Influence of autoantibodies on the pathology of primary biliary cirrhosis; with special reference to autoimmune cholangiopathy

We conducted a study to determine whether the characteristics of the pathology of primary biliary cirrhosis (PBC) varied according to the autoantibodies, and to investigate whether autoimmune cholangiopathy (AIC) could be clearly diffrentiated from PBC as an independent disease. The subjects of this...

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Published inKanzo Vol. 36; no. 7; pp. 408 - 414
Main Authors MIYACHI, Kiyomitsu, ISHIGURO, Noriko, HAYASHI, Naoaki, HASHIMOTO, Etsuko, AOKA, Keiko
Format Journal Article
LanguageJapanese
Published The Japan Society of Hepatology 1995
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ISSN0451-4203
1881-3593
DOI10.2957/kanzo.36.408

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Abstract We conducted a study to determine whether the characteristics of the pathology of primary biliary cirrhosis (PBC) varied according to the autoantibodies, and to investigate whether autoimmune cholangiopathy (AIC) could be clearly diffrentiated from PBC as an independent disease. The subjects of this study were 72 patients with clinically and pathologically diagnosed as PBC whom we classified into 4 groups based on the presence or absence of anti-mitochondrial antibody (AMA), antinuclear antibody (ANA), and M2 antibody. Group I (n=15) was positive for either AMA or M2 as well as ANA, group II (n=44) was positive for either AMA or M2 and negative for ANA, group III (n=9) was negative for both AMA and M2 and positive for ANA, and group IV (n=4) was negative for all 3 autoantibodies. The only significant difference among the 4 groups was a low IgM level in group III, which corresponded to AIC. No differences in the ANA titer or pattern were found between the AMA-positive group I and the AMA-negative group III. Steroid therapy was given to 3 patients in group III, but a marked response was only observed in 1 patient. Thus, no characteristic differences of pathology were found when PBC was classified on the basis of autoantibodies.
AbstractList We conducted a study to determine whether the characteristics of the pathology of primary biliary cirrhosis (PBC) varied according to the autoantibodies, and to investigate whether autoimmune cholangiopathy (AIC) could be clearly diffrentiated from PBC as an independent disease. The subjects of this study were 72 patients with clinically and pathologically diagnosed as PBC whom we classified into 4 groups based on the presence or absence of anti-mitochondrial antibody (AMA), antinuclear antibody (ANA), and M2 antibody. Group I (n=15) was positive for either AMA or M2 as well as ANA, group II (n=44) was positive for either AMA or M2 and negative for ANA, group III (n=9) was negative for both AMA and M2 and positive for ANA, and group IV (n=4) was negative for all 3 autoantibodies. The only significant difference among the 4 groups was a low IgM level in group III, which corresponded to AIC. No differences in the ANA titer or pattern were found between the AMA-positive group I and the AMA-negative group III. Steroid therapy was given to 3 patients in group III, but a marked response was only observed in 1 patient. Thus, no characteristic differences of pathology were found when PBC was classified on the basis of autoantibodies.
