Korean J Hepatol.
2010 Jun;16(2):139-146. 10.3350/kjhep.2010.16.2.139.
Clinical features and prognosis of primary biliary cirrhosis in Korea
- Affiliations
-
- 1Department of Internal Medicine, Ilsan Paik Hospital, Inje University, Korea.
- 2Department of Internal Medicine, Seoul National University Bundang Hospital, Korea. jsh@snubh.org
- 3Department of Internal Medicine, Inha University College of Medicine, Korea.
- 4Department of Internal Medicine, Korea University College of Medicine, Korea.
- 5Department of Internal Medicine, Yeungnam University College of Medicine, Korea.
- 6Department of Internal Medicine, Konkuk University School of Medicine, Korea.
- 7Department of Internal Medicine, The Catholic University College of Medicine, Korea.
- 8Department of Internal Medicine, Gyeongsang University College of Medicine, Korea.
- KMID: 1792592
- DOI: http://doi.org/10.3350/kjhep.2010.16.2.139
Abstract
- BACKGROUND/AIMS
This study investigated the clinical features and prognosis of primary biliary cirrhosis (PBC) in Korea.
METHODS
Clinical data of patients diagnosed as PBC between 1997 and 2008 at eight referral hospitals were analyzed retrospectively. PBC was diagnosed based on liver function tests, presence of serum antimitochondrial antibody (AMA), and histopathological findings.
RESULTS
In total, 251 patients (218 females, 33 males; mean age 54 years) were enrolled, and the mean follow-up duration was 33.5 months. At the diagnosis, 61% of the patients were asymptomatic, 12% had decompensated liver cirrhosis, and 98% were positive for AMA. The serum alkaline phosphate (ALP) level was 2.6 times the upper limit of normal, aspartate aminotransferase was 105 U/L, and bilirubin was 2.0 mg/dL. The mean Mayo risk score was 5.5, and the Child-Pugh class was A, B, and C in 79%, 19%, and 2% of the patients, respectively. Ursodeoxycholic acid (UDCA) was used for treatment in 88% of the patients, among which 70% exhibited biochemical responses defined as normalization or a >40% decrease in ALP at 6 months. Eight deaths occurred during the follow-up; the causes were variceal bleeding, hepatic failure, and sepsis. The overall 5-year survival rate was 95%. The poor prognostic factors were being older than 60 years, high bilirubin, low albumin, ascites, high Mayo risk score, Child-Pugh class C, and initial presence of hepatic decompensation.
CONCLUSIONS
Most patients diagnosed as PBC were asymptomatic, and these patients had a favorable short-term prognosis. The prognosis of PBC was dependent on the initial severity of liver disease.
MeSH Terms
-
Adult
Age Factors
Aged
Aged, 80 and over
Autoantibodies/metabolism
Bacterial Proteins/blood
Endopeptidases/blood
Female
Humans
Liver Cirrhosis, Biliary/*diagnosis/drug therapy/mortality
Liver Function Tests
Male
Middle Aged
Prognosis
Republic of Korea
Retrospective Studies
Severity of Illness Index
Survival Rate
Ursodeoxycholic Acid/therapeutic use
- Full Text Links
-
- Actions
-
Cited
- CITED
-
- Close
- Share
-
- Similar articles
-
- Prognosis of Korean patients with primary biliary cirrhosis
- Primary Biliary Cirrhosis-Autoimmune Hepatitis Overlap Syndrome
- Four cases of autoimmune cholangitis
- A case of the overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis
- Hepatocellular Carcinoma in Primary Biliary Cirrhosis: A Case Report
