Cholestatic liver diseases: Dr. Mohammad Shaikhani. MBChB- CABM- FRCP.
Primary Biliary Cirrhosis : <ul><li>PBC occurs primarily in women between 40- 60 years .  </li></ul><ul><li>The most commo...
Primary sclerosing Cholangitis : <ul><li>PSC occurs most often in men between 20 - 30   </li></ul><ul><li>Up to 80% also h...
Cholestatic liver disease: DD <ul><li>Other causes of cholestasis as: </li></ul><ul><li>Cholestatic phase of viral hepatit...
Primary Biliary Cirrhosis : EPIDEMIOLOGY <ul><li>A chronic progressive cholestatic liver disease of unknown cause.  </li><...
Primary Biliary Cirrhosis : Features <ul><li>The most common symptom is persistent fatigue, occurs in 80%.  </li></ul><ul>...
Primary Biliary Cirrhosis :Physical exam <ul><li>Include skin thickening, hyperpigmentation from repeated excoriations, xa...
Primary Biliary Cirrhosis : Diagnosis <ul><li>The diagnostic triad includes cholestatic liver profile, positive antimitoch...
Primary Biliary Cirrhosis : Treatment <ul><li>Treatment with ursodeoxycholic acid improves the biochemical profile, reduce...
PSC: Epidemiology   <ul><li>A chronic cholestatic liver disease of unknown cause characterized by progressive bile duct de...
PSC: Features   <ul><li>The most common presenting symptoms are pruritus, jaundice, abdominal pain, fatigue, although almo...
PSC: Diagnosis   <ul><li>Lab findings include a cholestatic liver profile, with SAP *3-5> normal& mild hyperbilirubinemia....
Primary Sclerosing Cholangitis: DD   <ul><li>Include bile duct surgical injury, infectious cholangitis (including AIDS cho...
PSC: Complications   <ul><li>Patients are at risk for developing cholangiocarcinoma (lifetime prevalence of 10-30%).  </li...
PSC: Management   <ul><li>Includes assessment of dominant strictures </li></ul><ul><li>treatment of superimposed bacterial...
 
 
 
 
 
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Git Cholestatic Liver Dis2010

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Git Cholestatic Liver Dis2010

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Git Cholestatic Liver Dis2010

