Diagnostic Criteria for Autoimmune Hepatitis (AIH)

Autoimmune hepatitis (AIH) is characterized by inflammatory liver histology, circulating non-organ-specific autoantibodies, and increased levels of immunoglobulin (Ig) G in the absence of a known etiology.

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.

 

 

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

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