GIT Cholestatic AI HBD 17

1. Autoimmune & Cholestatic liver
diseases:
Dr. Mohammad Shaikhani.
MBChB- CABM- FRCP-EBGH.
2017update

4. Intrahepatic Cholestasis:
•Jaundice.
•High TSB, More direct.
•Liver enzymes elevated.
•Elevated SAP.
•No extrahepatic obstruction on imaging.

5. AI Cholestatic liver disease:
• PBC.
• PSC.
• IG4-related pancreatobiliary disease.
• AIH.

6. AI Cholestatic liver disease: DD
• Medical cholestasis:
• Cholestatic phase of viral hepatitis.
• Drug-induced cholestasis.
• Herbals-induced cholestasis.
• Intrahepatic cholestasis of pregnancy.
• Alcoholic hepatitis.
• Metabolic causes as Wilson disease.
• Surgical cholestasis:
• Intrahepatic or extrahepatic biliary obstruction on imaging.

7. Primary Biliary Cirrhosis (Now cholangitis( :
• PBC occurs primarily in women between 40 - 60 years.
• The most common symptom is persistent fatigue.
• An antimitochondrial antibody titer of ≥1:40 is the serologic
hallmark for the diagnosis

8. Primary Biliary cholangitis: EPIDEMIOLOGY
• A chronic progressive cholestatic liver disease of unknown cause.
• It is an autoimmune disorder occurs predominantly in women
(80- 90%( between 40- 60 years.
• The prevalence has been increasing, most likely because of
earlier diagnosis & increased survival.

9. Primary Biliary cholangitis: Features
• The most common symptom is persistent fatigue, occurs in 80%.
• Either localized or general pruritus frequently develops.
• The pruritus often begins in the perineal area or on the palmar /
plantar surfaces typically worse at night or in a warm
environment.
• Jaundice / abdominal pain may also develop.
• Many patients may be asymptomatic at presentation.

10. Primary Biliary cholangitis:Physical exam
• Include skin thickening, hyperpigmentation from repeated
excoriations, xanthomas, xanthelasma,hepatomegaly.
• Patients with advanced disease may have clinical manifestations
of portal hypertension.
• Other autoimmune diseases are frequently present.
• Metabolic bone disease, hypercholesterolemia, fat-soluble vitamin
deficiencies are common.

11. Primary Biliary cholangitis: 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.
• The diagnosis is confirmed by liver biopsy, characteristically
shows nonsuppurative cholangitis plus findings ranging from bile
duct lesions to cirrhosis.

12. Primary Biliary cholangitis: Treatment
• Ursodeoxycholic acid improves the biochemical profile, reduces
pruritus, decreases progression to cirrhosis&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.

13. PSC: Epidemiology
• A chronic cholestatic liver disease of unknown cause
characterized by progressive bile duct destruction& may lead to
secondary biliary cirrhosis.
• The disease develops more often in men than in women (3:1),
generally occurs in patients 20-30 years.
• Up to 80% have an IBD (most often ulcerative colitis), but < 5%
with UC develop PSC.

14. PSC: Features
• The most common presenting symptoms are pruritus, jaundice,
abdominal pain, fatigue, although almost 50% of patients are
asymptomatic at initial diagnosis.
• Patients with more advanced disease may present with cirrhosis
& related complications.
• Other associated disorders include bacterial cholangitis,
pigmented bile stones, steatorrhea, malabsorption, metabolic
bone disease.

15. PSC: Diagnosis
• Lab findings include a cholestatic liver profile, with SAP *3-5>
normal& mild hyperbilirubinemia.
• The diagnosis is confirmed by ERCP or MRCP that shows
findings of multifocal strictures / dilatation of the intra&
extrahepatic bile ducts, resembling beads on a string.
• 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.

16. PSC: Cholangiography
MRCP ERCP

17. Primary Sclerosing Cholangitis: DD
• Bile duct surgical injury.
• Infectious cholangitis (including AIDS cholangiopathy)
• Malignancy.

