Friday, December 9, 2016

Regarding Primary Biliary Cirrhosis



Primary biliary cirrhosis is most characteristically associated with:
A. Anti-nuclear antibodies
B. Anti-ribonuclear protein antibodies
C. Anti-mitochondrial antibodies
D. Rheumatoid factor
E. Anti-neutrophil cytoplasmic antibodies

Answer:
C. Anti-mitochondrial antibodies

Discussion:

Primary biliary cirrhosis - the M rule

  • IgM
  • anti-Mitochondrial antibodies, M2 subtype 
  • Middle aged females
Primary biliary cirrhosis is a chronic liver disorder typically seen in middle-aged females (female:male ratio of 9:1). 

The etiology is not fully understood although it is thought to be an autoimmune condition. Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis. 

Clinical Presentation: The classic presentation is itching in a middle-aged woman 

Associations :
 Sjogren's syndrome (seen in up to 80% of patients) 
 rheumatoid arthritis  systemic sclerosis 
 thyroid disease 

Diagnosis :
 anti-mitochondrial antibodies (AMA) M2 subtype are present in 98% of patients and are highly specific 
 smooth muscle antibodies in 30% of patients 
 raised serum IgM 

Management :
1. For pruritus: cholestyramine
2.Ffat-soluble vitamin supplementation 
3. Ursodeoxycholic acid 
4. Liver transplantation  if bilirubin > 100 (PBC is a major indication) - recurrence in graft can occur but is not usually a problem

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