Relative prevalence |
>80% |
20% in Europe, 4% in United States |
Autoantibodies commonly associated |
Antinuclear, antismooth muscle |
Liver kidney microsome-1 |
Patient demographic |
Approximately 70% women |
Female predominance |
Age of onset |
Peak incidence between 16 and 30 years of age, although 50% are older than 30 years of age |
Average 10 years of age but seen in adults, specifically in Europe |
Other commonly associated autoimmune diseases |
Prevalence of 17% to 48%. Thyroid disease, synovitis, ulcerative colitis |
Prevalence unclear. Diabetes, thyroid disease, vitiligo, pernicious anemia |
Presentation |
Acute onset rare |
Frequently presents with cirrhosis in children and more aggressively |
Response to treatment |
Excellent |
May be more treatment resistant |
Progression of disease |
25% have cirrhosis at diagnosis; 45% develop cirrhosis |
Approximately 80% develop cirrhosis |