自己免疫性肝炎の診断基準

Scoring system by the international autoimmune hepatitis group, 1993 (Hepatology 1993;18:998-1005).

1993年Johnson PJ, MacFarlane IG, Alvarez FらのInternational autoimmune hepatitis groupが自己免疫性肝炎の診断基準をriviseして発表しました。日本人AIH患者と欧米のAIH患者では前者はHLA DR4、後者ではHLA DR3が大部分を占めており、遺伝的な背景が異なると考えられています。日本人AIHの診断基準はこのInternational autoimmune hepatitis groupの診断基準からスコアを求めて診断の参考にすることになっています。このページでは各項目を選択して最後にスコア計算ボタンをクリックしてください。ウイルスマーカーがすべて陰性であれば選択しなくてもかまいません。また、データが無い場合には選択しないでおけば、その項目は0として扱います。つまりAdditional Parameterがまだ不明の時にはこれらの項目は選択しないで計算してください。なお、JavaScriptが必要です。(森實作）

(1) Minimum required parameters

Gender

Female (+2)Male (0)

Serum biochemistry

Ratio of elevation of serum alkaline phosphatase vs. aminotransferase

>3 (-2)<3 (+2)

Total serum globulin, γ-globulin or IgG

Times upper normal limit

>2.0 (+3) 1.5〜2.0 (+2) 1.0〜1.5 (+1) <1.0 (0)

Autoantibodies (titers by immunofluorescence on rodent tissues)

Adults: ANA, SMA or LKM-1

>1 : 80 (+3) 1 : 80 (+2) 1 : 40 (+1) <1 : 40 (0)

AMA

negative (0) positive (-2)

Viral markers

IgM anti-HAV, HBsAg or IgM anti-HBc

positive (-3) Negative

Anti-HCV positive by ELISA and/or RIBA

positive (-2) Negative

Anti-HCV positive by ELISA and/or RIBA, and HCV RNA positive by PCR

positive (-3) Negative

Positive test indicating active infection with any other virus

positive (-3) Negative

Seronegative for all of the above (+3) (no selection needed, judged by the above)

Other etiological factors

History of recent hepatotoxic drug usage or parenteral exposure to blood products

Yes (-2) No (+1)

Alcohol (average consumption)

Male <35gm/day; Female <25gm/day (+2)
Male 35-50gm/day; Female 25-40gm/day (0)
Male 50-80gm/day; Female 40-60gm/day (-2)
Male >80gm/day; Female >60gm/day (-1)

Genetic factors

Other autoimmune diseases in patient or first-degree relatives

Yes (+1) No (0)

(2) Additional parameters

Histology

Chronic active hepatitis with piecemeal necrosis

With lobular involvement and bridging necrosis (+3)
Without lobular involvement and bridging necrosis (+2)

Rosetting of liver cells

Yes (+1) No (0)

Marked/predominantly plasma cell infiltrate

Yes (+1) No (0)

Biliary changes (-1)

Any other changes (e.e., granulomas, sederosis and copper deposits) suggestive of a different etiology

Yes (-1) No (0)

Autoantibodies

In patients who are seronegative for ANA, SMA, and LKM-1
Any defined "liver autoantibody" (e.e., antibodies to SLA, ASGP-R, LSP, LC1, LP, HHPM and sulfatide)

Positive (+2) Negative (0)

Genetic factors

HLA B8-DR3 haplotype, or DR4 allotype

Yes (+1) No (0)

Response to therapy

Complete response (+2)
Partial response (0)
Treatment failure (0)
No response (in terms of disease activity) (-2)
Relapse during or after treatment withdrawal after complete initial response (+3)

AIH score

Diagnosis: if before treatment, if after treatment.

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