Yamamoto et al developed a score to aid in the diagnosis of primary biliary cirrhosis. The authors are from Okayama University and Ehime University in Japan.
Parameters:
(1) gender
(2) ratio of serum alkaline phosphatase to ALT
(3) serum IgM level
(4) total cholesterol level
(5) antimitochondrial antibody (AMA or AMA-M2, by immunofluorescence or ELISA)
(6) antinuclear antibody (ANA)
(7) viral marker studies (hepatitis B, hepatitis C, other virus)
(8) recent history of blood transfusion
(9) daily alcohol intake
(10) immune disease in family
Additional Parameters:
(11) histologic features
(12) imaging diagnosis
(13) autoantibodies (anti-centromere antibody, SS-A, SS-B)
(14) HLA DR8
|
Parameter |
Finding |
Points |
|---|---|---|
|
gender |
male |
0 |
|
|
female |
2 |
|
alkaline phosphatase to ALT ratio |
< 0.5 |
-1 |
|
|
0.5 to 1.00 |
0 |
|
|
1.01 to 2.00 |
1 |
|
|
2.01 to 3.00 |
2 |
|
|
> 3.0 |
3 |
|
serum IgM |
less than upper limit of normal |
0 |
|
|
1 to 1.5 times ULN |
1 |
|
|
1.51 to 2.0 times ULN |
2 |
|
|
> 2.0 times ULN |
3 |
|
total cholesterol |
normal |
0 |
|
|
elevated |
1 |
|
AMA |
negative |
-3 |
|
|
positive |
5 |
|
ANA |
<= 1:40 |
0 |
|
|
> 1:40 |
-1 |
|
HBsAg |
negative |
0 |
|
|
present |
-1 |
|
HCV (antibody or RNA) |
negative |
0 |
|
|
present |
-1 |
|
other virus marker |
negative |
0 |
|
|
present |
-1 |
|
all 3 virus markers |
all negative |
3 |
|
|
any positive |
0 |
|
recent history of blood transfusion |
no |
1 |
|
|
yes |
-2 |
|
alcohol intake per day |
female and < 25 g |
2 |
|
|
female and 25 to 40 g |
0 |
|
|
female and 41 to 60 g |
-1 |
|
|
female and > 60 g |
-2 |
|
|
male and < 35 g |
2 |
|
|
male and 35 to 50 g |
0 |
|
|
male and 51 to 80 g |
-1 |
|
|
male and > 80 g |
-2 |
|
PBC in first or second degree relative |
no |
0 |
|
|
yes |
2 |
|
autoimmune disease in patient or first degree relative |
no |
0 |
|
|
yes |
1 |
|
chronic nonsuppurative destructive cholangitis |
absent |
0 |
|
|
present |
10 |
|
granuloma |
absent |
0 |
|
|
present |
5 |
|
number of bile ducts divided by number of hepatic arteries in a portal tract |
>= 0.8 |
0 |
|
|
< 0.8 |
5 |
|
bile duct degeneration |
absent |
0 |
|
|
present |
2 |
|
ductular proliferation and plasma cell infiltration |
absent |
0 |
|
|
present |
1 |
|
focal necrosis |
absent |
0 |
|
|
present |
-2 |
|
any histologic finding suggesting another diagnosis |
absent |
0 |
|
|
present |
-2 |
|
imaging diagnosis |
fatty liver |
-1 |
|
|
gallstone or bile duct stone |
-3 |
|
|
bile duct obstruction |
-10 |
|
|
other |
0 |
|
autoantibody (ACA, SS-A, SS-B) |
negative |
0 |
|
|
positive |
2 |
|
HLA DR8 |
absent |
0 |
|
|
present |
1 |
where:
• Table 1 has several breakpoints where there is overlap in values.
• The autoantibodies are considered PBC-specific forms of ANA (page 55): anti-centromere, anti-Sp100 or anti-gp210.
• The ratio of serum alkaline phosphatase to ALT is ratio of each as a multiple of the upper limit of normal.
total score without additional parameters =
= SUM(points for all of the key parameters)
total score with additional parameters) =
= SUM(points for all of the parameters)
Interpretation:
• The higher the score the more likely the diagnosis of PBC.
|
Pretreatment Total Without Additional Parameters |
Pretreatment Total With Additional Parameters |
Interpretation |
|---|---|---|
|
> 12 |
> 17 |
definite |
|
6 to 12 |
9 to 17 |
probable |
|
< 6 |
< 9 |
nondiagnostic |
Purpose: To evaluate a patient for primary biliary cirrhosis (PBC) using the score of Yamamoto et al.
Specialty: Gastroenterology, Immunology/Rheumatology
Objective: other testing, criteria for diagnosis
ICD-10: K74.3,
