Primary Biliary Cholangitis : Pathophysiology, Clinical Features

Primary biliary cholangitis is a condition that causes inflammation and destruction of the bile ducts running through the liver. It is an autoimmune disorder in which the anti-mitochondrial antibodies develop and attack the bile ducts. The bile backs up in the liver causing liver damage and that can lead to cirrhosis of the liver.

Pathophysiology of Primary Biliary Cholangitis

• It is an autoimmune disorder in which anti-mitochondrial antibodies act against the cholangiocytes causing non-suppurative damage.
• The first manifestation is Cholestasis.
• Initially, there is eosinophilic infiltration and neutrophilic infiltration but later on granuloma formation occurs.
• Ultimately if not treated, the condition can progress to cirrhosis and features of portal hypertension will occur.
• Cirrhosis is of micronodular type mainly.
• There are different stages of primary biliary cholangitis:
  • Stage 1- Portal stage of Ludwig
  • Stage 2- Periportal damage or inflammation
  • Stage 3- Septal damage
  • Stage 4- Cirrhosis

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Clinical Features of Primary Biliary Cholangitis

• It mainly occurs in 40 to 60 years of age and is common in females.
• It is insidious in onset and the earliest manifestation is fatigue.
• The Patient will present with pruritis. It will be followed by lichenification of the skin. Due to liver damage, there is the production of endogenous opioid peptides that are responsible for pruritis in these patients.
• Jaundice and hepatomegaly
• Palmer erythema
• Spider Naevi
• Temporal and proximal muscle wasting
• Ascitis and Caput Medusae can develop.
• Xanthoma over the eyelids can also be seen. The LDL is elevated in these patients and this is the reason for xanthoma.
• Osteopenia can develop due to vitamin D3 deficiency.
• Steatorrhea and deficiency of fat-soluble vitamins.
• SICCA Syndrome manifestations like dry eyes and dry mouth will be present.

Diagnosis and Workup of Primary Biliary Cholangitis

• Liver function tests should be done to monitor bilirubin, SGOT, SGPT, and serum alkaline phosphatase. The serum alkaline phosphatase will be elevated four times its normal value.
• Anti-mitochondrial antibody will be positive.
• AMA positive in 50% of the patients.
• Prothrombin time will be elevated
• INR will be altered
• Platelet count is reduced
• Hypersplenism due to portal hypertension.
• Lipid profile will be abnormal
• Ultrasonography to rule out the physical obstruction of the tract.
• Liver Biopsy is the investigation of choice.

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Management of Primary Biliary Cholangitis

• Ursodeoxycholic acid slows down the progression of the disease.
• Cholestyramine acts as a sequestrant for bile acids.
• Antihistamine can be given for pruritis.
• Dronabinol can be given to neutralize the endogenous opioid peptide.
• Naltrexone
• Fat-soluble vitamins should be added to the diet.
• Plasmapheresis is done in case the patient deteriorates despite medical treatment.
• Treatment of choice is liver transplant.

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Differential Diagnosis

• Autoimmune hepatitis
• Primary sclerosing cholangitis
• Sarcoidosis

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