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Diagnostic Criteria for Autoimmune Hepatitis (AIH)

Requisites

Diagnostic Criteria

Definite

Probable

No genetic liver disease

Normal alpha1 antitrypsin phenotype

Normal serum ceruloplasmin, iron, and ferritin levels

Partial alpha1 antitrypsin deficiency

Nonspecific serum copper, ceruloplasmin, iron, and/or ferritin abnormalities

No active viral infection

No markers of current infection with hepatitis A, B, and C viruses

No markers of current infection with hepatitis A, B, and C viruses

No toxic or alcohol injury

Daily alcohol < 25 g/d and no recent use of hepatotoxic drugs

Daily alcohol < 50 g/d and no recent use of hepatotoxic drugs

Laboratory features

Predominant serum aminotransferase abnormality

Globulin, gamma-globulin or immunoglobulin G level >/= 1.5 times normal

Predominant serum aminotransferase abnormality

Hypergammaglobulinemia of any degree

Autoantibodies

ANA, SMA, or anti-LKM1 >/= 1:80 in adults and >/=1:20 in children; no AMA

ANA, SMA, or anti-LKM1 >/= 1:40 in adults or other autoantibodies*

Histologic findings

Interface hepatitis

No biliary lesions, granulomas, or prominent changes suggestive of another disease

Interface hepatitis

No biliary lesions, granulomas, or prominent changes suggestive of another disease

 

Abbreviation: AMA, antimitochondrial antibodies.

*Includes perinuclear anti-neutrophil cytoplasmic antibodies and the not generally available antibodies to soluble liver antigen/liver pancreas, actin, liver cytosol type 1, and asialoglycoprotein receptor.

 

 

Diagnostic Scoring System for Atypical Autoimmune Hepatitis in Adults

 

Category

Factor

Score

Gender

Female

+2

Alk Phos:AST (or ALT) ratio

>3

<1.5

-2

+2

Gamma-globulin or IgG (times above upper limit of normal)

>2.0

1.5-2.0

1.0-1.5

<1.0

+3

+2

+1

0

ANA, SMA, or anti-LKM1 titers

>1:80

1:80

1:40

<1:40

+3

+2

+1

0

AMA

Positive

-4

Viral markers of active infection

Positive

Negative

-3

+3

Hepatotoxic drugs

Yes

No

-4

+1

Alcohol

<25 g/d

>60 g/d

+2

-2

Concurrent immune disease

Any nonhepatic disease of an immune nature

+2

Other autoantibodies*

Anti-SLA/LP, actin, LC1, pANCA

+2

Histologic features

Interface hepatitis

Plasma cells

Rosettes

None of above

Biliary changes

Atypical features

+3

+1

+1

-5

-3

-3

HLA

DR3 or DR4

+1

Treatment response

Remission alone

Remission with relapse

+2

+3

Pretreatment score

Definite diagnosis

Probable diagnosis

Posttreatment score

Definite diagnosis

Probable diagnosis

 

 

>15

10-15

 

>17

12-17

 

Abbreviations: Alk phos, serum alkaline phosphatase level; AST, serum aspartate aminotransferase level; ALT, serum alanine aminotransferase level; IgG, serum immunoglobulin G level; AMA, antimitochondrial antibodies; HLA, human leukocyte antigen.

 

 

Recommendations

1. The diagnosis of Autoimmune Hepatitis (AIH) requires determination of the serum aminotransferase and gamma-globulin levels; detection of ANA and/or SMA, or in their absence, anti-LKM1; and liver tissue examination.

2. The diagnostic criteria for AIH should be applied to all patients.

3. If the diagnosis of AIH is not clear, a scoring method should be used.

 

 

References:

  1. Czaja AJ, Freese DK; American Association for the Study of Liver Disease. Diagnosis and treatment of autoimmune hepatitis. Hepatology 2002 Aug;36(2):479-97 [Medline]

  2. Alvarez F, Berg PA, Bianchi FB, Bianchi L, Burroughs AK, Cancado EL, Chapman RW, Cooksley WG, Czaja AJ, Desmet VJ, Donaldson PT, Eddleston AL, Fainboim L, Heathcote J, Homberg JC, Hoofnagle JH, Kakumu S, Krawitt EL, Mackay IR, MacSween RN, Maddrey WC, Manns MP, McFarlane IG, Meyer zum Buschenfelde KH, Zeniya M, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol  1999 Nov;31(5):929-38 [Medline]

Created: Feb 18, 2006
Last Modified: 03/02/2006