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