自己免疫性肝炎（AIH）診療ガイドライン（2016 年）

1 AIH 2016 2017 3 1 IgG 2013 2016 2017 3 COI 2016 2013 2.

2 5 .. 7 .. 9 .. 15 .. 20 .. 21 .. 23 I.. 23 QI -1 AIH .. 23 QI -2 .. 23 QI -3 .. 24 QI -4 .. 24 QI -5 .. 25 QI -6 .. 25 II. AIH .. 26 QII-1 .. 26 QII-2 .. 26 QII-3 .. 27 QII-4 .. 27 QII-5 .. 28 QII-6 .. 28 QII-7 .. 29 QII-8 2016 .. 29 QII-9 .. 29 QII-10 .. 30 QII-11 .. 30 QII-12 ( ) .. 31 III. AIH .. 32 QIII-1 .. 32 QIII-2 .. 32 QIII-3 .. 33 QIII-4 .. 33 QIII-5 .. 34 3 QIII-6 .. 34 QIII-7.

3 35 QIII-8 .. 35 QIII-9 .. 36 QIII-10 .. 36 QIII-11 .. 37 QIII-12 .. 37 QIII-13 .. 38 QIII-14 .. 38 QIII-15 .. 39 QIII-16 ? .. 39 QIII-17 ? .. 40 IV AIH .. 42 QIV-1 .. 42 QIV-2 .. 42 QIV-3 .. 43 QIV-4 .. 43 QIV-5 AIH .. 44 QIV-6 .. 44 QIV-7 AIH .. 44 QIV-8 .. 45 V.. 46 QV-1 .. 46 QV-2 .. 46 QV-3 .. 47 QV-4 AIH .. 47 QV-5 AIH .. 48 VI. AIH .. 49 QVI-1 .. 49 QVI-2 .. 49 QVI-3 .. 50 QVI-4 .. 50 QVI-5 .. 51.

4 52 4 (AIH) AIH (2013 ) 1993/01/01 2015/12/31 PubMed-Medline Cochrane Library AIH CQ [ ] * American Association for the Study of Liver Diseases British Society of Gastroenterology European Association for the Study of Liver AIH 1) Manns MP, Czaja AJ, Gorham JD, et al.

5 Diagnosis and Management of Autoimmune Hepatitis. Hepatology 2010; 51: 2193-213. 2) Gleeson D, Heneghan MA. British Society Gastroenterology (BSG) guidelines for management of autoimmune hepatitis. Gut 2011; 1611-29. 3) European Association for the Study of Liver. EASL Clinical Practice Guidelines: Autoimmune hepatitis. J Hepatol 2015; 971-1004. GRADE Minds ( ) (2014 ) A B C D . ( ) COI 5 1. Autoimmune hepatitis: AIH 1)-6) AIH 1950 Waldenstr m7), 1951 Zimmerman8), Kunkel9) 1956 Mackay LE lupoid hepatitis 10) 1965 Mackay Autoimmune hepatitis 11) AIH 1979 12)-17) 1996 AIH 18) 2009 2013 19) 1993 AIH (IAIHG) 20) 1999 21) 2008 22) AIH 23), 24) 25) AIH 26)

6 IgG4 IgG4 IgG4 AIH 27) 1) . (2013 ). 2) Krawitt EL. Autoimmune hepatitis. N Engl J Med 2006; 354: 54-66. 3) Czaja A, Manns MP. Advances in the diagnosis, pathogenesis, and management of autoimmune hepatitis. Gastroenterology 2010; 139: 58-72. 4) Manns MP, Czaja AJ, Gorham JD, et al. Dignsosis and management of autoimmune hepatitis. Hepatology 2010; 51: 2193-213. 5) Glesson D, Heneghan MA. British Society of Gastroenterology (BSG) guidelines for management of autoimmune hepatitis. Gut 2011; 60: 1611-29. 6) European Association for the Study of the Liver. EASL clinical practice guidelines: Autoimmune hepatitis. J Hepatol 2015; 63: 971-1004. 7) Waldenstr m J. Leber, Blutproteine und Nahrungseiweisse. Dtsch Gesellsch Verd Stoffw 1950; 15: 113-9. 8) Zimmerman HJ, Heller P, Hill RP. Extreme hyperglobulinema in subacute hepatic necrosis.

