autoimmune

Arch Gastroenterohepatol 2000; 19 ( No 1 - 2 ):59-64

Criteria for diagnosis of autoimmune hepatitis are revised: Report of the international autoimmune Hepatitis Group
Revizija kriterijuma za dijagnozu autoimunog hepatitisa: Izvestaj medjunarodne grupe za autoimuni hepatitis (accepted November 30th, 1999)

Address correspondence to:
Associate Professor Dr Neda Svirtlih, MD, PhD,
Institute for Infectious Diseases,
Bul JA 16 St,
YU- 11000 Belgrade, Serbia, Yugoslavia
FAX: ( 381 11 ) 684 272
E-mail: [email protected]

remark: on-line version of this article is still incomplete

Abstract

Descriptive criteria and the numerical scoring system for autoimmune hepatitis are revised by the International Autoimmune Hepatitis Group and published as a consensus report. These recommendations are the final result of a retrospective evaluation of already accepted criteria and include only minor modifications.

The main new suggestions for descriptive criteria are as follow: some histological features which are not so significant to exclude autoimmune hepatitis are omitted; specification for titers of autoantibodies in children is not necessary; search for potentially hepatotropic viruses is exceptionally recommended; history of blood transfusion and alcohol abuse are simplified or not longer recommended.
In the scoring system, the principal changes are: reduced alkaline phosphatase: aminotransferases ratio is less important, more negative points bears antimitochrondrial antibodies positivity , use of hepatotoxic drugs, and an absence of interface hepatitis characteristic for autoimmune hepatitis. Finally, the response to immunosuppressive therapy is simplified.

Table 1: Revised descriptive criteria for diagnosis of autoimmune hepatitis

Features Definite Probable

liver histology Interface hepatitis( as defined in text) of moderate or severe activity with or without lobular hepatitis or central-portal bridging necrosis, but without biliary lesions or well defined granulomas or other prominent changes suggestive of different etiology. Same as for "definite"

serum biochemistry Any abnormality in serum aminotransferases, especially (but not exclusively) if the serum alkaline phosphatase is not markedly elevated. Normal serum concetrations of alpha-1 anti-trypsin, copper and ceruloplasmin. Same as for "definite" but patients with abnormal serum concentration of copper or ceruloplasmin may be included, provided that Wilson's disease has been excluded by appropriate investigations

serum immuno-globulin Total serum globulin or gamma-globulin or IgG concentrations greater than 1.5 times the upper normal limits. Any elevation of serum globulin or gamma-globulin or IgG concentrations above the upper normal limits.

serum autoanti-bodies Seropositivity for ANA, SMA or anti-LKM-1 antibodies at titres greater than 1:80. Lower titres (particularly of anti- LKM-1) may be significant in children. Seronegativity for AMA. Same as for "definite" but at titres of 1:40 or greater. Patients who are seronegative for these antibodies but who are seropositive for other anti-bodies specified in the text may be included.

viral markers Seronegativity for markers of current infection with hepatitis A, B and C viruses. Same as for "definite"

other etiological factors Average alcohol consumption less than 25 g/day. No history of recent use of known hepatotoxic drugs. Alcohol consumption less than 50 g/day and no recent use of known hepatotoxic drugs. Patients who have consumed larger amounts of alcohol or who have recently taken potentially hepatotoxic drugs may be included, if there is clear evidence of continuing liver damage after abstinence from alcohol or withdrawal of the drug.

REFERENCES:

