Materiały źródłowe

• #1 Autoimmune Hepatitis: Epidemiology, Subtypes, and Presentation – PubMed

  https://pubmed.ncbi.nlm.nih.gov/37945151/

  Autoimmune hepatitis (AIH) is a chronic immunologic disorder in which the immune system targets the liver. The disease has a genetic basis and this accounts for the epidemiologic variation observed in serologic testing and clinical presentation across different populations. The incidence of AIH increases with age into the 70s and seems to be increasing in prevalence. […] Most patients test positive for antinuclear antibody, ASMA, or anti-LKM but about 20% of patients do not have these serologic markers. At clinical presentation, patients may be asymptomatic, symptomatic, have acute liver failure, or decompensated cirrhosis.

• #2 Overview of autoimmune hepatitis – UpToDate

  https://www.uptodate.com/contents/overview-of-autoimmune-hepatitis

  Autoimmune hepatitis can present at any age and in all ethnic groups, but it occurs predominantly in women. For type 1 autoimmune hepatitis, the female to male ratio is 4:1, but for type 2 autoimmune hepatitis, the ratio is 10:1. […] The worldwide incidence of autoimmune hepatitis ranges from 0.7 (southern Israel) to 2 (Canterbury, New Zealand) per 100,000 population, while the prevalence ranges from 4 (Singapore) to 25 (Canterbury, New Zealand) per 100,000. In studies from Europe, the incidence is 0.9 to 2 per 100,000 population per year, with a prevalence of 11 to 25 per 100,000 population. No prevalence data on autoimmune hepatitis exists for the United States.

• #3 Epidemiology of Autoimmune Hepatitis (AIH) in the United States between 2014 and 2019: A Population Based National Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8050120/

  Autoimmune Hepatitis (AIH) is a chronic, inflammatory disease of the liver with increasing prevalence. However, limited epidemiological data exists for prevalence of AIH in the United States. We used a large database to describe the prevalence of AIH in the USA and the autoimmune diseases associated with it. […] We found that the estimated prevalence of AIH in the USA is 31.2/100,000, which is comparable to the reported prevalence of AIH in Europe. […] The prevalence of AIH was increased in females compared to males [OR 3.21, p0.0001], elderly (aged 65years) compared to adults (aged 1865years) and children (aged 18years) [OR 2.51, p0.0001] and Caucasians compared to African- Americans, Asians and Hispanics [OR 1.12, p0.0001]. […] Given the increasing disease burden, we sought to describe the prevalence of AIH in the United States and among various age, gender and race based subgroups utilizing a large population-based database.

• #4

  https://journals.lww.com/jcge/fulltext/2021/11000/epidemiology_of_autoimmune_hepatitis__aih__in_the.15.aspx

  Autoimmune hepatitis (AIH) is a chronic, inflammatory disease of the liver with increasing prevalence. However, limited epidemiological data exist for the prevalence of AIH in the United States. We used a large database to describe the prevalence of AIH in the United States and the autoimmune diseases associated with it. […] Of the 37,161,280 individuals active in the database from April 2014 to 2019, we identified 11,600 individuals with a diagnosis of AIH with an overall prevalence rate of 31.2/100,000. The prevalence of AIH was increased in females compared with males [odds ratio (OR)=3.21, P0.0001], elderly (aged above 65y) compared with adults (aged 18 to 65y) and children (aged below 18y) (OR=2.51, P0.0001) and whites compared with African Americans, Asians, and Hispanics (OR=1.12, P0.0001). […] We found that the estimated prevalence of AIH in the United States is 31.2/100,000, which is comparable to the reported prevalence of AIH in Europe. We confirmed that AIH has a strong association with other autoimmune diseases studied in the literature.