18. PSC: Complications
• Cholangiocarcinoma CC ( 10-30%).
• Detecting CC at an early stage is difficult.
• Tumor markers CA 19-9+/- CEA
• Cytologic sampling through ERCP or cholangioscopy.
• Patients with advanced disease& cirrhosis are at risk for HCC.
• Patients with both PSC& UC have an increased risk of CRC&
aggressive surveillance needed.

19. PSC: Management
• Includes assessment & management of dominant strictures
• Treatment of superimposed bacterial cholangitis
• Symptomatic therapy.
• Only liver transplantation improves overall survival & quality of
life.
• Up to date no TRT has provided the long-term benefits of
transplantation.
• Median survival from the time of diagnosis is 12 years.

29. BO5:1
•1.The main point in differentiating intrahepatic from
extra-hepatic cholestasis is:
•A. TSB.
•B. SAP.
•C. Liver enzymes.
•D. Obstruction on imaging.
•E. Fever.

30. BO5:2
•2.Autoimmune cholestatic liver diseases include:
•A. Wilson disease.
•B. Alcholoic liver disease.
•C. Viral-induced cholestasis.
•D. Primary biliary & primary sclerosing cholangitis.
•E. Drug-induced cholangitis.

31. BO5:3
•3.Characteristic features of BPC include all except:
•A. More in women.
•B. More in middle ages.
•C. High anti-mitochondrial antibodies.
•D. Associated with IBD.
•E. Response to Ursodeoxycholic acid.

32. BO5:4
•4.PSC is characterized by all except:
•A. More in women.
•B. More in youngs.
•C. Predisposes to cholangiocarcinoma.
•D. Associated with IBD.
•E. No response to Ursodeoxycholic acid.

33. BO5:5
•5.PBC differs from PSC by:
•A. Occurring in young males.
•B. Causing intrahepatic cholestasis.
•C. Responding to ursodeoxycholic acid.
•D. Causing pruritus.
•E. High serum alkaline phosphatase.

34. BO5:6
•6.Good prognostic marker of PBC is:
•A. AMA titer.
•B. TSB.
•C. SAP.
•D. Liver enzymes level.
•E. Pruritus.

35. BO5:7
•7.Pregnancy-related liver diseases include all
except:
•A. Hypermesis.
•B. Toxemia.
•C. Intrahepatic cholestasis.
•D. Acute fatty liver.
•E. PBC.

36. BO5:8
•8.The following lab tests are abnormal in normal
pregnancy except:
•A. WBC.
•B. SAP.
•C. Albumin.
•D. Liver enzymes.
•E. Alpha feto protein.

37. BO5:9
•9.The most common pregnancy-related liver
disease is:
•A. Acute fatty liver of pregnancy.
•B. Hyperemesis gravidarum.
•C. Toxemia of pregnancy.
•D. Intrahepatic cholestsis of pregnancy.
•E. HELP syndrome.

38. BO5:10
•10.The most seious pregnancy-related liver disease
is:
•A. Acute fatty liver of pregnancy.
•B. Hyperemesis gravidarum.
•C. Toxemia of pregnancy.
•D. Intrahepatic cholestsis of pregnancy.
•E. HELP syndrome.

39. BO5:11
•11.The most benign pregnancy-related liver disease
is:
•A. Acute fatty liver of pregnancy.
•B. Hyperemesis gravidarum.
•C. Toxemia of pregnancy.
•D. Intrahepatic cholestsis of pregnancy.
•E. HELP syndrome.

40. BO5:12
•12.The following pregnancy-related liver diseases
coexist together except:
•A. Acute fatty liver of pregnancy.
•B. Intrahepatic cholestasis of pregnancy.
•C. Toxemia of pregnancy.
•D. HELP syndrome.
•E. All of the above.

41. BO5:13
•13.The following pregnancy-related liver diseases
occur in late pregnancy trimesters except:
•A. Acute fatty liver of pregnancy.
•B. Intrahepatic cholestasis of pregnancy.
•C. Hyperemesis gravidarum.
•D. HELP syndrome.
•E. Eclampsia.