|
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.
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