Primary Biliary Cirrhosis (PBC) - Summary
Epidemiology:
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Description

Primary Biliary Cirrhosis (PBC) - Summary
Epidemiology:
 - Female:Male 9: 1
 - Common European descent
 - Age : 30-65 years
Pathophysiology:
 - T lymphocyte mediated attack on small intralobular bile ducts
 - Loss of intralobular bile ducts causes cholestasis Cirrhosis/Liver failure
Signs and symptoms:
 - 50-60% are asymptomatic
 - Pruritus, Fatigue
Physical Examination: Skin hyperpigmentation (later manifestation), Xanthomas, Hepatosplenomegaly, Spider Nevi, Temporal/Proximal limb muscle wasting
LABORATORY WORKUP:
 - Alkaline phosphatase
 - Gamma-glutamyltransferase (GGT)
 - 5-Nucelotidase
 - AST & ALT - normal or slightly elevated
 - Bilirubin- Normal early on but elevated as disease progresses
 - AMA+ present 95% of patients
 - ANA+ up to 70%
 - Hypercholestrolemia (85%)
 - IgM, Ceruloplasmin, Antithyroid antibodies
Diagnosis:
 - No extrahepatic biliary obstruction
 - ALP > 1.5 X ULN
 - AMA titer 1:40 or higher
 - Liver biopsy- Histo shows non-suppurative destructive cholangitis and destruction of interlobular bile ducts

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Contributed by

Dr. Ravi Singh K
@rav7ks
Academic Hospitalist and APD @SinaiBmoreIMRes, Clinical reasoning,Simulation and POCUS enthusiast - https://twitter.com/rav7ks
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