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Cholestatic Liver Disease

Cholestatic Liver Disease. Primary Biliary Cirrhosis. Causes of Alk Phosphatase Elevation. Hepatic: cirrhosis,tumors,Steatosis,PBC, PSC, drugs, TPN Biliary: Cholangitis, Obstruction Bone: Paget’s,Hyperparathyroid, Mets Septicemia cholestasis of sepsis

jocelyn-zimmerman
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Cholestatic Liver Disease

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  1. Cholestatic Liver Disease Primary Biliary Cirrhosis

  2. Causes of Alk Phosphatase Elevation • Hepatic: cirrhosis,tumors,Steatosis,PBC, PSC, drugs, TPN • Biliary: Cholangitis, Obstruction • Bone: Paget’s,Hyperparathyroid, Mets • Septicemia cholestasis of sepsis • Physiologic puberty, pregnancy

  3. Medications • Anabolic steroids • Chlorpromazine • Erythromycin • Oral contraceptives

  4. ALP of hepatic origin • < 2 x Normal • Repeat in 4 wks • Normal ____ No action • Unchanged ___ US__ dil ducts __ ERCP CT = mets__ FNA

  5. ALP > 2 x Normal • CT …. Normal – hepatitis(immune, viral disease)… Liver biopsy CT ….. Dilated ducts, stone or mass Proceed with ERCP/ CT Bx or Surg ( Bile duct obstruction, PSC, PBC )

  6. Primary Biliary Cirrhosis • Autoimmune cholestatic disorder • Female to male ratio of 9 : 1 • Inflammatory destruction of bile ducts, leads to cholestasis and cirrhosis • Autoantibodies reactive with antigens on the surface of biliary epithelium • Antimitochondrial antibodies

  7. PBC: Symptoms and Signs • Fatigue 70 % • Pruritis 55 • Jaundice 10 • Hepatomegaly 25 • Splenomegall 15 • Xanthelasma 10 • None 25

  8. PBC: Associated Diseass • Keartoconjuntivitis sicca 75% • Arthritis 4 – 40 • Scleroderma 15 • Thyroiditis 20 • RTA 50 • Gallstones 33

  9. Diag: Liver Profile • Alk phosphatase elevation • AST / ALT less than 5 x N • Increase in bilirubin, albumin and prothrombin time = poor prognosis

  10. Diag: Serology • Antimitochondrial antibodies 90-95% • M2 antibody 98 % • Rh factor 70 • Anti smooth muscle 66 • Antithyroid 41 • ANA 35

  11. Liver Histology • Ludwig’s Classification Stage I. Inflammation within portal space Focussed on bile ducts Stage II. Inflam extending into hepatic parenchyma Stage III. Fibrosis Stage IV. Regenerative nodules ( cirrhosis)

  12. Natural History: Asymptomatic PBC • Mitchison et al 1986 • 29 patients with normal LFT’s • Followed for 17.8 years (11 – 24 yrs) • Abnormal LFT’s in 5.6 yrs • Symptoms developed in 76 %

  13. Natural History: Symptomatic PBC Once symptoms develop, life expectancy falls sigficantly, with a median survival time of approximately 10 years.

  14. Prediction of SurvivalMayo Risk Score • Advanced age • Serum bilirubin • Serum albumin • Fluid retention ( ascites and edema) • Variceal bleeding • Advanced histologic stage • Bilirubin > 10 mg = Life expectancy < 2 yrs

  15. Medical Treatment • Glucocorticoids • Budeonide • D-Penicillamine • Colchicene no improvent in Azathioprine survival • Cyclosporine • Methotrexate

  16. treatment • Ursodeoxycholic acid • Small quantities in bile --- < 4 % • Endogenous Bile acids ( cholic, cheno, litho) hepatoxic • Treatment increases Urso content to 30-60% • Level of Urso parallels improvement in liver profile and Mayo Risk Score • Dose : 13 – 15 mg/kg/day • If pt on Cholestyramine, Urso to be taken 2 hrs before or after cholestyramine administration

  17. PBC: Complications • Bone Disease: osteoporosis and fractures vitamin D 25,000 to 50,000 iu / week • Fat-Soluble Vit Deficiency vit A 25,000 IU 3/WK vit K 5 mg/d vit E 50 – 200 u / d • Steatorrhea Low fat diet, substitute MCT • Hypercholestrolemia RX Urso • Pruritis Cholystyramine, Rifampin, liver transplant.

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