Retrospective analysis of autoimmune hepatitis–primary biliary cirrhosis overlap syndrome in Korea: characteristics, treatments, and outcomes
3
조회
0
다운로드
3
인용
0
참고문헌
키워드
초록
Background/Aims: Overlap syndrome of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) (AIH-PBC overlap syndrome) is a rare disease that has not been clearly characterized in Korean patients. This study investigated the clinical features of AIH-PBC overlap syndrome compared with those of AIH and PBC alone. Methods: This retrospective cohort study included 158 consecutive patients who were diagnosed as AIH (n=61), PBC (n=81), or AIH-PBC overlap syndrome (n=9) based on the Paris and the International Autoimmune Hepatitis Group (IAIHG) criteria from 2001 to 2011 in Korea. We compared the clinical features of these three groups retrospectively, including their biochemical characteristics, treatments, responses, and clinical outcomes. Results: The AIH-PBC overlap syndrome patients exhibited biochemical characteristics of both AIH and PBC, and showed a similar response to ursodeoxycholic acid (UDCA) monotherapy as for the PBC patients. However, the response of AIH-PBC overlap syndrome patients to UDCA and steroid combination therapy was worse than the response of AIH patients to steroid-based therapy (P=0.024). Liver cirrhosis developed more rapidly in AIH-PBC overlap syndrome patients than in AIH patients group (P=0.013), but there was no difference between AIH-PBC overlap syndrome patients and PBC patients. The rates of developing hepatic decompensation did not differ significantly between the groups. Conclusions: The AIH-PBC overlap syndrome patients exhibited a worse response to UDCA and steroid combination therapy and a faster cirrhotic progression compared with AIH patients. (Clin Mol Hepatol 2015;21:150-157)
Abstract
Background/Aims: Overlap syndrome of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) (AIH-PBC overlap syndrome) is a rare disease that has not been clearly characterized in Korean patients. This study investigated the clinical features of AIH-PBC overlap syndrome compared with those of AIH and PBC alone. Methods: This retrospective cohort study included 158 consecutive patients who were diagnosed as AIH (n=61), PBC (n=81), or AIH-PBC overlap syndrome (n=9) based on the Paris and the International Autoimmune Hepatitis Group (IAIHG) criteria from 2001 to 2011 in Korea. We compared the clinical features of these three groups retrospectively, including their biochemical characteristics, treatments, responses, and clinical outcomes. Results: The AIH-PBC overlap syndrome patients exhibited biochemical characteristics of both AIH and PBC, and showed a similar response to ursodeoxycholic acid (UDCA) monotherapy as for the PBC patients. However, the response of AIH-PBC overlap syndrome patients to UDCA and steroid combination therapy was worse than the response of AIH patients to steroid-based therapy (P=0.024). Liver cirrhosis developed more rapidly in AIH-PBC overlap syndrome patients than in AIH patients group (P=0.013), but there was no difference between AIH-PBC overlap syndrome patients and PBC patients. The rates of developing hepatic decompensation did not differ significantly between the groups. Conclusions: The AIH-PBC overlap syndrome patients exhibited a worse response to UDCA and steroid combination therapy and a faster cirrhotic progression compared with AIH patients. (Clin Mol Hepatol 2015;21:150-157)
참고문헌 (22)
- Geubel AP, "Responses to treatment can differentiate chronic active liver disease with cholangitic features from the primary biliary cirrhosis syndrome", Gastroenterology, 71, pp.444~449 (1976)
- Kenny RP, "Frequency and significance of antimitochondrial antibodies in severe chronic active hepatitis", Digestive diseases and sciences, 31, pp.705~711 (1986)
- Alvarez F, "International Autoimmune Hepatitis Group Report : review of criteria for diagnosis of autoimmune hepatitis", J Hepatol, 31, pp.929~938 (1999)
- Hennes EM, "Simplified criteria for the diagnosis of autoimmune hepatitis", Hepatology, 48, pp.169~176 (2008)
- Ho Eun Jung, "Prognostic indicators in primary biliary cirrhosis: significance of revised IAHG (International Autoimmune Hepatitis Group) score", Clinical and Molecular Hepatology, 18(4), pp.375~382 (2012), 대한간학회[DOI]
- Neuhauser M, "Autoimmune hepatitis-PBC overlap syndrome : a simplified scoring system may assist in the diagnosis", Am J Gastroenterol, 105, pp.345~353 (2010)
- 김민석, "원발성 담즙성 간경변증으로 진단되고 자가면역성 간염의 Feature를 보이는 한국인 환자에 대한 Simplified Scoring Criteria의 의미", 대한내과학회지, 84(2), pp.211~220 (2013), 대한내과학회[DOI]
- Chazouilleres O, "Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome : clinical features and response to therapy", Hepatology, 28, pp.296~301 (1998)
- Chazouilleres O, "Long term outcome and response to therapy of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome", J Hepatol, 44, pp.400~406 (2006)
- Tanaka A, "Primary biliary cirrhosis-Autoimmune hepatitis overlap syndrome : A rationale for corticosteroids use based on a nationwide retrospective study in Japan", Hepatol Res, 41, pp.877~886 (2011)
- Wang Q, "Epigenetic considerations and the clinical reevaluation of the overlap syndrome between primary biliary cirrhosis and autoimmune hepatitis", Journal of autoimmunity, 41, pp.140~145 (2012)
- Boberg KM, "Overlap syndromes : the International Autoimmune Hepatitis Group(IAIHG)position statement on a controversial issue", J Hepatol, 54, pp.374~385 (2011)
- Joshi S, "Primary biliary cirrhosis with additional features of autoimmune hepatitis : response to therapy with ursodeoxycholic acid", Hepatology, 35, pp.409~413 (2002)
- Manns MP, "Diagnosis and management of autoimmune hepatitis", Hepatology, 51, pp.2193~2213 (2010)
- Corpechot C, "Biochemical response to ursodeoxycholic acid and long‐term prognosis in primary biliary cirrhosis", Hepatology, 48, pp.871~877 (2008)
- European Association for the study of the Liver, "EASL Clinical Practice Guidelines : management of cholestatic liver diseases", J Hepatol, 51, pp.237~267 (2009)
- Czaja AJ, "Diagnosis and treatment of autoimmune hepatitis", Hepatology, 36, pp.479~497 (2002)
- Kuiper EM, "Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome", Clin Gastroenterol Hepatol, 8, pp.530~534 (2010)
- Zhao P, "Low incidence of positive smooth muscle antibody and high incidence of isolated IgM elevation in Chinese patients with autoimmune hepatitis and primary biliary cirrhosis overlap syndrome : a retrospective study", BMC Gastroenterol, 12, pp.1~4 (2012)
- Silveira MG, "Overlap of autoimmune hepatitis and primary biliary cirrhosis : long-term outcomes", The American journal of gastroenterology, 102, pp.1244~1250 (2007)
- Kim BH, "Clinical features of autoimmune hepatitis and comparison of two diagnostic criteria in Korea: A nationwide, multicenter study", Journal of gastroenterology and hepatology, 28, pp.128~134 (2013)
- Yokokawa J, "Overlap of primary biliary cirrhosis and autoimmune hepatitis:Characteristics, therapy, and long term outcomes", Journal of gastroenterology and hepatology, 25, pp.376~382 (2010)