Primary biliary cholangitis, previously called primary biliary cirrhosis, is a chronic disease in which the bile ducts in your liver are slowly destroyed. Bile is a fluid made in your liver. It aids with digestion and helps your body get rid of cholesterol, toxins and worn-out red blood cells.

Essentials of Diagnosis:-
• Insidious onset.
• Pruritus followed by jaundice.
• Hepatosplenomegaly.
• Xanthomatous lesions around eyelids.

• Serological tests reflect cholestasis with elevated alkaline phosphatase, 5 nucleotidase, cholesterol, bilirubin.
• Serum is positive for antimitochondrial antibodies.
• Mainly in ladies of age group 40 to 60 years.

Investigation:-

-USG Abdomen & pelvis

-LFT

-CT ABDOMEN (if required)

Treatment:-

-Cholestyramine to relieve pruritus.
-Vitamin A, K and D for steatorrhoea (Parenteral administration).
-Corticosteroids and Azathioprine in selected cases.
-Portal hypertension (enlarged spleen, ascites, oesophageal varices) to be treated as discussed under nodular cirrhosis.
-Liver transplantation.

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