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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

Treatment progress of patients with AIH-PBC overlap syndrome. UDCA, ursodeoxycholic acid; AIH, autoimmune hepatitis; PBC, primary biliary cirrhosis.
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Figure 1: Treatment progress of patients with AIH-PBC overlap syndrome. UDCA, ursodeoxycholic acid; AIH, autoimmune hepatitis; PBC, primary biliary cirrhosis.

Mentions: For the patients with overlap syndrome, treatment options were diverse. The treatment courses for each of patients are illustrated in Figure 1. Six patients were initially treated with UDCA monotherapy for more than 1 year, and 50% (2/4) of patients showed treatment responses according to the CO criteria. The result was comparable with that of the patients with PBC (P=0.574). Two patients received additional steroid-based therapy because of an incomplete normalization of liver enzyme, and five patients eventually received the combination therapy. One out of five patients (20%) achieved a clinical remission, showing poorer response rate than the outcome of the steroid-based therapy for the patients with AIH (P=0.024).


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)

Treatment progress of patients with AIH-PBC overlap syndrome. UDCA, ursodeoxycholic acid; 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 1: Treatment progress of patients with AIH-PBC overlap syndrome. UDCA, ursodeoxycholic acid; AIH, autoimmune hepatitis; PBC, primary biliary cirrhosis.
Mentions: For the patients with overlap syndrome, treatment options were diverse. The treatment courses for each of patients are illustrated in Figure 1. Six patients were initially treated with UDCA monotherapy for more than 1 year, and 50% (2/4) of patients showed treatment responses according to the CO criteria. The result was comparable with that of the patients with PBC (P=0.574). Two patients received additional steroid-based therapy because of an incomplete normalization of liver enzyme, and five patients eventually received the combination therapy. One out of five patients (20%) achieved a clinical remission, showing poorer response rate than the outcome of the steroid-based therapy for the patients with AIH (P=0.024).

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