PBC&PSC primary biliary chriosis primary sclerosing cholangitis .pptx

PBC&PSC primary biliary chriosis

1. Primary Biliary Cirrhosis & Primary Sclerosing Cholangitis Maryam Naveed Roll no 99

2. It is a chronic, progressive cholestatic liver disease of unknown cause, possibly autoimmune, characterized by destruction of intrahepatic bile ducts. Primary Biliary Cirrhosis

3. Clinical Features; .Most commonly presents in middle-aged women ( female:male is 6:1) .Pruritus (most common initial complaint) .Jaundice . Osteomalacia (due to fat-soluble vitamin deficiency: A, D, E, K) .Osteoporosis .Skin xanthomas (focal accumulations of cholesterol) .Hepatomegaly & Splenomegaly >Most common cause of death: liver failure

4. Associations; Sjogren's syndrome Raynaud's phenomenon Celiac disease Systemic sclerosis Associated with AMA +

5. Diagnosis; . Elevated serum cholesterol . Cholestatic jaundice (i.e., conjugated hyperbilirubinemia) . Elevated alkaline phosphatase (ALP) . Elevated gamma- glutamyltransferase (GGT) . Increased serum IgM . Anti-mitochondrial antibody (AMA): Positive in >95% of cases, highly sensitive and specific; if positive, no need for biopsy LIVER BIOPSY : .lymphocytic infiltrate in portal tracts . granulomatous destruction of bile ducts

6. Treatment Ursodeoxycholic acid (slows progression of disease; mainstay of treatment) Pruritus: First-line agent: Cholestyramine Second-line agents: Rifampicin, Naltrexone, Plasmapheresis & liver support device Fat-soluble vitamin supplementation Calcium and vitamin D for osteoporosis Liver transplantation (definitive treatment)

7. Primary Sclerosing Cholangitis :- It is a chronic, progressive cholestatic liver disease of unknown cause, possibly autoimmune, characterized by progressive inflammation, fibrosis, and strictures of extrahepatic and intrahepatic bile ducts

8. Clinical Features: . Most commonly presents in young males (20-50 years; female:male is 1:2) Progressive jaundice, pruritus, and fatigue Increased risk for cholangiocarcinoma and gallbladder cancer.

9. Associations Ulcerative colitis (most common) Crohn's disease (much rarer) Retro-peritoneal fibrosis Chronic pancreatitis HIV infection Associated with MPO-ANCA/p-ANA +

10. Diagnosis Cholestatic jaundice (i.e., conjugated hyperbilirubinemia) Elevated alkaline phosphatase (ALP) Elevated gamma- glutamyltransferase (GGT) MRCP/ERCP: Beading appearance (multiple areas of bile duct strictures and dilations) Liver Biopsy: Periductal portal tract “onion-skin” fibrosis and inflammation

11. Management Ursodeoxycholic acid-improves cholestasis Cholestyramine-for pruritus Antibiotics for bacterial cholangitis Fat-soluble vitamin replacement. Yearly ultrasound to detect cholangiocarcinoma Liver transplantation-definitive treatment