Autoantibodies and autoantigens in autoimmune hepatitis: important tools in clinical practice and to study pathogenesis of the disease

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.