  1. 1. Cholestatic liver diseases: Dr. Mohammad Shaikhani. MBChB- CABM- FRCP.
  2. 2. Primary Biliary Cirrhosis : <ul><li>PBC occurs primarily in women between 40- 60 years . </li></ul><ul><li>The most common symptom is persistent fatigue . </li></ul><ul><li>An antimitochondrial antibody titer of ≥ 1:40 is the serologic hallmark for the diagnosis </li></ul>
  3. 3. Primary sclerosing Cholangitis : <ul><li>PSC occurs most often in men between 20 - 30 </li></ul><ul><li>Up to 80% also have an IBD . </li></ul><ul><li>The most common symptoms are pruritus, jaundice, abdominal pain, fatigue . </li></ul><ul><li>The diagnosis is confirmed by imaging studies showing a “string of beads” pattern of the intra- & extrahepatic bile ducts . </li></ul><ul><li>Patients are at increased risk for developing cholangiocarcinoma , HCC& CRC if with IBD. </li></ul><ul><li>Liver transplantation is associated with improved quality of life and survival . </li></ul>
  4. 4. Cholestatic liver disease: DD <ul><li>Other causes of cholestasis as: </li></ul><ul><li>Cholestatic phase of viral hepatitis. </li></ul><ul><li>Drug-induced or herbals-induced cholestasis. </li></ul><ul><li>Intrahepatic cholestsis of pregnancy. </li></ul><ul><li>Alcoholic hepatitis. </li></ul><ul><li>Intrahepatic or extrahepatic biliary obstruction. </li></ul>
  5. 5. Primary Biliary Cirrhosis : EPIDEMIOLOGY <ul><li>A chronic progressive cholestatic liver disease of unknown cause. </li></ul><ul><li>It is an autoimmune disorder occurs predominantly in women (80- 90%) between 40- 60 years. </li></ul><ul><li>The prevalence has been increasing, most likely because of earlier diagnosis & increased survival. </li></ul>
  6. 6. Primary Biliary Cirrhosis : Features <ul><li>The most common symptom is persistent fatigue, occurs in 80%. </li></ul><ul><li>Either localized or general pruritus frequently develops. </li></ul><ul><li>The pruritus often begins in the perineal area or on the palmar / plantar surfaces typically worse at night or in a warm environment. </li></ul><ul><li>Jaundice / abdominal pain may also develop. </li></ul><ul><li>Many patients may be asymptomatic at presentation. </li></ul>
  7. 7. Primary Biliary Cirrhosis :Physical exam <ul><li>Include skin thickening, hyperpigmentation from repeated excoriations, xanthomas, xanthelasma,hepatomegaly. </li></ul><ul><li>Patients with advanced disease may have clinical manifestations of portal hypertension. </li></ul><ul><li>Other autoimmune diseases are frequently present. </li></ul><ul><li>Metabolic bone disease, hypercholesterolemia, fat-soluble vitamin deficiencies are common. </li></ul>
  8. 8. Primary Biliary Cirrhosis : Diagnosis <ul><li>The diagnostic triad includes cholestatic liver profile, positive antimitochondrial antibody titers&compatible histologic findings on liver biopsy. </li></ul><ul><li>SAP & γ-GT are usually elevated *10 or more above normal. </li></ul><ul><li>TSB increases as the disease progresses & a helpful prognostic marker. </li></ul><ul><li>An antimitochondrial antibody titer of ≥ 1:40 is the serologic hallmark occurs in 90-95% . </li></ul><ul><li>The titer does not appear to correlate with the severity or progression of the clinical disease. </li></ul><ul><li>The diagnosis is confirmed by liver biopsy, characteristically shows nonsuppurative cholangitis plus findings ranging from bile duct lesions to cirrhosis. </li></ul>
  9. 9. Primary Biliary Cirrhosis : Treatment <ul><li>Treatment with ursodeoxycholic acid improves the biochemical profile, reduces pruritus, decreases progression to cirrhosis, and delays the need for liver transplantation. </li></ul><ul><li>Therapy is usually continued indefinitely. </li></ul><ul><li>Liver transplantation is considered for patients with intractable pruritus or complications from cirrhosis. </li></ul><ul><li>Long-term outcomes tend be better than outcomes achieved for other indications for transplantation. </li></ul>
  10. 10. PSC: Epidemiology <ul><li>A chronic cholestatic liver disease of unknown cause characterized by progressive bile duct destruction& may lead to secondary biliary cirrhosis. </li></ul><ul><li>The disease develops more often in men than in women (3:1), generally occurs in patients 20-30 years. </li></ul><ul><li>Up to 80% have an IBD (most often ulcerative colitis), but < 5% with UC develop PSC. </li></ul>
  11. 11. PSC: Features <ul><li>The most common presenting symptoms are pruritus, jaundice, abdominal pain, fatigue, although almost 50% of patients are asymptomatic at initial diagnosis. </li></ul><ul><li>Patients with more advanced disease may present with cirrhosis & related complications. </li></ul><ul><li>Other associated disorders include bacterial cholangitis, pigmented bile stones, steatorrhea, malabsorption, metabolic bone disease. </li></ul>
  12. 12. PSC: Diagnosis <ul><li>Lab findings include a cholestatic liver profile, with SAP *3-5> normal& mild hyperbilirubinemia. </li></ul><ul><li>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. </li></ul><ul><li>Liver biopsy is usually done for staging rather than for diagnosis may show histologic findings ranging from portal hepatitis to biliary cirrhosis. </li></ul><ul><li>The classic histologic lesion, termed periductal (“onionskin”) fibrosis, is seen in only 10% of biopsy specimens. </li></ul>
  13. 13. Primary Sclerosing Cholangitis: DD <ul><li>Include bile duct surgical injury, infectious cholangitis (including AIDS cholangiopathy) &malignancy. </li></ul>
  14. 14. PSC: Complications <ul><li>Patients are at risk for developing cholangiocarcinoma (lifetime prevalence of 10-30%). </li></ul><ul><li>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. </li></ul><ul><li>Patients with advanced disease& cirrhosis are also at risk for developing HCC& patients with both PSC& UC have an increased risk of developing CRC. </li></ul><ul><li>An aggressive surveillance program for neoplasia should begin immediately in patients diagnosed with both diseases. </li></ul>
  15. 15. PSC: Management <ul><li>Includes assessment of dominant strictures </li></ul><ul><li>treatment of superimposed bacterial cholangitis </li></ul><ul><li>symptomatic therapy. </li></ul><ul><li>Only liver transplantation appears to improve overall survival & quality of life. </li></ul><ul><li>Although multiple medical therapies have been studied, to date none has provided the long-term benefits of transplantation. </li></ul><ul><li>Median survival from the time of diagnosis is 12 years. </li></ul>

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