frequently develops.
Jaundice / abdominal
pain may also develop.
Same. Jaundice,pruritis,
amenorrhea,acne.

8. Primary Biliary Cirrhosis: Diagnosis
• The diagnostic triad includes cholestatic liver profile, positive
antimitochondrial antibody titers&compatible histologic findings
on liver biopsy.
• SAP & γ-GT are usually elevated *10 or more above normal.
• TSB increases as the disease progresses & a helpful prognostic
marker.
• An antimitochondrial antibody titer of ≥1:40 is the serologic
hallmark occurs in 90-95% .
• The titer does not appear to correlate with the severity or
progression of the clinical disease.

9. Primary Biliary Cirrhosis: Treatment
• Treatment with ursodeoxycholic acid improves the biochemical
profile, reduces pruritus, decreases progression to cirrhosis, and
delays the need for liver transplantation.
• Therapy is usually continued indefinitely.
• Liver transplantation is considered for patients with intractable
pruritus or complications from cirrhosis.
• Long-term outcomes tend be better than outcomes achieved for
other indications for transplantation.

10. PSC: Epidemiology
• A chronic cholestatic liver disease of unknown cause
characterized by progressive bile duct destruction& may lead to
secondary biliary cirrhosis.
• Up to 80% have an IBD (most often ulcerative colitis), but < 5%
with UC develop PSC.

11. PSC: Diagnosis
• Liver biopsy is usually done for staging rather than for diagnosis
may show histologic findings ranging from portal hepatitis to
biliary cirrhosis.
• The classic histologic lesion, termed periductal (“onionskin”)
fibrosis, is seen in only 10% of biopsy specimens.

12. Primary Sclerosing Cholangitis: DD
• Include bile duct surgical injury, infectious cholangitis (including
AIDS cholangiopathy) &malignancy.

13. PSC: Management
• Includes assessment of dominant strictures
• Treatment of superimposed bacterial cholangitis
• symptomatic therapy.
• Only liver transplantation appears to improve overall survival &
quality of life.
• Median survival from the time of diagnosis is 12 years.