Autoimmune hepatitis
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Autoimmune hepatitis is a chronic inflammatory disease of the liver that results from an autoimmune attack against the liver cells. It was first described in the 1950s under various names before being termed autoimmune hepatitis in 1992. It predominantly affects females and is often diagnosed between ages 40-50. Treatment involves immunosuppressive medications like prednisone and azathioprine to induce remission of symptoms and liver inflammation. Relapses may occur upon withdrawing treatment, requiring retreatment with the same medications.
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Autoimmune hepatitis
- 2. Since it was first described in the 1950s, this disorder has been known by a variety of terms, including, chronic active hepatitis, chronic aggressive hepatitis, lupoid hepatitis, plasma cell hepatitis, and, most commonly, autoimmune chronic active hepatitis. In 1992, the International Autoimmune Hepatitis Group recommended autoimmune hepatitis as the most appropriate term for this disease
- 3. EPIDEMIOLOGY & INCIDENCE Female : male ratio of 3.6 : 1 Often diagnosed in patients in their 40s – 50s Incidence is 0.9 – 2 per 100.000 population per year.
- 5. Autoimmune hepatitis – Primary Biliary Cirhossis overlaps • 1-14 % of PBC patients had features of AIH • 2 categories AIH – PBC overlaps AMA + ve AIH Autoimmune Cholangiopathy
- 6. Autoimmune hepatitis – primary sclerosing cholangitis overlaps • Suspect when a patient with serological evidence of AIH plus: Pruritis Cholestatic jaundice Chronic ulcerative colitis Abnormal cholangiogram Liver biopsy showing bile duct abnormalities No response to steroids alone
- 7. Clinical picture • AIH has a heterogeneous and fluctuating nature leading to marked variability in its clinical manifestations. • Some patients may present with mild to severe nonspecific symptoms such as fatigue, lethargy and small joint arthralgia.
- 8. Extrahepatic manifestations • Hemolytic anemia • Immune thrombocytopenia • Type 1 diabetes • Thyroiditis • Ulcerative colitis • Polyglandular autoimmune syndrome. Complications • Are those seen with any progressive liver disease.
- 9. Lab features • As a general rule, aminotranferase elevations are more striking than those of biliruben or alkaline phosphatase. • Hypergamma globulinemia is generally associated with circulating autoantibodies which is useful in the diagnosis
- 10. Histology
- 11. Scoring system Item 1 point Autoantibodies ANA or ASMA 1/40 IgG level Liver biopsy ANA or ASMA ≥ 1/ 80 ALKM ≥ 1/40 +ve SLA 6 points ≥ upper limit of ≥ 1.1 times upper limit of normal normal Compatible with AIH Viral hepatitis lymphocytic lymphoplasm acytic infltration 2 points Typical features ◊ 7 points • Probable AIH • Definite AIH Absent interface hepatitis Hepatic rosette formation Emperipolesis
- 13. American association for Study of Liver disease (AASLD) treatment Guidelines
- 14. AASLD initial therapy • Prednisone 60 mg daily or 30 mg daily plus Azathioprine 50 mg daily • Patients should have a minimum duration of biochemical remission of 2 years. • Until normal enzymes, IgG and biopsy. • Liver transplantation should be considered in patients with Acute liver failure.
- 15. AASLD recommendations regarding relapses, treatment failure & cirrhosis • 1st relapse after drug withdrawal should be retreated with prednisone and AZA at the same treatment regimen used for initial therapy. • Gradual withdrawal should be attempted after at least 2 years. • Consider using Mycophenolate mofetil or Cyclosporin.