Autoimmune

Hepatitis

Ahmed Abdulghany

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 : 1Often diagnosed in patients in their 40s – 50sIncidence 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 Complications• Are those seen with any

progressive liver disease.• Hemolytic anemia• Immune thrombocytopenia• 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 systemItem 1 point 2 points

Autoantibodies ANA or ASMA 1/40

ANA or ASMA ≥ 1/ 80ALKM ≥ 1/40+ve SLA

IgG level ≥ upper limit of normal

≥ 1.1 times upper limit of normal

Liver biopsy Compatible with AIH

Typical features ◊

Viral hepatitis Absent

EmperipolesisHepatic rosette

formation

interface hepatitis

lymphocytic lymphoplasm

acytic infltration

6 points

• Probable AIH

7 points

• Definite AIH

Differential diagnosis

American association for Study of Liver disease (AASLD) treatment Guidelines

Treat with immunosuppressive if:

• AST & ALT ≥ 10 folds upper limit of normal OR

• AST & ALT ≥ 5 folds plus IgG ≥ 2 folds AND/OR

• Biopsy showing bridging necrosis or multilobular necrosis

DONOT treat if:• Minimal or no disease

activity or inactive cirrhosis just follow up every 3 – 6 months

• Serious comorbid pre-existing conditions.

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.

Thank you