Autoimmune liver diseases including autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are rare diseases. The aim of this review is to examine the epidemiology and clinical characteristics of AIH and PBC in South Korea. There were 4,085 patients registered as AIH in the Rare Intractable Disease Registry of Korea between 2009-2013, with a median age of 56 years and female-to male ratio of 6.4. The age-adjusted incidence and prevalence of AIH were 1.07/100,000/year and 4.82/100,000 persons, respectively. Among the patients, 1.1% underwent liver transplantation, and case fatality was 2.18%. Liver cirrhosis at diagnosis was accompanied in 23%; liver biopsy was performed in 75.2%, and prednisolone therapy or prednisolone and azathioprine combination therapy was done in 73% with a remission rate of 86%. There were 2,824 patients with PBC (≥20 years) registered in Korea between 2009-2013 with a median age of 57 years and female-to male ratio of 6.2. The age-adjusted incidence and prevalence of PBC were 0.86/100,000/year and 4.75/100,000 persons, respectively. Among the patients, 2.5% underwent liver transplantation, and case fatality was 2.2% with a 5-year transplantation-free survival of 95.4%. Ursodeoxycholic acid (UDCA) was prescribed in 90% of the patients with a UDCA inadequate response rate of 30%. In conclusion, AIH and PBC are rare but mostly treatable diseases if diagnosed in the early stages. However, scarce data, low awareness, delayed diagnosis and non-availability of 2nd line therapeutics are important issues to be solved. Therefore, governmental support for research and drug development and nationwide cooperative studies are warranted.
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Background/Aims Exclusion of liver disease from other causes such as autoimmune hepatitis is necessary for diagnosis of nonalcoholic fatty liver disease (NAFLD). However, there has been no study on the prevalence and significance of autoantibodies in the patients with clinically suspected NAFLD in Korea, where hepatitis B is endemic and autoimmune hepatitis is relatively uncommon. Methods: We prospectively tested for anti- nuclear antibody (ANA), anti-smooth muscle antibody (ASMA), and anti-mitochondrial antibody (AMA) in 135 serially enrolled patients with suspected NAFLD. We compared the clinical characteristics and biochemical indices of the ANA-positive or ASMA-positive group with those of the autoantibody-negative group. Results: Sixteen patients (11.8%) had serum autoantibodies; there was ANA in 8 patients (5.9%), ASMA in 7 (5.1%), and AMA in 2 (1.5%). Both ANA and AMA were positive in one patient. The ANA-positive or ASMA-positive group showed an older age (49.5±13.0 vs. 42.0±10.9 years, respectively, P=0.018) and higher levels of serum globulin (3.1±0.4 vs. 2.9±0.4g/dL, respectively, P=0.037), compared with the autoantibody-negative group. Two cases with positive ANA or ASMA fulfilled the diagnostic criteria for probable autoimmune hepatitis and two cases with positive AMA were suspected as primary biliary cirrhosis. Conclusions: These findings suggest that autoantibodies could be found in some patients with suspected NAFLD in Korea, AMA-positivity or ASMA-positivity could be associated with old age and high serum globulin, and some of the autoantibody-positive cases could be diagnosed as autoimmune hepatitis or primary biliary cirrhosis. Further studies are necessary to clarify the clinical significance of autoantibody positivity in those patients. (Korean J Hepatol 2005;11:261-267)
Background/Aims Chronic hepatitis C virus (HCV) infection is often associated with extrahepatic autoimmune disease, and autoantibodies such as anti-nuclear antibody (ANA) or anti-smooth muscle antibody (ASA). The presence of autoantibodies may make discrimination between chronic hepatitis C with autoimmune features and type 1 autoimmune hepatitis difficult. We studied the prevalence of autoantibodies in patients with chronic HCV infection and their clinical significance. Materials and Methods: ANA, ASA, anti-mitochondrial antibody (AMA), anti-microsomal antibody (AmA), rheumatoid factor (RF), anti-cardiolipin antibody (aCL) and lupus anti-coagulant (LA) were tested in 116 patients (80 chronic hepatitis C, 36 liver cirrhosis). Genotypes of HCV were determined in 25 patients by INNO LiPA. Results: The overall prevalence of autoantibody was 65.5%. The most common autoantibody was aCL (34.5%), followed by ANA (25%), RF (18%), LA (15.5%), ASA (6.9%), anti-microsomal antibody (6%) and AMA (1%). The positive rate of either ANA or ASA was 30.2%, but both were positive in 1.7% only. There was no difference in the demographic features, biochemistry, HCV genotypes and disease status between autoantibody-positive and autoantibody-negative patients. Conclusions: Autoantibodies were commonly found in patients with chronic HCV infection. But, the presence of autoantibodies may be a non-specific finding in chronic hepatitis C infection without clinical significance. (Korean J Hepatol 1999;5:200－207)
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