Current epidemiology and clinical characteristics of autoimmune liver diseases in South Korea

Abstract

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.

Long‐term clinical outcomes in patients with autoimmune hepatitis according to treatment response in Asian country

“…Thus, we were not able to add these components of the criteria for AIH to scores resulting in more ‘probable’ AIH patients. Indeed, another study from Korea of 343 AIH patients classified 224 (72.5%) as ‘probable’ by AIH scores, which was similar to our results (68.7%) . Last, the primary objective of this study was not a complete analysis of patients with AIH, but was to investigate and compare the long‐term prognosis of AIH patients under adequate immunosuppression with sufficient treatment duration.…”

“…Consequently, autoimmunity is thought to play a pivotal role in the pathogenesis of this disease. The prevalence of AIH varies widely according to geographical region and ethnicity, ranging from 4.8 per 100 000 persons in South Korea to 24 per 100 000 persons in Europe . AIH is a relatively rare disease with a lower prevalence than other common liver diseases, and this rarity hampers a large‐scale clinical studies.…”

“…The prevalence of AIH varies widely according to geographical region and ethnicity, ranging from 4.8 per 100 000 persons in South Korea to 24 per 100 000 persons in Europe. 3,4 AIH is a relatively rare disease with a lower prevalence than other common liver diseases, and this rarity hampers a large-scale clinical studies. Another obstacle is that AIH is sometimes difficult to definitively diagnose because there is a wide spectrum of symptoms, serologies, autoimmune markers and histologies.…”

Additional fibrate treatment in UDCA‐refractory PBC patients

“…Initially, PBC may display mild biochemical abnormalities such as slight elevations in the levels of alkaline phosphatase (ALP) and/or gamma‐glutamyltransferase (GGT). As the disease progresses, non‐specific symptoms such as pruritus and fatigue develop, ultimately leading to cirrhosis or death due to liver failure . PBC patients have higher risk of hepatocellular carcinoma (HCC) compared to the general population, and ursodeoxycholic acid (UDCA) non‐responders have a higher risk of HCC compared to UDCA responders …”

“…As the disease progresses, nonspecific symptoms such as pruritus and fatigue develop, ultimately leading to cirrhosis or death due to liver failure. [1][2][3] PBC patients have higher risk of hepatocellular carcinoma (HCC) compared to the general population, and ursodeoxycholic acid (UDCA) non-responders have a higher risk of HCC compared to UDCA responders. 4,5 UDCA has been the only drug that modifies the course of PBC disease.…”