Diagnostic Criteria for Autoimmune Hepatitis (AIH)

Requisites	Diagnostic Criteria
Requisites	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:

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]
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]