Primary Biliary Cirrhosis (PBC) - Summary PBC Epidemiology: • Female:Male 9:1 • Common European descent • Age: 30-65 years PBC Pathophysiology: • T lymphocyte mediated attack on small intralobular bile ducts • Loss of intralobular bile ducts causes cholestasis → Cirrhosis/Liver failure PBC Signs and Symptoms: • 50-60% are asymptomatic • Pruritus • Fatigue PBC Physical Examination: • Skin hyperpigmentation • Jaundice (later manifestation) • Xanthomas • Hepatosplenomegaly • Spider nevi • Temporal/Proximal limb muscle wasting PBC Labs: • Alkaline phosphatase • Gamma-glutamyltransferase (GGT) • 5-Nucleotidase • ALT & AST normal or slightly elevated • Bilirubin - Normal early on but elevated as disease progresses • +AMA - 95% of patients • +ANA - up to 70% • IgM • Hypercholesterolemia (85%) • Ceruloplasmin • Antithyroid antibodies PBC Diagnosis: • No extrahepatic biliary obstruction • ALP > 1.5 x ULN • AMA titer 1:40 or higher • Liver biopsy - Histo shows nonsuppurative destructive cholangitis and destruction of interlobular bile ducts Differential Diagnosis: • Viral hepatitis, TPN, Intrahepatic cholestasis of pregnancy, Cardiac diseases, Endocrinopathy, Lymphoma and solid organ malignancies, Hepatic amyloidosis, Bacterial, fungal, viral infections, Sarcoidosis, Drug-induced cholestasis, Primary sclerosis group cholangitis, IgG4 related disease, Bile duct obstruction - gallstone or malignancy #Primary #Biliary #Cirrhosis #PBC #cholangitis #diagnosis #workup #hepatology
