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Retrospective analysis of autoimmune hepatitis-primary biliary cirrhosis overlap syndrome in Korea: characteristics, treatments, and outcomes.

Park Y, Cho Y, Cho EJ, Kim YJ - Clin Mol Hepatol (2015)

Bottom Line: We compared the clinical features of these three groups retrospectively, including their biochemical characteristics, treatments, responses, and clinical outcomes.The rates of developing hepatic decompensation did not differ significantly between the groups.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine and Liver Research institute, Seoul National University College of Medicine, Seoul, Korea.

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.

No MeSH data available.


Related in: MedlinePlus

Time-to-progression curves of liver cirrhosis for patients with AIH-PBC overlap syndrome (dash-dotted line), PBC (solid line), and AIH (dashed line). The 1-year and 5-year progression rate were 3.4% and 24.4%, respectively, for the PBC patients, 4.7% and 9.8% for the AIH patients, and 0.0% and 34.4% for the AIH-PBC overlap syndrome patients. Liver cirrhosis developed significantly faster in patients with AIH-PBC overlap syndrome than in the AIH patients (P=0.013). The progression rate of the AIH-PBC overlap syndrome patients did not differ significantly from that of the PBC patients (P=0.163). AIH, autoimmune hepatitis; PBC, primary biliary cirrhosis.
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Figure 2: Time-to-progression curves of liver cirrhosis for patients with AIH-PBC overlap syndrome (dash-dotted line), PBC (solid line), and AIH (dashed line). The 1-year and 5-year progression rate were 3.4% and 24.4%, respectively, for the PBC patients, 4.7% and 9.8% for the AIH patients, and 0.0% and 34.4% for the AIH-PBC overlap syndrome patients. Liver cirrhosis developed significantly faster in patients with AIH-PBC overlap syndrome than in the AIH patients (P=0.013). The progression rate of the AIH-PBC overlap syndrome patients did not differ significantly from that of the PBC patients (P=0.163). AIH, autoimmune hepatitis; PBC, primary biliary cirrhosis.

Mentions: During the median follow-up period of 60 months (range, 0.2-155.5 months), patients were followed-up for the development of liver cirrhosis and hepatic decompensation. Among the patients with initially non-cirrhotic liver, 24.1% (15/62) of the PBC patients and 13.2% (7/53) of the AIH patients progressed to liver cirrhosis during the follow-up period, respectively. Half of the overlap syndrome patients (4/8) eventually developed liver cirrhosis. The overlap syndrome patients had a significantly shorter time-to-progression of liver cirrhosis (1-year and 5-year progression rate, 0.0% and 34.4%, respectively) than that of the patients with AIH (1-year and 5-year progression rate, 4.7% and 9.8%, respectively, P=0.013). However, there was no difference between the patients with overlap syndrome and the patients with PBC (1-year and 5-year progression rate, 3.4% and 24.4%, respectively, P=0.163) (Fig. 2).


Retrospective analysis of autoimmune hepatitis-primary biliary cirrhosis overlap syndrome in Korea: characteristics, treatments, and outcomes.

Park Y, Cho Y, Cho EJ, Kim YJ - Clin Mol Hepatol (2015)

Time-to-progression curves of liver cirrhosis for patients with AIH-PBC overlap syndrome (dash-dotted line), PBC (solid line), and AIH (dashed line). The 1-year and 5-year progression rate were 3.4% and 24.4%, respectively, for the PBC patients, 4.7% and 9.8% for the AIH patients, and 0.0% and 34.4% for the AIH-PBC overlap syndrome patients. Liver cirrhosis developed significantly faster in patients with AIH-PBC overlap syndrome than in the AIH patients (P=0.013). The progression rate of the AIH-PBC overlap syndrome patients did not differ significantly from that of the PBC patients (P=0.163). AIH, autoimmune hepatitis; PBC, primary biliary cirrhosis.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4493358&req=5

Figure 2: Time-to-progression curves of liver cirrhosis for patients with AIH-PBC overlap syndrome (dash-dotted line), PBC (solid line), and AIH (dashed line). The 1-year and 5-year progression rate were 3.4% and 24.4%, respectively, for the PBC patients, 4.7% and 9.8% for the AIH patients, and 0.0% and 34.4% for the AIH-PBC overlap syndrome patients. Liver cirrhosis developed significantly faster in patients with AIH-PBC overlap syndrome than in the AIH patients (P=0.013). The progression rate of the AIH-PBC overlap syndrome patients did not differ significantly from that of the PBC patients (P=0.163). AIH, autoimmune hepatitis; PBC, primary biliary cirrhosis.
Mentions: During the median follow-up period of 60 months (range, 0.2-155.5 months), patients were followed-up for the development of liver cirrhosis and hepatic decompensation. Among the patients with initially non-cirrhotic liver, 24.1% (15/62) of the PBC patients and 13.2% (7/53) of the AIH patients progressed to liver cirrhosis during the follow-up period, respectively. Half of the overlap syndrome patients (4/8) eventually developed liver cirrhosis. The overlap syndrome patients had a significantly shorter time-to-progression of liver cirrhosis (1-year and 5-year progression rate, 0.0% and 34.4%, respectively) than that of the patients with AIH (1-year and 5-year progression rate, 4.7% and 9.8%, respectively, P=0.013). However, there was no difference between the patients with overlap syndrome and the patients with PBC (1-year and 5-year progression rate, 3.4% and 24.4%, respectively, P=0.163) (Fig. 2).

Bottom Line: We compared the clinical features of these three groups retrospectively, including their biochemical characteristics, treatments, responses, and clinical outcomes.The rates of developing hepatic decompensation did not differ significantly between the groups.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine and Liver Research institute, Seoul National University College of Medicine, Seoul, Korea.

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.

No MeSH data available.


Related in: MedlinePlus