Prevalence Of Autoimmune Hepatitis Research Articles

S2016 Rising Incidence and Prevalence of Autoimmune Hepatitis in a Southern European Country Cohort: Clinical Features and Treatment Response

S2016 Rising Incidence and Prevalence of Autoimmune Hepatitis in a Southern European Country Cohort: Clinical Features and Treatment Response

Global incidence and prevalence of autoimmune hepatitis, 1970–2022: a systematic review and meta-analysis

Autoimmune hepatitis (AIH) varies significantly in incidence and prevalence across countries and regions. We aimed to examine global, regional, and national trends in incidence and prevalence of AIH from 1970 to2022. We conducted a thorough search of the PubMed/MEDLINE, Embase, CINAHL, Google Scholar, and Cochrane databases from database inception to August 9, 2023, using the search term "autoimmune hepatitis" in combination with "incidence," "prevalence," or "trend." Only general population-based observational studies with larger samples sizes were considered for inclusion. Studies that recruited convenience samples, and those with fewer than 50 participants were excluded. Summary data were extracted from published reports. A random effects model was used and pooled estimates with 95% CI were used to calculate the incidence and prevalence of AIH. Heterogeneity was evaluated using the I2 statistic. The study protocol was registered with PROSPERO, CRD42023430138. A total of 37 eligible studies, encompassing more than 239 million participants and 55,839 patients with AIH from 18 countries across five continents, were included in the analysis. Global pooled incidence and prevalence of AIH were found to be 1.28 cases per 100,000 inhabitant-years (95% CI, 1.01-1.63, I2=99·51%; number of studies, 33; sample population, 220,673,674) and 15.65 cases per 100,000 inhabitants (95% CI, 13.42-18.24, I2=99·75%; number of studies, 26; sample population, 217,178,684), respectively. The incidence of AIH was greater in countries with high Human Development Index (>0.92), in North America and Oceania (compared with Asia), among females, adults (compared with children), and high latitude (>45°). Similar patterns in AIH prevalence were observed. Pooled AIH prevalence increased gradually from 1970 to 2019 (1970-1999; 9.95 [4.77-15.13], I2=95·58% versus 2015-2022; 27.91 [24.86-30.96], I2=99·32%; cases per 100,000 inhabitants). The overall incidence and prevalence of AIH, as well as some subgroup analyses of the studies, displayed asymmetry in the funnel plots, suggesting potential evidence of publication bias. AIH incidence and prevalence have increased significantly and exhibit substantial variation across regions worldwide. Further research is required to assess the incidence and prevalence of AIH, specifically in South America and Africa. National Research Foundation of Korea.

Sophoricoside attenuates autoimmune‑mediated liver injury through the regulation of oxidative stress and the NF‑κB signaling pathway.

The prevalence of autoimmune hepatitis (AIH) is increasing, yet specific pharmacotherapies remain to be explored. The present study aimed to investigate the effects of sophoricoside (SOP), a bioactive component of medical herbs, on AIH and to elucidate the underlying mechanisms. Bioinformatic approaches were used to predict the potential targets and underlying regulatory mechanisms of SOP on AIH. The effects of SOP on AIH were evaluated by determining the expression levels of inflammatory cytokines, histological liver injury and hepatic fibrosis in an improved chronic cytochrome P450 2D6 (CYP2D6)‑AIH mouse model and in a model of concanavalin‑A (ConA)‑induced acute immune‑mediated liver injury. The antioxidant activity of SOP was detected in invivo and invitro experiments. The selected signal targeted by SOP in AIH was further confirmed using western blot analysis and immunofluorescence staining. The results of bioinformatic analysis revealed that the targets of SOP in AIH were related to oxidative stress and the NF‑κB gene set. The NF‑κB transcription factor family is a key player that controls both innate and adaptive immunity. The activation of the NF‑κB signaling pathway is often associated with autoimmune disorders. In the animal experiments, SOP attenuated CYP2D6/ConA‑induced AIH, as evidenced by a significant reduction in the levels of hepatic enzymes in serum, inflammatory cytokine expression and histological lesions in the liver. The oxidative response in AIH was also significantly inhibited by SOP, as evidenced by a decrease in the levels of hepatic malondialdehyde, and elevations in the total antioxidant capacity and glutathione peroxidase levels. The results of the invivo and invitro experiments revealed that SOP significantly reduced the enhanced expression and nuclear translocation of phosphorylated p65 NF‑κB in the livers of mice with AIH and in lipopolysaccharide‑stimulated AML12 cells. On the whole, the present study demonstrates the protective role of SOP in AIH, which may be mediated by limiting the oxidative response and the activation of the NF‑κB signaling pathway in hepatocytes.