1. Johnson PJ, McFarlane IG. Meeting Report: International Autoimmune Hepatitis Group. Hepatology 1993; 18: 998-1005.
2. Ludwig J, McFarlane IG, Rakela J, Demetris AJ, Wanless IR, Panel Chair. Terminology of chronic hepatitis, hepatic allografft rejection and nodular lesions of the liver: summary of recommendations developed by an International working party. Am J Gastroenterol 1994; 89: S177-81.
3. Ludwig J, McFarlane IG, Rakela J, Panel Chair. Terminology of Chronic Hepatitis: International Working Party. Am J Gastroenterol 1995; 90: 181-9.
4. Švirtlih N. Autoimmune hepatitis: Pathogenesis and Diagnostic Criteria. Arch Gastroenterohepatol 1998; 17 (suppl 8): S46-52.
5. Czaja AJ, Carpenter HA. Validation of scoring system for diagnosis of autoimmune hepatitis. Dig Dis Sci 1996; 44: 305-14.
6. Bianchi FB, Cassani F, Lenzi M, Ballardini G, Muratori L, Giostra F, et al. Impact of International Autoimmune Hepatitis Group scoring system in definition of autoimmune hepatitis. An Italian Experience. Dig Dis Scii 1996; 41: 166-71.
7. Toda G, Zeniya M, Watanabe F, Imawari M, Kioysawa K., Nishioka M, et al., and the Japanese National Study Group of Autoimmune Hepatitis. Present status of autoimmune hepatitis in Japan - correlating the characteristics with internattional criteria in area with a high rate of HCV infection. J Hepatol 1997; 26: 1207-12.
8. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis, (special article). J Hepatol 1999; 3: 929-38.
9. Boberg KKKM, Fausa O, Haaland T, Holter E, Mellbye OJ, Sprukland A, et al. Features of autoimmune hepatitis in primary sclerosing cholangitis: an evaluation of 114 primary sclerosing cholangitis patients according to a scoring system for diagnosis of autoimmune hepatitis. Hepatology 1996; 23: 1369-76.
10. Mc Farlane IG. The relationship between autoimmune markers and different clinical syndromes in autoimmune hepatitis. Gut 1998; 42: 599-602.
11. Targan SR, Landers C, Vidrich A, Czaja AJ. High- titer antineutrophilll cytoplasmatic antibodies in type-1 autimmune hepatitis. Gastroenterology 1995; 108: 1159-66.
12. Zauli D, Ghetti S, Grassi A, Descovich C, Cassani F, ballardini G, et al. Anti- neutrophil cytoplasmatic autoantibodies in type 1 and 2 autoimmune hapatitis.. Hepatology 1997; 25: 1105-7.
13. Maddrey WC. How many types of autoimmune hepatitis are there? Gastroenterology 1993; 105: 1571-4.
14.Czaja AJCarpenter HA, Santrach PJ, Moore SB. Immunologic features and HLA associations in chronic viral hepatitis. Gassstroenterology 1995; 108: 157-64.
15. Cassani F, Cataleta M, Valentini P, Muratori P, Giostra F, Francesconi R, et al. Serum autoantibodies in chronic hepatitis C: comparision with autoimmune hepatitis and impact on disease profile. Hepatology 1997; 26: 5661-6.
17. Gregorio GV, Jones H, Choudhuri K, Vegnente A, Bortolotti F, Mieli- Vergani G, et al. Autoantibody prevalence in chronic hepatitis B virus infection: effect of interferon alfa. Hepatology 1996; 24:520-3.
18. Inverizzi P, Crosignani A, Battezzatiii PM, Covini G, De Valle G, Larghi A, et al. Comparision of the clinical features and clinical course of antimitochondrial antibody- positive and -negative primary biliary cirrhosis. Hepatology 1997; 25: 1090-5.
19. Goodman ZD, McNally PR, Davis DR, Ishak KG. Autoimmune cholangitis. A variant of primary biliary cirrhosis: clinicopathological and serological correlations in 200 cases. Dig Dis Sci 1995; 40: 1232-42.
20. Neuberg J, Thomson R. PBC and AMA- what is the connection? Hepatology 1999; 29: 271-6.
2221. Metcalf J, Mitchison HC, Palmer JM. Natural history of early primary biliaris cirrhosis. Lancet 1996; 348: 1399-402.
22. McNair ANB, Moloney M, Portmann BC, Wiliams R, McFarlane IG. Autoimmune hepatitis overlapping with primary sclerosing cholangitis iin five cases. Am J Gastroenerol 1998; 93: 777-84.
23. Gohlke F, Lohse AW, Dienes HP, Lohr H, Marker-Hermann E, Gerken G, et al. Evidence for an overlap syndrome of autoimmune hepatitis and primary sclerosing cholengitis. J Hepatol 1996; 24: 699-705.
24. Roberts S, Poterucha JJ, Gores GJ, Czaja AJ. Overlap between type 1 autoimmune hepatitis and primary sclerosing cholangitis (abstract). Hepatology 1995; 22: 129A.
Zrology 1997; 112(2): 594-642.

Hosted by www.Geocities.ws

Features	Definite	Probable
liver histology	Interface hepatitis( as defined in text) of moderate or severe activity with or without lobular hepatitis or central-portal bridging necrosis, but without biliary lesions or well defined granulomas or other prominent changes suggestive of different etiology.	Same as for "definite"
serum biochemistry	Any abnormality in serum aminotransferases, especially (but not exclusively) if the serum alkaline phosphatase is not markedly elevated. Normal serum concetrations of alpha-1 anti-trypsin, copper and ceruloplasmin.	Same as for "definite" but patients with abnormal serum concentration of copper or ceruloplasmin may be included, provided that Wilson's disease has been excluded by appropriate investigations
serum immuno-globulin	Total serum globulin or gamma-globulin or IgG concentrations greater than 1.5 times the upper normal limits.	Any elevation of serum globulin or gamma-globulin or IgG concentrations above the upper normal limits.
serum autoanti-bodies	Seropositivity for ANA, SMA or anti-LKM-1 antibodies at titres greater than 1:80. Lower titres (particularly of anti- LKM-1) may be significant in children. Seronegativity for AMA.	Same as for "definite" but at titres of 1:40 or greater. Patients who are seronegative for these antibodies but who are seropositive for other anti-bodies specified in the text may be included.
viral markers	Seronegativity for markers of current infection with hepatitis A, B and C viruses.	Same as for "definite"
other etiological factors	Average alcohol consumption less than 25 g/day. No history of recent use of known hepatotoxic drugs.	Alcohol consumption less than 50 g/day and no recent use of known hepatotoxic drugs. Patients who have consumed larger amounts of alcohol or who have recently taken potentially hepatotoxic drugs may be included, if there is clear evidence of continuing liver damage after abstinence from alcohol or withdrawal of the drug.