Author AOKA, Keiko
HAYASHI, Naoaki
HASHIMOTO, Etsuko
ISHIGURO, Noriko
MIYACHI, Kiyomitsu
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  fullname: ISHIGURO, Noriko
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  fullname: HAYASHI, Naoaki
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  fullname: HASHIMOTO, Etsuko
  organization: Department of Gastroenterology and Internal Medicine, Tokyo Women's Medical College
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  fullname: AOKA, Keiko
  organization: Department of Gastroenterology and Internal Medicine, Tokyo Women's Medical College
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References 2) Berg PA, Wiedmann KH, Kloppel G, et al: Serological classification of chronic cholestatic liver disease by the use of two different types of antimitochondrial antibodies. Lancet II: 1329-1332, 1980
13) Nakanuma Y: Necroinflammatory changes in hepatic lobules in primary biliary cirrhosis with less well-define cholestatic changes. Hum Pathol 24: 378-383, 1993
11) Lassoued K, Brenard R, Benhamou JP, et al: Antinuclear antibodies directed to a 200-kilodalton polypeptide of the nuclear envelope in primary biliary cirrhosis. Gastroenterology 99: 181-186, 1990
20) Flora K, Benner K, Fennimore S, et al: Primary autoimmune cholangitis as an early variant of primary biliary cirrhosis. Hepatology 20: 152A, 1994
16) 三浦徹,前田隆,大西三朗,他:HLAが同一の姉妹に発症し,抗ミトコンドリア抗体陰性,抗核抗体強陽性,PBCの肝組織像を呈した,いわゆる“免疫性胆管炎”に相当する2症例.肝臓 34 (12): 995-1001, 1993
7) Michieletti P, Wanless IR, Heathcote EJ, et al: Antimitochondorial antibody negative primary biliary cirrhosis; a distinct syndorome of autoimmune cholangitis. Gut 35: 260-265, 1994
12) Kitami N, Ishii H, Sato N, et al: Immunoreactivity to M2 proteins in antimitochondorial antibody negative patients with primary biliary cirrhosis. J Gastroenterol Hepatol 9: 7-12, 1994
1) Popper H, Schaffner F: Pathophysiology of cholestasis. Hum Pathol 1: 1-23, 1970
8) Goodman ZD, McNally PR, Ishack KG, et al: "Autoimmune cholangitis"; a variant of primary biliary cirrhosis [Abstract]. Hepatology 18: 109, 1993
5) Dickson ER, Desmet VJ: Chronic cholestasis. Liver Biopsy (interpretation for the 1990s): 8-38, 1991
14) Powell F, Schroeter AL, Dickson ED: Antinuclear antibodies in primary biliary cirrhosis. Lancet I: 288-289, 1984
9) Ludwig J, Rosen CB, Watson KR, et al: Chronic cholestasis in a young man. Hepatology 20: 1351-1355, 1994
17) 稲垣恭孝,宮地清光,桐生恭好,他:慢性関節リウマチを合併した自己免疫性胆管炎の1例.日消誌91 (5): 1057-1061, 1994
15) Taylor SL, Dean PJ, Riely CA: Primary autoimmune cholangitis; an alternative to antimitochondrial antibody-negative primary bliary cirrhosis. Am J Surg Pathol 18: 91-99, 1994
10) Joplin RE, Johnson GD, Neuberger JM, et al: Destribution of pyruvate dehydrogenase dihydrolipoamid acetyltransferase (PDC-E2) and biliary epithelium from patients with primary biliary cirrhosis. Hepatology 19: 1375-1380, 1994
18) 黒田聖仁,高木徹,粕川禮司,他:抗核抗体陽性原発性胆汁性肝硬変症の検討[Abstract].日消誌91(臨時増刊号): 1872, 1994
3) Brünner G, Klinge O: Ein der chronischdestruierenden nicht-eitrigen Cholangitis ahnliches Krankheitsbild mit antinuklearen Antikorpern (Immunocholangitis). Dtsch Med Wschr 112: 1454-1458, 1987
4) Carrougher JG, Shaffer RT, Goodman ZD, et al: A 33-year-old woman with an autoimmune syndrome. Semin Liver Dis 11: 256-262, 1991
6) Ben-Ari Z, Dhillon AP, Sherlock S: Autoimmune cholangiopathy; part of the spectrum of autoimmune chronic active hepatitis. Hepatology 18 (1): 10-15, 1993
19) Mitchison HC, Palmer JM, James OFW, et al: A controlled trial of prednisolone treatment in primary biliary cirrhosis; three-year results. J Hepatol 15: 336-344, 1992
