PRIMARY BILIARY CIRRHOSIS

• Also called as primary biliary cholangitis.
• PBC is the inflammatory autoimmune disease characterised by non suppurative, inflammatory destruction of medium size intrahepatic bile ducts.

    

RISK FACTORS-

• Familial incidence
• Elevated cholesterol level with xanthoma and xanthelasma
• Also associated with-

i) Sjogren’s syndrome (70%)

ii) Thyroid disease (20%)

 

CLINICAL FEATURES-

• Females are mostly affected (6:1) in 40- 50 years of age
• It is a cholestatic disorder
• Persistent pruritis, dark urine, pale stools, steatorrhoea, jaundice
• Skin pigmentation, lethargy
• Hepatomegaly
• Malaise
• Most common cause of death liver cell failure > variceal bleeding

    

 

INVESTIGATIONS-

1. Conjugated hyperbilirubinemia
2. Earliest lab finding- increase serum alkaline phosphatise level
3. AMA-  positive (95%)

• M2 form of anti- mitochondrial antibody
• Most commonly AMA is IgG1 and IgG3 classes
• ANA positive
• ANCA positive

    

TREATMENT-

• Liver transplantation

 

COMPLICATIONS-

• Portal hypertension
• Ascites
• Variceal bleeding

 

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