Autoimmune cholestatic liver diseases:

Dr. Mohammad Shaikhani.

Professor

MBChB- CABM- FRCP-EBGH.

Intrahepatic Cholestatic liver diseases:

• Increased TSB mainly direct.• Increased SAP.• Liver enzymes may be elevated.• No extra-hepatic obstruction.

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.

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 diagnosisconfirmed by liver biopsy, shows nonsuppurative cholangitis ranging from bile duct lesions

to cirrhosis.

The diagnosis is confirmed by MRCP/ERCP : “string of beads” pattern of the intra & extrahepatic

bile ducts.

ANF,Anti-sm,Anti kidney-liver Ags.

Confirmed by liver biopsy.

Features PBC PSC AIH

Complications Patients are at increased risk for metabolic bone diseases.

Patients are at increased risk for developing cholangiocarcinoma , 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 & immunosuppressants.

Features: Either localized or general pruritus

frequently develops. Jaundice / abdominal

pain may also develop.

Same. Jaundice,pruritis,amenorrhea,acne.

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.

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.

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.

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.

Primary Sclerosing Cholangitis: DD • Include bile duct surgical injury, infectious cholangitis (including

AIDS cholangiopathy) &malignancy.

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.