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



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