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Autoimmune hepatitis
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
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.
Classification
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
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
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.
Extrahepatic manifestations
• Hemolytic anemia
• Immune thrombocytopenia
• Type 1 diabetes
• Thyroiditis
• Ulcerative colitis
• Polyglandular autoimmune
syndrome.

Complications
• Are those seen with any
progressive liver disease.
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
Histology
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
Differential diagnosis
American association for Study of Liver
disease (AASLD) treatment Guidelines
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.
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.
Autoimmune hepatitis
Autoimmune hepatitis

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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
  • 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.