|
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.
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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]
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