Acute decompensation superimposed on chronic liver injury
Results
5 yr pt and graft survival: 80-90%
Recurrence: 15-40%
Higher rates of acute and chronic rejection
Autoimmune Hepatitis Take Home Points
Chronic hepatocellular disease of unknown etiology
Clinical presentation is variable
Diagnosis based upon LFTs, serology, gamma globulins, and histology
Immunosuppressive therapy is the mainstay of treatment
Tailor therapy based upon treatment endpoints
Autoimmune Hepatitis Question 1
A previously healthy 40 yr. old woman presents with fatigue and dark urine for 2 weeks. There is no history of significant alcohol or drug use. Physical exam is remarkable for jaundice and tender hepatomegaly. Labs are notable for AST 1000 U/L, ALT 1500 U/L, and alkaline phosphatase of 350 U/L. The total bilirubin is 10 mg/dl and the INR is 1.3. ASMA is positive to 1:320 and IgG is twice normal. Liver ultrasound reveals hepatomegaly o/w normal. What findings would be found on liver biopsy?
DDSEP 6, AGA Press, 2011.
Autoimmune Hepatitis Question 1
A. Perivenular neutrophil inflammation with ballooned hepatocytes and Mallory bodies
B. Infiltration of portal tracts with lymphocytes and plasma cells, interface hepatitis, piecemeal necrosis along limiting plate
C. Infiltration of portal tracts with destruction of interlobular bile ducts
D. Periportal and lobular non-caseating granulomas
E. Ground glass hepatocytes and Councilman bodies
DDSEP 6, AGA Press, 2011.
Autoimmune Hepatitis Question 2
Which one of the following statements about prognostic factors and autoimmune hepatitis is true?
A. Mild periportal hepatitis is associated with 90% mortality at 10 years without treatment.
B. 60% of patients die within 6 months of diagnosis.
C. AST ≥ 10 times ULN or ≥ 5 times ULN + gamma globulins ≥ 2 times ULN are associated with 90% mortality at 10 yrs without treatment
D. Bridging necrosis is associated with a favorable prognosis
E. Young pts are less likely to go to transplant when compared to older patients
Autoimmune Hepatitis Question 3
A 12 yr. old female presents with malaise, fatigue, and myalgias. She mentions that her stools are lighter color than normal. Physical examination is remarkable for jaundice and a liver edge 2 finger breaths below the right costal margin. Her laboratory evaluation reveals a total bilirubin of 13.1 mg/dl, AST of 2300 U/L, an ALT of 3124 U/L, and an INR of 1.4. Type 2 autoimmune hepatitis is suspected. Which laboratory test is appropriate?
Autoimmune Hepatitis Question 3
A. Antinuclear (ANA) and antismooth muscle antibodies (ASMA)
B. Antimitochondrial antibodies (AMA) and total lipid profile
C. Antibodies to soluble liver antigen (SLA)
D. Serum IgM
E. Anti liver-kidney-microsomal (LKM-1) antibodies
Autoimmune Hepatitis Question 4
Which one of the following is an absolute indication for treatment with steroids and azathioprine?
A. Cirrhosis with minimal activity
B. Bridging and multilobular necrosis
C. AST < 3 times ULN
D. Periportal hepatitis
E. Mild symptoms
Autoimmune Hepatitis Question 5
A 28 yr. old female presents for evaluation of abnormal liver-associated enzymes. Overall, she feels well and the physical exam is unremarkable. Labs reveal AST of 2124 U/L, ALT of 2256 U/L, ANA and ASMA are positive. Liver biopsy shows severe panlobular necrosis. Which one of the following is the appropriate next step?
Autoimmune Hepatitis Question 5
A. Begin azathioprine as monotherapy of 50 mg daily until remission achieved.
B. Begin cyclosporine 100 mg twice daily in combination with mycophenolate 500 mg twice daily.
C. Refer patient for liver transplant evaluation
D. Begin prednisone 30 mg daily in combination with azathioprine 50 mg daily
E. Repeat liver associated enzymes in 3-4 weeks prior to making treatment decisions
Autoimmune Hepatitis Question 6
Which treatment is most appropriate for patients that have worsening liver enzymes despite standard treatment with steroids and azathioprine?
A. Increase prednisone to 60 mg daily or to 30 mg daily in combination with azathioprine 150 mg daily for at least 1 month.
B. Refer immediately for liver transplant evaluation
C. Add tacrolimus 2 mg twice daily to prednisone 10 mg daily and azathioprine 50 mg daily.
D. Stop prednisone and start azathioprine 50 mg daily, mycophenolate 500 mg daily, and tacrolimus 1 mg twice daily
E. Continue steroids and azathioprine at same dose and repeat liver enzymes in 6 weeks.
