Prognostic indicators in primary biliary cirrhosis: significance of revised IAHG (International Autoimmune Hepatitis Group) score
Background/AimsPrimary biliary cirrhosis (PBC) is a slowly progressing autoimmune disease of the liver that is characterized by portal inflammation and immune-mediated destruction of the intrahepatic bile ducts. Serum total bilirubin is one of the various prognostic factors that have been proposed....
| Main Authors: | , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Korean Association for the Study of the Liver
2012-12-01
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| Series: | Clinical and Molecular Hepatology |
| Subjects: | |
| Online Access: | http://e-cmh.org/upload/pdf/cmh-18-375.pdf |
| _version_ | 1819021255236386816 |
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| author | Ho Eun Jung Jae Young Jang Soung Won Jeong Jin Nyoung Kim Hee Yoon Jang Yun Ju Cho Sung Ae Woo Sae Hwan Lee Sang Gyune Kim Sang-Woo Cha Young Seok Kim Young Deok Cho Hong Soo Kim Boo Sung Kim |
| author_facet | Ho Eun Jung Jae Young Jang Soung Won Jeong Jin Nyoung Kim Hee Yoon Jang Yun Ju Cho Sung Ae Woo Sae Hwan Lee Sang Gyune Kim Sang-Woo Cha Young Seok Kim Young Deok Cho Hong Soo Kim Boo Sung Kim |
| author_sort | Ho Eun Jung |
| collection | DOAJ |
| description | Background/AimsPrimary biliary cirrhosis (PBC) is a slowly progressing autoimmune disease of the liver that is characterized by portal inflammation and immune-mediated destruction of the intrahepatic bile ducts. Serum total bilirubin is one of the various prognostic factors that have been proposed. A recent study found that PBC with accompanying autoimmune hepatitis (AIH) carries a negative prognosis. This study examined the clinical characteristics of PBC and analyzed the factors that affect its prognosis.MethodsPatients diagnosed with PBC between January 1998 and December 2010 based on clinical and histopathological findings were compiled and analyzed retrospectively.ResultsAmong 27 patients, 24 (1 male and 23 females, ages 50.0±9.3 years) were followed up. The follow-up period was 8.6±0.9 years. Of the 24 patients, 9 patients progressed to liver cirrhosis (LC). Comparison between patients who did and did not progress to LC revealed statistically significant differences in the patients' serum total bilirubin (2.7±1.8 vs. 0.8±0.4, P=0.012), the Mayo risk score (5.1±0.7 vs. 3.9±0.6, P=0.001), the revised IAHG (International Autoimmune Hepatitis Group) score (9.2±2.3 vs. 5.4±3.0, P=0.004) and frequency of AIH overlap (5/9 [55.6%] vs. 0/15 [0%], P=0.001) at the time of diagnosis.ConclusionsWe propose that serum total bilirubin, the Mayo risk score, and the revised IAHG score at the time of diagnosis are helpful for predicting PBC prognosis. In particular, since all of the patients with accompanying AIH progressed to LC, the presence of overlap syndrome at the time of diagnosis is helpful for predicting PBC prognosis and providing an adequate treatment. |
| first_indexed | 2024-12-21T04:04:12Z |
| format | Article |
| id | doaj.art-9317146c2a1a4313a6405c0bbc888839 |
| institution | Directory Open Access Journal |
| issn | 2287-2728 2287-285X |
| language | English |
| last_indexed | 2024-12-21T04:04:12Z |
| publishDate | 2012-12-01 |
| publisher | Korean Association for the Study of the Liver |
| record_format | Article |
| series | Clinical and Molecular Hepatology |
