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Primary Biliary Cholangitis (Primary Biliary Cirrhosis)

#1 Foam-finger Bile-duck and Cola-angel
Primary Biliary Cirrhosis (PBC) is an inflammatory autoimmune reaction leading to destruction of intrahepatic bile ducts and liver cirrhosis. This disease typically happens to middle-aged women who present with symptoms such as fatigue, pruritus and skin hyperpigmentation.
Autoimmune Destruction of Bile Ducts
Auto-in-moon Destroys Liver Bile-duck

The pathophysiology of primary biliary cholangitis involves autoimmune destruction, with the specific mechanism still unknown. It is proposed that autoreactive T cells and anti-mitochondrial antibodies target bile ducts and bile duct epithelium, leading to destruction.


It is important to note that the autoimmune destruction in primary biliary cholangitis occurs via intrahepatic damage, with a proposed mechanism that autoreactive T cells and anti-mitochondrial antibodies target bile ducts and bile duct epithelium, leading to destruction.


With destruction of intralobular bile ducts, bile cannot be properly transported, resulting in cholestasis.

Occurs in Middle-Aged Females
Middle-aged Woman in the Middle

Primary biliary cholangitis occurs mostly in middle-aged women and peak incidence is between the ages of 40-50.


This disease is insidious in onset, but often the initial presenting symptom is fatigue, occurring in approximately 65% of patients with this disease.


Due to cholestasis and bile absorption, around 55% of patients become pruritic, which is often another initial presenting symptom.

Skin Hyperpigmentation
Hiker-pig with Hyperpigmentation

In addition to jaundice from bile absorption, hyperpigmentation occurs in 25-40% of patients due to increased melanin deposition. Patients can also complain of dark urine and light stools.


Primary biliary cholangitis can lead to significant liver enlargement in approximately 25% of patients, though likelihood increases and enlargement worsens as the disease advances. Hepatomegaly can also be found in previously asymptomatic patients.

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