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

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Autoimmune & Cholestatic liver diseases:

Dr. Mohammad Shaikhani.MBChB- CABM- FRCP-EBGH.

2017 update

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Intrahepatic Cholestasis:•Jaundice.

•High TSB, More direct.•Liver enzymes elevated.

•Elevated SAP.•No extrahepatic obstruction on imaging.

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

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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.

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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

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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BO5:6•6 .Good prognostic marker of PBC is:

•A. AMA titer.•B. TSB.•C. SAP.

•D. Liver enzymes level.•E. Pruritus.

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BO5:7•7 .Pregnancy-related liver diseases include all

except:•A. Hypermesis.

•B. Toxemia.•C. Intrahepatic cholestasis.

•D. Acute fatty liver.•E. PBC.

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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.

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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.

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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.

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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.

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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.

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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.