Primary Biliary Cirrhosis ⭐
Also written as PBC.
Topics tested: jaundice and liver tests, alcoholic liver disease, hepatitis B and C, primary biliary cirrhosis, NASH, Child-Pugh score, GI bleeding, acute pancreatitis, bone marrow failure, haemophilia and myelodysplasia.
Primary Biliary Cirrhosis (PBC) ⭐
- Autoimmune disease - Lymphocyte-mediated destruction of intrahepatic bile duct epithelium - Chronic progressive + often fatal cholestatic liver disease - Characterized by destruction of intrahepatic bile ducts + portal inflammation + scarri
- Demographics: - Middle-aged women (onset 20-80 yrs) ⭐ - Insidious onset
- Clinical Course: - Pruritis (early) → later jaundice appears - Cirrhosis + hepatic failure develops over 2 or more decades ⭐ - Signs of portal hypertension + hepatic encephalopathy
- Lab: - ↑ Alk. phosphatase + cholesterol - Bilirubin = late development indicating hepatic decompensation ⭐
- Autoantibody: - Anti-mitochondrial Ab in 90% of patients ⭐ - Target: mitochondrial pyruvate dehydrogenase ⭐
Read this in Liver Histology & Physiology
Primary Biliary Cirrhosis (PBC) ⭐
- Autoimmune disease - Lymphocyte-mediated destruction of intrahepatic bile duct epithelium - Chronic progressive + often fatal cholestatic liver disease - Characterized by destruction of intrahepatic bile ducts + portal inflammation + scarri
- Demographics: - Middle-aged women (onset 20-80 yrs) ⭐ - Insidious onset
- Clinical Course: - Pruritis (early) → later jaundice appears - Cirrhosis + hepatic failure develops over 2 or more decades ⭐ - Signs of portal hypertension + hepatic encephalopathy
- Lab: - ↑ Alk. phosphatase + cholesterol - Bilirubin = late development indicating hepatic decompensation ⭐
- Autoantibody: - Anti-mitochondrial Ab in 90% of patients ⭐ - Target: mitochondrial pyruvate dehydrogenase ⭐
Read this in Liver Histology & Physiology
What our notes say
- Topics tested: jaundice and liver tests, alcoholic liver disease, hepatitis B and C, primary biliary cirrhosis, NASH, Child-Pugh score, GI bleeding, acute pancreatitis, bone marrow failure, haemophilia and myelodysplasia. (Internal Medicine CAT 1 (MBChB 4), 2022: liver, pancreas and bone marrow, 30 MCQ)
- Primary biliary cirrhosis begins by damaging small bile ducts, giving a pre-sinusoidal block early on. (Internal Medicine 4th Year 1st Semester CAT: HIV and the gut, liver disease and )
- Immunoglobulins: Increased in chronic liver diseaseIgM: Primary biliary cirrhosis (KIDNEY FUNCTION TESTS & GASTRIC FUNCTION)
- Immunoglobulins ↑ in chronic liver disease due to GIT antigen shunting:IgM ↑ → Primary biliary cirrhosis (Liver Function Tests (LFTs))