| Autoimmune
Hepatitis: Revised Scoring System (1999) (International Autoimmune Hepatitis Group, J. Hepatology 31: 929-938, 1999) |
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|---|---|---|---|---|---|---|---|---|---|
| Feature | -5 | -4 | -3 | -2 | -1 | 0 | +1 | +2 | +3 |
| Sex | Male | Female | |||||||
| Alk phos:ALT or Alk phos:AST (note 1) | >3 | 1.5-3.0 | <1.5 | ||||||
| Serum globulins or IgG above normal | <1x normal | 1-1.5x normal | 1.5-2x normal | >2x normal | |||||
| ANA, SMA, or LKM1 (note 2) | <1:40 | 1:40 | 1:80 | >1:80 | |||||
| AMA | Positive | Negative | |||||||
| Hepatitis viral markers (note 3) | Positive | Negative | |||||||
| Drug history (note 4) | Yes | No | |||||||
| Average alcohol intake | > 60 gm/day | <25 gm/day | |||||||
| Histology | Absence of all of the following: interface hepatitis, lympho- plasmacytic infiltrate, and liver cell rosettes | Biliary changes (note 5) or other defined changes (note 6) (-3 each) | Predominantly lympho- plasmacytic infiltrate, liver cell rosettes (1 each) | Interface hepatitis | |||||
| Other autoimmune disease (note 7) | Absent | Present | |||||||
| Seropositivity for other defined autoantibodies (note 8) | Present | ||||||||
| HLA DR3 or DR4 (note 9) | Absent | Present | |||||||
| Response to therapy (note 10) | Complete | Relapse | |||||||
| Interpretation of scores: An aggregate score greater than 15 prior to therapy constitutes a definite diagnosis of AIH. A score of 10-15 is interpreted as probable AIH. A score greater than 17 following therapy is considered positive, and a score of 12-17 after therapy is considered probable, for the diagnosis of AIH. | |||||||||
Note 1 | The ratio refers to the degree of elevation above upper normal limits (UNL) of these enzymes, i.e., (IU/L alk phos/UNL alk phos)/(IU/L ALT/UNL ALT) | return |
Note 2 | As determined by indirect immunofluorescence on rodent tissues or, for ANA, on HEp-2 cells. Lower titers, esp. of LKM-1, are significant in children and should be scored at least +1 | return |
Note 3 | SCore for markers of hepatitis A, B, and C viruses (i.e., positive or negative for IgM anti-HAV, HBsAg, IgM anti-HBc, anti-HCV and HCV-RNA). If a viral etiology is suspected despite seronegativity for these markers, tests for other potentially hepatotropic viruses such as CMV and EBV may be relevant. | return |
Note 4 | History of recent or current use of known or suspected hepatotoxic drugs. | return |
Note 5 | "Biliary changes" refers to bile duct changes typical of PBC or PSC, ie granulomatous cholangitis or severe concentric periductal fibrosis, with ductopenia, established in an adequate biopsy specimen, and/or a substantial periportal ductular reaction, so-called marginal bile duct proliferation with a cholangiolitis, with copper/copper-associated protein accumulation. | return |
Note 6 | Any other prominent feature or combination of features suggestive of a different etiology | return |
Note 7 | Score for history of any other autoimmune disorder(s) in patient or first-degree relatives. | return |
Note 8 | The additional points should be allocated only in patients seronegative for ANA, SMA, and LKM-1. Other "defined" autoantibodies include pANCA, anti-LC1, anti-SLA, anti-ASGPR, anti-LP, and anti-sulfatide. | return |
Note 9 | The additional points should be allocated only in patients seronegative for ANA, SMA, and LKM-1. HLA DR3 and DR4 are mainly of relevance to North European, Caucasoid, and Japanese populations. One point may be allocated for other Class II antigens for which there is published evidence of their association with AIH in other populations. | return |
Note 10 | Assessment of response to therapy is shown in the Table and may be made at any time. Points should be added to those accrued for features at initial presentation. | return |
| Definitions of Response to Therapy (AIH Scoring System 1999) (International Autoimmune Hepatitis Group, J. Hepatology 31: 929-938, 1999) |
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|---|---|---|---|
| Response | Definition | ||
| Complete | Either or both of the following: marked improvement of symptoms and return of serum ALT or AST, bilirubin and immunoglobulin values completely to normal within 1 year and sustained for at least a further 6 months on maintenance therapy, or a liver biopsy specimen at some time during this period showing at most minimal activity. | or | Either or both of the following: marked improvement of symptoms together with at least 50% improvement of all liver test results during the first month of treatment with AST or ALT levels continuing to fall to less than twice the upper normal limit within 6 months during any reductions toward maintenance therapy, or a liver biopsy within 1 year showing only minimal activity. |
| Relapse | Either or both of the following: an increase in serum AST or ALT levels of greater than twice the upper normal limit or a liver biopsy showing active disease, with or without reappearance of symptoms, after a "complete" response as defined above. | or | Reappearance of symptoms of sufficient severity to require increased (or reintroduction of) immunosuppression, accompanied by any increase in serum AST or ALT levels, after a "complete" response as defined above. |
Reference
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