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DESCRIPTIONPrimary biliary cirrhosis (PBC) is a chronic autoimmune disease which affects primarily the bile ducts of the liver. PBC is characterized by inflammatory destruction of the intrahepatic biliary system. The origin of the disease still remains an enigma.
The first hint of autoimmune disorder in PBC came with the recognition that the blood of patients with the disease gave a high-titer reaction in the autoantibody test. Soon it was recognized that antimitochondrial reactivity was highly specific for the disease. Subsequently, evidence accumulated for many autoimmune disorders in PBC and for the likelihood that the disease had a multisystem expression but with the intrahepatic biliary system as the main target. The autoimmune attack causes inflammation and, eventually, damage to the bile ducts of the liver. Chronic inflammation causes scar tissue to form in the bile ducts and results in interference with the necessary excretion of bile.
PBC also may be described as chronic nonsuppurative destructive cholangitis or as primary autoimmune cholangitis. Cirrhosis is actually a late manifestation of the disease. PBC ranges from very mild, when it may not even be detected, to a life-threatening illness. Early diagnosis and treatment is important to the outcome of the illness.
LINKS
Primary Biliary Cirrhosis (PBC)
What Is Primary Biliary Cirrhosis (PBC)
Primary Biliary Cirrhosis Organization AKA PBCers
GI Information: Primary Biliary Cirrhosis
Liver Letter: Primary Biliary Cirrhosis
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