Estimating autoimmune hepatitis in patients with hepatic dysfunction

Autoimmune Hepatitis (AIH) is a chronic inflammatory disease of the liver of unknown aetiology characterized by polyclonal hypergammaglobulinemia and circulating autoantibodies. Objective of the study is to find out the prevalence of autoimmune hepatitis and its causative autoantibody (Anti-nuclear, anti-smooth muscle, anti-liver kidney microsomes-1) following comprehensive diagnostic criteria given by International Autoimmune Hepatitis Group. Material and methods250 patients with raised aminotransferase but, negative for the commonest three clinical conditions viz, viral hepatitis, drug induced liver injury and alcohol induced liver injury were suspected to be a case of autoimmune hepatitis. Recent IAIHG criteria was followed in making diagnosis of autoimmune hepatitis. We did the investigation for viral hepatitis (acute/chronic), drug induced liver injury and alcohol induced liver injury and then measured serum IgG. Indirect Immunofluorescence (IIF) was done for Anti-nuclear antibody and was also done for anti-smooth muscle antibody and anti-liver kidney microsomes-1 antibody simultaneously. ResultAs per IAIHG criteria, out of 250 patients, Ig “G” was elevated in 88 patients with significant titres for Anti-nuclear antibody (50 patients), anti-smooth muscle antibody (65 patients), anti-liver kidney microsomes-1 antibody (7 patients) and Anti-mitochondrial antibody (18 patients) were found. As per International Autoimmune Hepatitis Group Comprehensive scoring system, probable diagnosis was made in 83 patients (33.2%) and 12 were confirmed for autoimmune hepatitis (4.8%). ConclusionFemale preponderance was observed and type 1 Autoimmune Hepatitis was most common whereas type 2 Autoimmune Hepatitis was found in few cases. Most patients had anti-smooth muscle antibody and anti-nuclear antibody positivity indicating the type, but no significant difference was seen in clinical symptoms or biochemical parameters between the different types. 162 patients couldn't be evaluated for autoimmune hepatitis because of serum IgG cut-off criteria >1500 ​mg/l.

Prevalence of autoimmune, cholestatic and nonalcoholic fatty liver disease in celiac disease.

While there is higher prevalence of autoimmune, cholestatic and fatty liver disease in celiac disease (CeD), most data is from small-scale studies. We evaluated the prevalence and risk factors of the same using large cohort data. A population-based cross-sectional study was conducted using Explorys, a multi-institutional database. Prevalence and risk factors of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and nonalcoholic fatty liver disease (NAFLD) in CeD were assessed. Out of 70 352 325 subjects, 136 735 had CeD (0.19%). The prevalence of AIH (0.32%), PBC (0.15%), PSC (0.004%) and NAFLD (0.7%) were high in CeD. After adjusting for age, gender, Caucasian race and anti-tissue transglutaminase antibody (anti-TTG), CeD subjects had higher odds of AIH [adjusted odds ratio (aOR) 7.06, 95% confidence interval (CI) 6.32-7.89] and PBC (aOR 4.16, 95% CI 3.46-5.0). Even after adjusting for CeD, anti-TTG positivity concurred with higher odds of AIH (aOR 4.79, 95% CI 3.88-5.92) and PBC (aOR 9.22, 95% CI 7.03-12.1). After adjusting for age, gender, Caucasian race, diabetes mellitus (DM), obesity, hypothyroidism and metabolic syndrome, there was higher prevalence of NAFLD in CeD, with the aOR in the presence of DM type 1 being 2.1 (95% CI 1.96-2.25), and in the presence of DM type 2 being 2.92 (95% CI 2.72-3.14). Subjects with CeD are more likely to have AIH, PBC, PSC and NAFLD. AIH and PBC have higher odds in the presence of anti-TTG. The odds of NAFLD in CeD are high regardless of type of DM.

