Git Cholestatic Liver Dis2010
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Git Cholestatic Liver Dis2010

Git Cholestatic Liver Dis2010

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Git Cholestatic Liver Dis2010 Presentation Transcript

  • 1. Cholestatic liver diseases: Dr. Mohammad Shaikhani. MBChB- CABM- FRCP.
  • 2. Primary Biliary Cirrhosis :
    • 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
  • 3. Primary sclerosing Cholangitis :
    • PSC occurs most often in men between 20 - 30
    • Up to 80% also have an IBD .
    • The most common symptoms are pruritus, jaundice, abdominal pain, fatigue .
    • The diagnosis is confirmed by imaging studies showing a “string of beads” pattern of the intra- & extrahepatic bile ducts .
    • Patients are at increased risk for developing cholangiocarcinoma , HCC& CRC if with IBD.
    • Liver transplantation is associated with improved quality of life and survival .
  • 4. Cholestatic liver disease: DD
    • Other causes of cholestasis as:
    • Cholestatic phase of viral hepatitis.
    • Drug-induced or herbals-induced cholestasis.
    • Intrahepatic cholestsis of pregnancy.
    • Alcoholic hepatitis.
    • Intrahepatic or extrahepatic biliary obstruction.
  • 5. Primary Biliary Cirrhosis : 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.
  • 6. Primary Biliary Cirrhosis : 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.
  • 7. Primary Biliary Cirrhosis :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.
  • 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.
    • The diagnosis is confirmed by liver biopsy, characteristically shows nonsuppurative cholangitis plus findings ranging from bile duct lesions to cirrhosis.
  • 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.
    • 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.
  • 11. 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.
  • 12. 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.
  • 13. Primary Sclerosing Cholangitis: DD
    • Include bile duct surgical injury, infectious cholangitis (including AIDS cholangiopathy) &malignancy.
  • 14. PSC: Complications
    • Patients are at risk for developing cholangiocarcinoma (lifetime prevalence of 10-30%).
    • Detecting this malignancy at an early stage is difficult despite the availability of studies for the tumor markers CA 19-9/ CEA ( alone or in combination), cytologic sampling&advanced imagings.
    • Patients with advanced disease& cirrhosis are also at risk for developing HCC& patients with both PSC& UC have an increased risk of developing CRC.
    • An aggressive surveillance program for neoplasia should begin immediately in patients diagnosed with both diseases.
  • 15. 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.
    • Although multiple medical therapies have been studied, to date none has provided the long-term benefits of transplantation.
    • Median survival from the time of diagnosis is 12 years.
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