7 N Engl J Med 1951; 244: 245-9. 9) Kunkel HG, Ahrens EH Jr, Eisenmenger WJ, et al. Extreme hypergammaglobulinemia in young women with liver disease of unknown etiology. J Clin Invest 1951; 30: 654. 10) Cowling DC, Mackay IR, Taft LI. Lupoid hepatitis. Lancet 1956; 271: 1323-6. 11) Mackay IR, Weiden S, Hasker J. Autoimmune hepatitis. Ann N Y Acad Sci 1965; 124: 767-80. 12) Monna T, Kuroki T, Yamamoto S. Autoimmune hepatitis: the present status in Japan. Gastroenterol Jpn 1985; 20: 260-72. 13) Onji M, Nonaka T, Horiike N, et al. Present status of autoimmune hepatitis in Japan. Gastroenterol Jpn 1993; 28 Supple 4: 134-8. 6 14) Toda G, Zeniya M, Watanabe F, et al. Present status of autoimmune hepatitis in Japan-correlating the characteristics with international criteria in an area with high rate of HCV infection. Japan. J Hepatol 1997; 26: 1207-12. 15) . 10 1999; p8-11. 16) Abe M, Mashiba T, Zeniya M, et al.

8 Present status of autoimmune hepatitis in Japan: a nationwide survey. J Gastroenterol 2011; 46: 1136-45. 17) Takahashi A, Arinaga-Hino T, Ohira H, et al. Autoimmune hepatitis in Japan: trends in a nationwide survey. J Gastroenterol 2016 DOI: 18) .. 1996; 37: 298-300. 19) . (2013 ) 2013; 54: 723-5. 20) Johnson PJ, McFarlane IG. Meeting Report: International Autoimmune Hepatitis Group. Hepatology 1993; 18: 988-1005. 21) Alvarez F, Berg PA, Bianchi FB, et al. International Autoimmune Hepatitis Group Report: review of criteria of autoimmune hepatitis. J Hepatol 1999; 31: 929-38. 22) Hennes EM, Zeniya M, Czaja AJ, et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 2008; 48: 169-76. 23) Onji M, Autoimmune Hepatitis Study Group. Proposal of autoimmune hepatitis presenting with acute hepatitis, severe hepatitis and acute liver failure. Hepatol Res 2011; 41: 497. 24) .. 2011; 52: 237. 25) Yamamoto K, Miyake T, Ohira H, et al.

9 Prognosis of autoimmune hepatitis showing acute presentation. Hepatol Res 2013; 43: 630-8. 26) Ohira H, Abe K, Takahashi A, et al. Clinical features of hepatocellular carcinoma in patients with autoimmune hepatitis in Japan. J Gastroenteol 2013; 48: 109-14. 27) Umemura T, Zen Y, Hamano H, et al. IgG4 associated autoimmune hepatitis: a differential diagnosis for classical autoimmune hepatitis. Gut 2007; 56: 1471-2. 7 2. 2016 I Autoimmune hepatitis: AIH 1 2 IgG AST ALT AST ALT International Autoimmune Hepatitis Group IAIHG 1.

10 HLA-DR4 2. 1 HEp-2 II 1. 2. 3. IgG 4. interface hepatitis 5. 1 2 5 3 1 2 5 1 2 1. 2. 8 3. 4. 2 3 5. 6. 7. 10 8. Drug-induced liver injury: DILI DDW-J 2004 9. III AST ALT 200 U l 5mg dl 60 1 2 3 1 , 2 , 3 1.