Table 1

Revised Scoring System for the Diagnosis of Autoimmune Hepatitis6.

Parameter/Features
Score

Gender

- Female/Male
+2/0
Degree of elevation above upper normal limit of ALP vs. aminotransferases

- <1.5
+2
- 1.5 – 3.0
0
- >3.0
-2
Total serum globulins, γ-globulins, or IgG above normal

- >2.0
+3
- 1.5 – 2.0
+2
- 1.0 – 1.5
+1
- <1.0
0
ANA, SMA or LKM-1 (titers by immunofluorescence on rodent tissues or HEp2-cells)

- >1 : 80
+3
- 1 : 80
+2
- 1 : 40
+1
- <1 : 40
0
- AMA positive
-4
Hepatitis viral markers (IgM anti-HAV, HBsAg, IgM anti-HBc, anti-HCV and HCV-RNA)

- Positive/Negative
-3/+3
Recent or current use of known or suspected hepatotoxic drugs

- Yes/No
-4/+1
Average alcohol intake

- <25 g/day
+2
- >60 g/day
-2
Other autoimmune disease(s) in patient or first degree relatives

- Yes/No
+2/0
Optional additional parameters (should be allocated only if ANA, SMA or LKM-1 are negative)

- HLA DR3, DR4, or other HLA with published association with AIH)
+1
- Seropositivity for any of ANCA, anti-LC1, anti-SLA/LP, anti-ASGPR and anti-sulfatide
+2
Liver histology

- Interface hepatitis
+3
- Predominant lymphoplasmacytic infiltrate
+1
- Rosetting of liver cells
+1
- None of the above
-5
- Biliary changes
-3
- Other changes
-3
Response to therapy (as defined in Table 2)

- Complete/Relapse
+2/+3

Definite AIH if greater than 15 before treatment or greater than 17 post-treatment; probable AIH if varies between 10–15 before treatment or 12–17 post-treatment. ALP: alkaline phospatase, IgM anti-HAV: hepatitis A virus IgM antibody, anti-HCV: hepatitis C virus antibody, HBsAg: surface antigen of hepatitis B virus, IgM anti-HBc: IgM antibody against the nuclear antigen of hepatitis B virus. Other abbreviations are the same as shown in the text.

Zachou et al. Journal of Autoimmune Diseases 2004 1:2   doi:10.1186/1740-2557-1-2

Open Data