Characteristics and Long-Term Outcome of 535 Patients with Autoimmune Hepatitis-The 20-Year Experience of a High-Volume Tertiary Center.

Background and aims: Autoimmune hepatitis (AIH) is a complex and progressive inflammatory liver disease characterized by immune-mediated destruction of the liver parenchyma, hypergammaglobulinemia, the presence of circulating autoantibodies, and good response to immunosuppressive therapy. Since the prevalence of AIH is relatively rare, data on the clinical course and the long-term outcome are scarce. Patients and methods: We retrospectively analyzed the data of 535 well-documented AIH patients treated at the University Hospital Essen between 2000 and 2020. Results: The majority of patients were middle-aged females (75% women, mean age 45 years) with AIH type 1 (97%). Approximately 32% of patients were diagnosed with cirrhosis due to AIH, 29% had concomitant autoimmune (predominantly autoimmune thyroiditis), and 10% had psychiatric diseases, respectively. Skin tumors were the most common malignant diseases (47% of all tumors), while hepatocellular carcinoma rarely occurred (only six cases). Overall long-term mortality and liver-associated mortality were 9.16% and 4.67%, respectively. However, long-term survival was strongly associated with disease remission. Conclusions: Although AIH is a silent disease and cirrhosis is present in many cases, a favorable long-term prognosis can be achieved by consequent immunosuppressive therapy. The incidence of (liver-associated) complications seems to be lower in comparison to other etiologies, such as viral hepatitis or NASH, and mainly depends on the long-term side effects of immunosuppressive therapy.

A nationwide study of autoimmune liver diseases in the Faroe Islands: Incidence, prevalence, and causes of death 2004 - 2021

ABSTRACT Background and aims: Autoimmune liver diseases are rare diseases, and population-based studies on the epidemiology of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are sparse. We aimed to assess the incidence of AIH, PBC, and PSC in the Faroe Islands. Methods: All cases of AIH, PBC, and PSC diagnosed in the Faroe Islands between January 1st, 2004, and December 31st, 2021, were included in this nationwide registry-based cohort study. In addition, we searched all medical records to assess diagnostic criteria and cause of death. Results: The incidences of AIH, PBC, and PSC in the Faroe Islands were 5.2, 2.5 and 0.7 per 100,000 population per year, respectively. Point prevalence per 100,000 population on December 31st 2021, was 71.8 for AIH, 38.5 for PBC, and 11.0 for PSC. Nine AIH patients died after a median of 3 years, three died of hepatocellular carcinoma (HCC), and two died of liver failure. Five PBC patients died after a median of 7 years, one of HCC and one of liver failure. One PSC patient died of cholangiocarcinoma. Conclusion: The incidence and prevalence of AIH, PBC and PSC in the Faroe Islands are among the highest reported in population-based settings.

Epidemiology of Autoimmune Liver Disease

Autoimmune liver disease is an important immune-mediated pathologic entity involving the liver and intrahepatic bile duct, including autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis. Although it is necessary to ascertain its presence in acute or chronic liver disease without common causes, it is not easy to diagnose this disease straightforwardly because of its rarity. Recently, the incidence and prevalence of autoimmune hepatitis and primary biliary cholangitis have increased in several regions. In contrast, there is limited data dealing with the trend of the epidemiology of primary sclerosing cholangitis worldwide. Physicians should consider the epidemiologic characteristics of autoimmune liver disease because early diagnosis and proper treatment might prevent the progression of advanced liver disease. In addition, more sophisticated epidemiologic studies will be needed to elucidate the trend of these rare diseases nationwide.

HLA-related genetic susceptibility in autoimmune hepatitis according to autoantibody profile.

Although the prevalence of autoimmune hepatitis in first-degree relatives is small, the relationship between genetic markers, especially human leucocyte antigens (HLA), and susceptibility to this disease, has been studied for over three decades. The genetic susceptibility to AIH is believed to be different in the two subtypes of the disease, AIH type 1 and AIH type 2. Type 1 AIH has anti-smooth muscle and anti-nuclear antibodies as its main markers, while those of type 2 AIH are the anti-liver/kidney microsome type 1 and anti-liver cytosol type 1 antibodies. The anti-soluble liver antigen/liver-pancreas antibodies, which, in addition to being present in both subtypes, mark an important number of patients without serological markers. Therefore, a third type of disease is questionable. The vast majority of immunogenetic studies compare the differences between the two main types and make no difference between which antibodies are present to define the subtype. This review seeks to analyze what was most important published in the AIH in this context, trying to relate the HLA alleles according to the AIH marker autoantibodies.

