Introduction Immune system attacks cells of the liver leading to hepatic inflammation causes unknown can be triggered by various diseases toxins drugs if left untreated can lead to cirrhosis and liver failure 2 main types type 1 (classic) autoimmune hepatitis most common can occur at any age patients often have other autoimmune disorders type 2 autoimmune hepatitis most common in young females patient will classically be a young female Epidemiology incidence 1-2 per 100,000 demographics women much more affected than men risk factors being female young age (type 2) family history of autoimmune diseases having a separate autoimmune disease Associated conditions other autoimmune disorders such as autoimmune thyroiditis (Hashimoto's) rheumadoid arthritis ulcerative colitis pernicious anemia hemolytic anemia celiac disease HLA DR3, DR4 association Prognosis with current treatment methods, remission in approximately 10-40% of cases only about 50% will stay in remission Presentation Symptoms range from minor to severe can be acute or chronic fatigue abdominal pain joint pain pruritus skin rashes dark-colored urine amenorrhea in women - often an initial complaint Physical exam inspection distended abdomen - ascites jaundice palpation hepatomegaly vascular bleeding from esophagus - esophageal varices spinder angiomas Evaluation Labs serology antibody tests help distinguish autoimmune hepatitis from viral hepatitis anti-smooth muscle antibody anti-nuclear antiboby elevated liver enzymes possible elevation of bilirubin Biopsy liver confirms the diagnosis determines the degree and type of liver damage Diagnosis combination of clinical, laboratory, and histological findings always requires a liver bx Treatment Goal slow or stop the body's immune system from attacking the liver Pharmacologic - Immunosuppressants prednisone indications usually recommended for anyone with autoimmune hepatitis duration at least 18-24 months some patients will be on it for life side effects diabetes osteoporosis osteonecrosis hypertension cataracts glaucoma weight gain azathioprine indications often used in combination with prednisone may allow for smaller doses of prednisone and thus lowering side effects outcomes side effects difficulty fighting infections nausea liver damage (rare) pancreatitis (rare) cancers (rare) other immunosuppressants indications if patient doesn't respond to azathioprine or prednisone drugs mycophenolate cyclosporine tacrolimus Operative liver transplant indications when medications don't halt disease progression or development of cirrhosis outcomes