| spelling | doaj.art-9317146c2a1a4313a6405c0bbc8888392022-12-21T19:16:38ZengKorean Association for the Study of the LiverClinical and Molecular Hepatology2287-27282287-285X2012-12-0118437538210.3350/cmh.2012.18.4.3751006Prognostic indicators in primary biliary cirrhosis: significance of revised IAHG (International Autoimmune Hepatitis Group) scoreHo Eun Jung0Jae Young Jang1Soung Won Jeong2Jin Nyoung Kim3Hee Yoon Jang4Yun Ju Cho5Sung Ae Woo6Sae Hwan Lee7Sang Gyune Kim8Sang-Woo Cha9Young Seok Kim10Young Deok Cho11Hong Soo Kim12Boo Sung Kim13Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Institute for Digestive Research, Digestive Disease Center, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.Background/AimsPrimary biliary cirrhosis (PBC) is a slowly progressing autoimmune disease of the liver that is characterized by portal inflammation and immune-mediated destruction of the intrahepatic bile ducts. Serum total bilirubin is one of the various prognostic factors that have been proposed. A recent study found that PBC with accompanying autoimmune hepatitis (AIH) carries a negative prognosis. This study examined the clinical characteristics of PBC and analyzed the factors that affect its prognosis.MethodsPatients diagnosed with PBC between January 1998 and December 2010 based on clinical and histopathological findings were compiled and analyzed retrospectively.ResultsAmong 27 patients, 24 (1 male and 23 females, ages 50.0±9.3 years) were followed up. The follow-up period was 8.6±0.9 years. Of the 24 patients, 9 patients progressed to liver cirrhosis (LC). Comparison between patients who did and did not progress to LC revealed statistically significant differences in the patients' serum total bilirubin (2.7±1.8 vs. 0.8±0.4, P=0.012), the Mayo risk score (5.1±0.7 vs. 3.9±0.6, P=0.001), the revised IAHG (International Autoimmune Hepatitis Group) score (9.2±2.3 vs. 5.4±3.0, P=0.004) and frequency of AIH overlap (5/9 [55.6%] vs. 0/15 [0%], P=0.001) at the time of diagnosis.ConclusionsWe propose that serum total bilirubin, the Mayo risk score, and the revised IAHG score at the time of diagnosis are helpful for predicting PBC prognosis. In particular, since all of the patients with accompanying AIH progressed to LC, the presence of overlap syndrome at the time of diagnosis is helpful for predicting PBC prognosis and providing an adequate treatment.http://e-cmh.org/upload/pdf/cmh-18-375.pdfPrimary biliary cirrhosisPrognosisOverlap syndrome |
| spellingShingle | Ho Eun Jung Jae Young Jang Soung Won Jeong Jin Nyoung Kim Hee Yoon Jang Yun Ju Cho Sung Ae Woo Sae Hwan Lee Sang Gyune Kim Sang-Woo Cha Young Seok Kim Young Deok Cho Hong Soo Kim Boo Sung Kim Prognostic indicators in primary biliary cirrhosis: significance of revised IAHG (International Autoimmune Hepatitis Group) score Clinical and Molecular Hepatology Primary biliary cirrhosis Prognosis Overlap syndrome |
| title | Prognostic indicators in primary biliary cirrhosis: significance of revised IAHG (International Autoimmune Hepatitis Group) score |
| title_full | Prognostic indicators in primary biliary cirrhosis: significance of revised IAHG (International Autoimmune Hepatitis Group) score |
| title_fullStr | Prognostic indicators in primary biliary cirrhosis: significance of revised IAHG (International Autoimmune Hepatitis Group) score |
| title_full_unstemmed | Prognostic indicators in primary biliary cirrhosis: significance of revised IAHG (International Autoimmune Hepatitis Group) score |
| title_short | Prognostic indicators in primary biliary cirrhosis: significance of revised IAHG (International Autoimmune Hepatitis Group) score |
| title_sort | prognostic indicators in primary biliary cirrhosis significance of revised iahg international autoimmune hepatitis group score |
| topic | Primary biliary cirrhosis Prognosis Overlap syndrome |
| url | http://e-cmh.org/upload/pdf/cmh-18-375.pdf |
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