Autoimmune Hepatitis Prevalence among Patients from Saudi Arabia with Chronic Liver Disease Referred for Transient Elastography

Background and study aim: Autoimmune hepatitis (AIH) is a chronic liver disease that can lead to progressive liver damage. The prevalence of AIH among the general population and among chronic liver disease (CLD) patients is variable worldwide. Currently, no published data on the prevalence of AIH among Saudi or among liver disease patients from the region. In this study, we aimed to assess the prevalence of AIH among CLD patients who were referred for transient elastography (FibroScan) in Saudi Arabia. Patients and methods: A retrospective study was conducted among CLD patients who had a transient elastography (FibroScan) during the 3-year study period. We obtained demographic data and test results for serum alanine aminotransferase (ALT), bilirubin, hemoglobin (Hgb), platelets, and international normalized ratio (INR) from all patients. We compared the results of the AIH patients to those of the non-AIH patients with CLD. In addition, for AIH patients, we measured serum immunoglobulin G (IgG), antinuclear antibody (ANA), smooth muscle antibody (SMA) and ALT at 2 - 6 weeks and at 3 - 4 months. Results: We included 494 patients, who were predominantly female (271 (60%)) and Saudi (299 (60.5%)). Thirty patients (6.1%) had AIH, which represented the 4th most common liver disease. Compared to non-AIH patients, AIH patients were younger (mean ages 49.9 years, SD 14.22 years, and 40.4 years, SD 13.94 years, respectively, P = 0.001). Patients with AIH had significantly lower Hgb and platelets (P = 0.008 for both) and higher ALT, bilirubin and INR (P = 0.05, 0.047 and 0.019, respectively). More than 50% of the AIH patients had cirrhosis. Older age was not associated with advanced disease stage among the AIH patients (P th most common cause of CLD in patients referred for transient elastography in Saudi Arabia. AIH tends to be severe in patients at a young age, with a marked biochemical response to steroids and azathioprine treatment.

Sjögren’s Syndrome Complicated with Type 2 Autoimmune Hepatitis

This case details a 33-year-old female who presented with a suggestive autoimmune history, arthralgias, and splenomegaly, and tested positive for Sjögren’s syndrome and anti-liver–kidney microsomal antibody. This was further validated by findings from a liver biopsy, confirming a very rare association with Type 2 autoimmune hepatitis. Primary Sjögren's syndrome is a sporadic disease with a global prevalence of 61 per 100,000 people and a total prevalence of 0.4% for secondary Sjögren's syndrome. The prevalence of autoimmune hepatitis in association with primary Sjögren's syndrome is 4–47%. It is divided into two types, associated with characteristic antibodies. Type 2 autoimmune hepatitis is rarely reported with Sjögren’s syndrome; much of the association reported in the literature has been with Type 1 autoimmune hepatitis.

Incidence, prevalence and mortality of autoimmune hepatitis in England 1997-2015. A population-based cohort study.

There are few population-based studies of the incidence and mortality of autoimmune hepatitis. The burden of the disease and how it has changed over time have not been fully explored. We conducted a population-based cohort study on the incidence and mortality of autoimmune hepatitis in England, 1997-2015. From the Clinical Practice Research Datalink we included 882 patients diagnosed with autoimmune hepatitis in England, 1997-2015. The patients were followed through 2015, and we calculated the sex- and age-standardized incidence and prevalence of autoimmune hepatitis. We examined variation in incidence by sex, age, calendar year, geographical region and socioeconomic status, and incidence rate ratios were calculated with Poisson regression. We calculated all-cause and cause-specific mortality. The overall standardized incidence rate of autoimmune hepatitis was 2.08 (95% confidence interval 1.94-2.22) per 100,000 population per year, higher in women, higher in older age and independent of region and socioeconomic status. From 1997 to 2015 the incidence doubled from 1.27 (95% confidence interval 0.51-2.02) to 2.56 (95% confidence interval 1.79-3.33) per 100,000 population per year. The 10-year cumulative all-cause mortality was 31.9% (95% confidence interval 27.6-36.5), and the 10-year cumulative liver-related mortality, including hepatocellular carcinoma was ~10.5%. This population-based study showed that the incidence of autoimmune hepatitis doubled over an eighteen-year period. The incidence was particularly high in older women and was similar across all regions of England and independent of socioeconomic status. Patients with autoimmune hepatitis had a high mortality.

