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

Autoimmune
AMA + ve AIH
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 Complications
• Hemolytic anemia • Are those seen with any
• Immune thrombocytopenia progressive liver disease.
• Type 1 diabetes
• Thyroiditis
• Ulcerative colitis
• Polyglandular autoimmune
syndrome.
 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 2 points
Autoantibodies ANA or ASMA ANA or ASMA ≥ 1/ 80 • Probable AIH
1/40 ALKM ≥ 1/40 6 points
+ve SLA
IgG level ≥ upper limit of ≥ 1.1 times upper limit of
normal normal • Definite AIH
7 points
Liver biopsy Compatible Typical features ◊
with AIH
Viral hepatitis Absent

lymphocytic
Hepatic
lymphoplasm interface
rosette Emperipolesis
acytic hepatitis
formation
infltration
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.