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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 hepatic decompensation for patients with AIH-PBC overlap syndrome (dash-dotted line), PBC (solid line), and AIH (dashed line). The progression curve for AIH-PBC patients (1-year and 5-year progression rates both 0.0%) did not differ from that of PBC patients (1-year and 5-year progression rates of 2.7% and 11.4%, respectively; P=0.740) or AIH patients (1-year and 5-year progression rates of 2.9% and 7.6%, respectively; P=0.996). AIH, autoimmune hepatitis; PBC, primary biliary cirrhosis.
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Figure 3: Time-to-progression curves of hepatic decompensation for patients with AIH-PBC overlap syndrome (dash-dotted line), PBC (solid line), and AIH (dashed line). The progression curve for AIH-PBC patients (1-year and 5-year progression rates both 0.0%) did not differ from that of PBC patients (1-year and 5-year progression rates of 2.7% and 11.4%, respectively; P=0.740) or AIH patients (1-year and 5-year progression rates of 2.9% and 7.6%, respectively; P=0.996). AIH, autoimmune hepatitis; PBC, primary biliary cirrhosis.

Mentions: During the follow-up period, the development of hepatic decompensation was also evaluated, including the presentation of symptoms, the incidences of liver-related mortality and liver transplantation. Among all the patients, 13.6% (11/81) of the patients with PBC and 8.8% (6/68) of the patients with AIH developed hepatic decompensation during the follow-up period. For the patients with overlap syndrome, one out of the nine patients manifested the symptom of hepatic decompensation. Time-to-progression of hepatic decompensation were analyzed for each group with the Kaplan-Meier method (Fig. 3). The 1-year and 5-year cumulative progression rates were 2.7% and 11.4% for the PBC patients; 2.9% and 7.6% for the AIH patients; and both 0.0% for the overlap syndrome patients, respectively. There was no difference between each groups (overlap syndrome vs. PBC, P=0.740; overlap syndrome vs. AIH, P=0.996).


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 hepatic decompensation for patients with AIH-PBC overlap syndrome (dash-dotted line), PBC (solid line), and AIH (dashed line). The progression curve for AIH-PBC patients (1-year and 5-year progression rates both 0.0%) did not differ from that of PBC patients (1-year and 5-year progression rates of 2.7% and 11.4%, respectively; P=0.740) or AIH patients (1-year and 5-year progression rates of 2.9% and 7.6%, respectively; P=0.996). 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 3: Time-to-progression curves of hepatic decompensation for patients with AIH-PBC overlap syndrome (dash-dotted line), PBC (solid line), and AIH (dashed line). The progression curve for AIH-PBC patients (1-year and 5-year progression rates both 0.0%) did not differ from that of PBC patients (1-year and 5-year progression rates of 2.7% and 11.4%, respectively; P=0.740) or AIH patients (1-year and 5-year progression rates of 2.9% and 7.6%, respectively; P=0.996). AIH, autoimmune hepatitis; PBC, primary biliary cirrhosis.
Mentions: During the follow-up period, the development of hepatic decompensation was also evaluated, including the presentation of symptoms, the incidences of liver-related mortality and liver transplantation. Among all the patients, 13.6% (11/81) of the patients with PBC and 8.8% (6/68) of the patients with AIH developed hepatic decompensation during the follow-up period. For the patients with overlap syndrome, one out of the nine patients manifested the symptom of hepatic decompensation. Time-to-progression of hepatic decompensation were analyzed for each group with the Kaplan-Meier method (Fig. 3). The 1-year and 5-year cumulative progression rates were 2.7% and 11.4% for the PBC patients; 2.9% and 7.6% for the AIH patients; and both 0.0% for the overlap syndrome patients, respectively. There was no difference between each groups (overlap syndrome vs. PBC, P=0.740; overlap syndrome vs. AIH, P=0.996).

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