Systematic review and meta-analysis on the incidence and prevalence of autoimmune hepatitis in Asian, European, and American population.

Reported incidence and prevalence rates of autoimmune hepatitis (AIH) have been sparse and heterogeneous. The aim of this meta-analysis was to estimate the worldwide incidence and prevalence rates of AIH and reveal population difference. Published articles on the epidemiology of AIH in PubMed, Embase, and Cochrane Library were systematically searched from inception to April 28, 2019. Two investigators independently reviewed these literatures and evaluated their quality. A random-effects model was used to pool the overall incidence and prevalence rates. The impact of population difference, gender, age, study period, study quality, diagnostic criteria, and study design was further analyzed with subgroup analysis and meta-regression. A total of 22 studies were included in the meta-analysis. The pooled worldwide annual incidence and prevalence of AIH were 1.37 (95% confidence interval [CI]: 0.95-1.80) and 17.44 (95% CI: 12.01-22.87) per 100000 persons, respectively. Subgroup analysis showed that the pooled annual incidence for Asian, European, and American population was 1.31 (95% CI: 0.42-2.20), 1.37 (95% CI: 1.10-1.64), and 1.00 (95% CI: 0.44-1.56) per 100000 persons, respectively; the pooled prevalence for Asian, European, and American population was 12.99 (95% CI: 2.05-23.92), 19.44 (95% CI: 15.63-23.24), and 22.80 (95% CI: -13.48 to 59.07) per 100000 persons, respectively. In addition, higher incidence and prevalence rates were observed in women than men, and a higher prevalence rate was observed in elderly than young people. Autoimmune hepatitis is a rare disease, with a similar incidence worldwide and a higher prevalence in European and American than in Asian population.

765 – Prevalence of Autoimmune Hepatitis in the United States from 2013 to 2018: A Population-Based National Study

765 – Prevalence of Autoimmune Hepatitis in the United States from 2013 to 2018: A Population-Based National Study

FRI-024-Incidence and prevalence of autoimmune hepatitis in England 1997-2015. A population-based cohort study

FRI-024-Incidence and prevalence of autoimmune hepatitis in England 1997-2015. A population-based cohort study

Incidence, prevalence, and causes of death of patients with autoimmune hepatitis: A nationwide register-based cohort study in Finland

BackgroundEpidemiological studies of autoimmune hepatitis are scarce and often based on single centre registries. AimsWe conducted a nationwide register study of incidence, prevalence, survival, and causes of death of autoimmune hepatitis patients in Finland. MethodsAutoimmune hepatitis cases 1995–2015 were retrieved from the national database of special reimbursements for drugs costs. Data on causes of death were retrieved from Statistics Finland. ResultsAfter incomplete registration of AIH during the first years, the incidence of autoimmune hepatitis stabilised to 1.1/100,000 person-years (1.6 in women and 0.52 in men) in 2008–2015. The prevalence of autoimmune hepatitis at the end of 2015 was 14.3/100,000, 23.0/100,000 in women and 6.6/100,000 in men. The all-cause standardized mortality ratio (SMR) of autoimmune hepatitis patients was 1.81 (95% confidence interval (CI) 1.47–2.20). The SMR was increased in all age groups and in both sexes. The SMR for hepatocellular carcinoma was 20.6 (95% CI 10.3–36.8), and for digestive diseases in overall 13.5 (95% CI 8.2–20.8), constituting mainly from autoimmune hepatitis and liver cirrhosis. ConclusionIncidence of autoimmune hepatitis has remained stable, with clear female predominance. Autoimmune hepatitis is associated with a markedly increased risk of death with hepatocellular cancer forming the greatest risk.