References_xml – reference: 6) Ben-Ari Z, Dhillon AP, Sherlock S: Autoimmune cholangiopathy; part of the spectrum of autoimmune chronic active hepatitis. Hepatology 18 (1): 10-15, 1993
– reference: 15) Taylor SL, Dean PJ, Riely CA: Primary autoimmune cholangitis; an alternative to antimitochondrial antibody-negative primary bliary cirrhosis. Am J Surg Pathol 18: 91-99, 1994
– reference: 20) Flora K, Benner K, Fennimore S, et al: Primary autoimmune cholangitis as an early variant of primary biliary cirrhosis. Hepatology 20: 152A, 1994
– reference: 5) Dickson ER, Desmet VJ: Chronic cholestasis. Liver Biopsy (interpretation for the 1990s): 8-38, 1991
– reference: 12) Kitami N, Ishii H, Sato N, et al: Immunoreactivity to M2 proteins in antimitochondorial antibody negative patients with primary biliary cirrhosis. J Gastroenterol Hepatol 9: 7-12, 1994
– reference: 18) 黒田聖仁,高木徹,粕川禮司,他:抗核抗体陽性原発性胆汁性肝硬変症の検討[Abstract].日消誌91(臨時増刊号): 1872, 1994
– reference: 1) Popper H, Schaffner F: Pathophysiology of cholestasis. Hum Pathol 1: 1-23, 1970
– reference: 16) 三浦徹,前田隆,大西三朗,他:HLAが同一の姉妹に発症し,抗ミトコンドリア抗体陰性,抗核抗体強陽性,PBCの肝組織像を呈した,いわゆる“免疫性胆管炎”に相当する2症例.肝臓 34 (12): 995-1001, 1993
– reference: 7) Michieletti P, Wanless IR, Heathcote EJ, et al: Antimitochondorial antibody negative primary biliary cirrhosis; a distinct syndorome of autoimmune cholangitis. Gut 35: 260-265, 1994
– reference: 3) Brünner G, Klinge O: Ein der chronischdestruierenden nicht-eitrigen Cholangitis ahnliches Krankheitsbild mit antinuklearen Antikorpern (Immunocholangitis). Dtsch Med Wschr 112: 1454-1458, 1987
– reference: 19) Mitchison HC, Palmer JM, James OFW, et al: A controlled trial of prednisolone treatment in primary biliary cirrhosis; three-year results. J Hepatol 15: 336-344, 1992
– reference: 10) Joplin RE, Johnson GD, Neuberger JM, et al: Destribution of pyruvate dehydrogenase dihydrolipoamid acetyltransferase (PDC-E2) and biliary epithelium from patients with primary biliary cirrhosis. Hepatology 19: 1375-1380, 1994
– reference: 14) Powell F, Schroeter AL, Dickson ED: Antinuclear antibodies in primary biliary cirrhosis. Lancet I: 288-289, 1984
– reference: 4) Carrougher JG, Shaffer RT, Goodman ZD, et al: A 33-year-old woman with an autoimmune syndrome. Semin Liver Dis 11: 256-262, 1991
– reference: 13) Nakanuma Y: Necroinflammatory changes in hepatic lobules in primary biliary cirrhosis with less well-define cholestatic changes. Hum Pathol 24: 378-383, 1993
– reference: 17) 稲垣恭孝,宮地清光,桐生恭好,他:慢性関節リウマチを合併した自己免疫性胆管炎の1例.日消誌91 (5): 1057-1061, 1994
– reference: 9) Ludwig J, Rosen CB, Watson KR, et al: Chronic cholestasis in a young man. Hepatology 20: 1351-1355, 1994
– reference: 2) Berg PA, Wiedmann KH, Kloppel G, et al: Serological classification of chronic cholestatic liver disease by the use of two different types of antimitochondrial antibodies. Lancet II: 1329-1332, 1980
– reference: 8) Goodman ZD, McNally PR, Ishack KG, et al: "Autoimmune cholangitis"; a variant of primary biliary cirrhosis [Abstract]. Hepatology 18: 109, 1993
– reference: 11) Lassoued K, Brenard R, Benhamou JP, et al: Antinuclear antibodies directed to a 200-kilodalton polypeptide of the nuclear envelope in primary biliary cirrhosis. Gastroenterology 99: 181-186, 1990
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SubjectTerms Autoimmune Cholangiopathy
Title Influence of autoantibodies on the pathology of primary biliary cirrhosis; with special reference to autoimmune cholangiopathy
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