• #5 Epidemiology of Autoimmune Hepatitis (AIH) in the United States between 2014 and 2019: A Population Based National Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8050120/

  Autoimmune Hepatitis (AIH) is a chronic, inflammatory disease of the liver with increasing prevalence. However, limited epidemiological data exists for prevalence of AIH in the United States. We used a large database to describe the prevalence of AIH in the USA and the autoimmune diseases associated with it. […] We found that the estimated prevalence of AIH in the USA is 31.2/100,000, which is comparable to the reported prevalence of AIH in Europe. […] The prevalence of AIH was increased in females compared to males [OR 3.21, p0.0001], elderly (aged 65years) compared to adults (aged 1865years) and children (aged 18years) [OR 2.51, p0.0001] and Caucasians compared to African- Americans, Asians and Hispanics [OR 1.12, p0.0001]. […] Given the increasing disease burden, we sought to describe the prevalence of AIH in the United States and among various age, gender and race based subgroups utilizing a large population-based database.

• #6 Autoimmune Hepatitis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/172356-overview

  The prevalence of autoimmune hepatitis is estimated at 31.2 cases per 100,000 persons in the United States. This is comparable to prevalence rates that are reported in Europe. The prevalence of autoimmune hepatitis is as high as 43 cases per 100,000 persons in a native Alaskan population. Autoimmune hepatitis is more common in Whites, compared to Blacks, Asians, and Hispanics. […] Autoimmune hepatitis is more prevalent in women than men, although men may be affected more commonly than women in older age groups. Amongst adults, 71-95% of patients are women. Amongst children, 60-76% of patients are girls. In patients with type 2 autoimmune hepatitis, more than 90% of patients are female. […] Classically, the new onset of autoimmune hepatitis was most commonly reported in the 10-30 and 40-60 age ranges. A study from New Zealand, however, reported that 72% of patients presented after age 40, with a peak age of presentation in the sixth decade of life. Autoimmune hepatitis may occur in people of any age, including infants and older adults. The diagnosis should not be overlooked in individuals older than 70 years.

• #7

  https://journals.lww.com/jcge/fulltext/2021/11000/epidemiology_of_autoimmune_hepatitis__aih__in_the.15.aspx

  Autoimmune hepatitis (AIH) is a chronic, inflammatory disease of the liver with increasing prevalence. However, limited epidemiological data exist for the prevalence of AIH in the United States. We used a large database to describe the prevalence of AIH in the United States and the autoimmune diseases associated with it. […] Of the 37,161,280 individuals active in the database from April 2014 to 2019, we identified 11,600 individuals with a diagnosis of AIH with an overall prevalence rate of 31.2/100,000. The prevalence of AIH was increased in females compared with males [odds ratio (OR)=3.21, P0.0001], elderly (aged above 65y) compared with adults (aged 18 to 65y) and children (aged below 18y) (OR=2.51, P0.0001) and whites compared with African Americans, Asians, and Hispanics (OR=1.12, P0.0001). […] We found that the estimated prevalence of AIH in the United States is 31.2/100,000, which is comparable to the reported prevalence of AIH in Europe. We confirmed that AIH has a strong association with other autoimmune diseases studied in the literature.

• #8 Overview of autoimmune hepatitis – UpToDate

  https://www.uptodate.com/contents/overview-of-autoimmune-hepatitis

  Autoimmune hepatitis can present at any age and in all ethnic groups, but it occurs predominantly in women. For type 1 autoimmune hepatitis, the female to male ratio is 4:1, but for type 2 autoimmune hepatitis, the ratio is 10:1. […] The worldwide incidence of autoimmune hepatitis ranges from 0.7 (southern Israel) to 2 (Canterbury, New Zealand) per 100,000 population, while the prevalence ranges from 4 (Singapore) to 25 (Canterbury, New Zealand) per 100,000. In studies from Europe, the incidence is 0.9 to 2 per 100,000 population per year, with a prevalence of 11 to 25 per 100,000 population. No prevalence data on autoimmune hepatitis exists for the United States.

• #9 Recent advances in clinical practice: epidemiology of autoimmune liver diseases | Gut

  https://gut.bmj.com/content/70/10/1989.abstract

  Autoimmune liver diseases are chronic inflammatory hepatobiliary disorders that when classically defined encompass three distinctive clinical presentations; primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH). […] Meaningful changes in disease epidemiology are reported, with increasing incidence and prevalence of AIH and PSC in Europe, and rising prevalence of PBC across Europe, North America and the Asia-Pacific region. […] However, there appears to be very significant global variation with contemporary incidence rates of disease per 100 000 ranging from 0.84 to 2.75 for PBC, 0.1 to 4.39 for PSC and 0.4 to 2.39 for AIH. […] Prevalence corresponds, and per 100 000 estimates for PBC range from 1.91 to 40.2, for PSC between 0.78 and 31.7 and for AIH from 4.8 to 42.9.