Alternative treatment regimens in autoimmune hepatitis: how justified is their choice?

Autoimmune hepatitis is a progressive immune-mediated liver disease of unknown etiology. Its key characteristics include hyper-gammaglobulinemia, circulating autoantibodies, and periportal inflammation seen in a liver biopsy sample. It is not infrequent that the lack of unified diagnostic tests makes the verification of the disease very challenging. Most patients respond well to standard immunosuppressive therapy; however, a significant proportion of them demonstrate side effects and disease relapses after treatment withdrawal. A wide range of side effects of systemic steroids and eventual disruptions with azathioprine (the agent of choice in the treatment algorithms for autoimmune hepatitis) supplies to the Russian market make it relevant to use alternative treatment regimens. In the real world practice, alternative treatment regimens are rarely used in such patients due to the absence of hard evidence of their efficacy. Low prevalence of autoimmune hepatitis, multiplicity of its clinical types, as well as a lack of understanding of its pathogenetic mechanisms hinder the synthesis of new agents and performing trials with already known immunosuppressants with a statistical power necessary to obtain persuasive data. One of solutions of the problem could be the accumulation of clinical data into registries for further systematization of the knowledge and formulation of new clinical guidelines.

Accuracy of the Simplified Criteria for Autoimmune Hepatitis in Children: Systematic Review and Decision Analysis

Several studies have been conducted on the accuracy of simplified criteria for autoimmune hepatitis that were presented in 2008 as an alternative to original criteria. Our purpose is to summarize the evidence available regarding their accuracy in children and to carry out a basic clinical decision analysis based on it. Electronic and manual searches were performed with keywords related to diagnostic validity terms. Data from included studies were extracted, and summary estimates of accuracy measures were calculated. An effect model was chosen depending on heterogeneity, and the presence of publication bias was also studied. Therapeutic threshold was calculated based on the already published data. Through a Bayesian approach, simplified criteria's clinical utility was simulated, taking into account the meta-analyzed indicators and several assumptions on the prevalence of autoimmune hepatitis. The search yielded 166 studies, four of which were finally included, providing a total population of 437 patients. Pooled sensitivity and specificity of the simplified criteria for the diagnosis of autoimmune hepatitis in children was 77% and 95%, respectively, with a diagnostic odds ratio of 67. No evidence of publication bias was found. For prevalences ranging from 8.5 to 85.7, the predictive value of either a positive or a negative result moved beyond the therapeutic threshold (estimated at 56%). The simplified criteria show high specificity and moderate sensitivity for the diagnosis of autoimmune hepatitis in children. A positive result can justify starting a therapeutic assay, but a negative result does not seem sufficient to rule out this condition.

Diabetes Mellitus and Autoimmune Hepatitis: Demographical and Clinical Description of a Relatively Rare Phenotype.

We studied demographic, metabolic, and clinical characteristics of patients with diabetes and autoimmune hepatitis (AIH) from the German/Austrian DPV registry. A total of 139 patients with diabetes and AIH were analyzed and compared to 437 728 patients with diabetes without AIH. The prevalence of AIH in patients with T1DM (44.8/100 000) seems higher than in the general population, the prevalence of AIH in patients with T2DM (23.6/100 000) does not seem to be increased. Patients with T2DM and AIH had a shorter duration of diabetes (p=0.007) and a higher proportion of females (p<0.001) compared to T2DM without AIH. Patients with diabetes (T1DM or T2DM) and AIH required higher insulin doses (p<0.001 and p=0.03, respectively) and showed increased liver enzymes (aspartate transaminase, alanine transaminase, gamma-glutamyltransferase) compared to diabetes patients without (all p<0.001). We detected a lower percentage of patients treated with oral antidiabetic drugs (p=0.01) and a higher percentage of patients treated by insulin in patients with T2DM and AIH (p<0.001) compared to patients with T2DM alone. We observed a higher incidence of autoimmune thyroid disease (AIT) in patients with diabetes (T1DM or T2DM) and AIH (p<0.001) compared to diabetes patients without AIH. AIH seems more frequent in patients with T1DM. Patients with diabetes and AIH require intensification of antidiabetic therapy and seem to have a higher prevalence of AIT.

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

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.