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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