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Autoimmune hepatitis
2012 / 02 / 22
GI CR 廖苡君
Introduction
• Autoimmune hepatitis (AIH) is a generally unresolving inflammation of the liver of unknown
cause.
• Onset is frequently insidious with non-specific
symptoms but the clinical spectrum is wide, ranging
from an asymptomatic presentation to an acute
severe disease
• Mean incidence is 1 to 2 per 100,000 persons per year
• Prevalence is 11 to 17 per 100,000 persons per year
DIAGNOSIS AND MANAGEMENT OF AUTOIMMUNE HEPATITIS:
AASLD PRACTICE GUIDELINES 2010
Pathogenesis
• A working model for its pathogenesis
postulates:
a. environmental triggers
b. failure of immune tolerance mechanisms
c. genetic predisposition collaborate to induce a
T-cell–mediated immune attack upon liver
antigens
leading to a progressive necro-inflammatory
and fibrotic process in the liver
DIAGNOSIS AND MANAGEMENT OF AUTOIMMUNE HEPATITIS:
AASLD PRACTICE GUIDELINES 2010
Pathogenesis
Feldman: Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 9th ed
Clinical manifestation
Clinical manifestation
Laboratory abnormalities:
• serum AST or ALT
• Increased serum total IgG or γ-globulin
• serological autoantibody (ANA, SMA, anti-LKM
1, or anti LC 1)
Diagnosis
• The diagnosis requires the presence of
characteristic clinical and laboratory features,
and the exclusion of other conditions.
• The diagnostic criteria for AIH / diagnostic
scoring system were codified by an
international panel in 1999
DIAGNOSIS AND MANAGEMENT OF AUTOIMMUNE HEPATITIS:
AASLD PRACTICE GUIDELINES 2010
Diagnosis
Feldman: Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 8th ed
Scoring system
Alvarez F, Berg PA, Bianchi FB, et al. International Autoimmune Hepatitis Group Report:
review of criteria for diagnosis of autoimmune hepatitis. J Hepatol. Nov 1999;31(5):929-38
Simplified score
HENNES ET AL. HEPATOLOGY, July 2008
Differential diagnosis
A. Other autoimmune liver diseases
• Primary biliary cirrhosis
• Primary sclerosing cholangitis
• Autoimmune cholangiopathy
• Overlapping syndrome
B. Chronic viral hepatitis
• Chronic hepatitis B / C / D / other virus
DIAGNOSIS AND MANAGEMENT OF AUTOIMMUNE HEPATITIS:
AASLD PRACTICE GUIDELINES 2010
Differential diagnosis
C. Other
• Chronic drug-induced hepatitis
• Alpha-1-antitrypsin deficiency
• Wilson's disease
• Cholangiopathy related to AIDS
• Steatohepatitis (Nonalcoholic or Alcoholic)
• Granulomatous hepatitis
• Systemic lupus erythematosus
• Graft-versus-host disease
• Cryptogenic chronic hepatitis or cirrhosis
DIAGNOSIS AND MANAGEMENT OF AUTOIMMUNE HEPATITIS:
AASLD PRACTICE GUIDELINES 2010
Histology
• Autoimmune hepatitis is characterized by a
chronic inflammatory cell infiltrate. Plasma
cells are the prominent cell type.
• Biopsies may show evidence for interface
hepatitis, bridging necrosis, and fibrosis.
• Lobular collapse, best identified by reticulin
staining, is a common finding.
DIAGNOSIS AND MANAGEMENT OF AUTOIMMUNE HEPATITIS:
AASLD PRACTICE GUIDELINES 2010
Classification
Overlap syndrome
Treatment
• Regimen
Combination therapy
Single drug
Prednisolone
(mg/day)
Azathioprine
(mg/day)
Prednisolone
(mg/day)
30 mg x 1 wk
20 mg x 1 wk
15 mg x 2 wk
10 mg until endpoint
50 mg until
endpoint
60 mg x 1 wk
40 mg x 1 wk
30 mg x 2 wk
20 mg until endpoint
Feldman: Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 8th ed
End point
•
1)
2)
3)
4)
Remission
No symptom
Histologic improvement
AST < 2x
Gamma Globulin normal
• Incomplete response
Failure to satisfy remission criteria
• Treatment failure
Worsening of AST / Bilirubin or histology
• Drug toxicity:
Cataract, osteopenia, etc
Feldman: Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 9th ed
Treatment result
• Average treaatment course: 22 months
• Response rate: 65 % in 3 years
• 10-year survival: 93%
• 9% treatment failure may use high dose Tx :
a. Prednisolone alone: 60 mg/day
b. Prednisolone 30 mg/day+ Azathiprine 150 mg/day
Clinical improvement to high dose Tx: 70% in 2 years
Feldman: Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 8th ed
Prognosis
Feldman: Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 8th ed
Liver transplantation
• Effective for decompensated AIH:
5 year survival rate: 83-92%
10 year survival rate: 75%
• Recurrence rate: 17%, de-novo AIH: 3-5%
Feldman: Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 8th ed
Thanks for your attention !!