• #10 Autoimmune Hepatitis| Cleveland Clinic

  https://my.clevelandclinic.org/departments/digestive/medical-professionals/hepatology/autoimmune-hepatitis

  Autoimmune hepatitis occurs worldwide but the exact incidence and prevalence of the disease in the United States is unknown. The point prevalence and incidence of AIH in Northern Europeans is approximately 18 per 100,000 people per year and 1.1 per 100,000 people per year, respectively, and it is assumed that this data can be extrapolated to the North American population. […] Interestingly the prevalence of AIH in Native Alaskan population is much higher, with a point prevalence of 42 per 100,000 people/year. Autoimmune hepatitis has a female predominance and a bimodal age distribution with 2 peaks, 1 in childhood and another in the 5th decade. However AIH occurs in both genders and in all age groups and there have been reports of newly diagnosed AIH in patients 80 years of age.

• #11 Autoimmune Hepatitis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/172356-overview

  The prevalence of autoimmune hepatitis is estimated at 31.2 cases per 100,000 persons in the United States. This is comparable to prevalence rates that are reported in Europe. The prevalence of autoimmune hepatitis is as high as 43 cases per 100,000 persons in a native Alaskan population. Autoimmune hepatitis is more common in Whites, compared to Blacks, Asians, and Hispanics. […] Autoimmune hepatitis is more prevalent in women than men, although men may be affected more commonly than women in older age groups. Amongst adults, 71-95% of patients are women. Amongst children, 60-76% of patients are girls. In patients with type 2 autoimmune hepatitis, more than 90% of patients are female. […] Classically, the new onset of autoimmune hepatitis was most commonly reported in the 10-30 and 40-60 age ranges. A study from New Zealand, however, reported that 72% of patients presented after age 40, with a peak age of presentation in the sixth decade of life. Autoimmune hepatitis may occur in people of any age, including infants and older adults. The diagnosis should not be overlooked in individuals older than 70 years.

• #12 Autoimmune Hepatitis Management: Recent Advances and Future Prospects

  https://www.mdpi.com/2673-4389/4/2/17

  Autoimmune hepatitis (AIH) is a varied inflammatory chronic liver disease. AIH’s prevalence varies and has increased recently. […] Recent times have witnessed notable fluctuations in AIH incidence and prevalence, ranging from 0.4 to 2.39 per 100,000 for incidence and 4.8 to 42.9 per 100,000 for prevalence. […] The primary treatment approach remains corticoid induction therapy, followed by maintenance with a combination of steroids and azathioprine. […] The ultimate therapeutic goal is achieving biochemical remission, characterized by normal liver transaminases and immunoglobin G levels. […] This review aims to provide the latest updates on the proposed disease pathogenesis, ongoing research on diagnostic biomarkers, and the current and investigational treatment options available for AIH.

• #13 Overview of autoimmune hepatitis – UpToDate

  https://www.uptodate.com/contents/overview-of-autoimmune-hepatitis

  Autoimmune hepatitis can present at any age and in all ethnic groups, but it occurs predominantly in women. For type 1 autoimmune hepatitis, the female to male ratio is 4:1, but for type 2 autoimmune hepatitis, the ratio is 10:1. […] The worldwide incidence of autoimmune hepatitis ranges from 0.7 (southern Israel) to 2 (Canterbury, New Zealand) per 100,000 population, while the prevalence ranges from 4 (Singapore) to 25 (Canterbury, New Zealand) per 100,000. In studies from Europe, the incidence is 0.9 to 2 per 100,000 population per year, with a prevalence of 11 to 25 per 100,000 population. No prevalence data on autoimmune hepatitis exists for the United States.

• #14 Autoimmune hepatitis epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Autoimmune_hepatitis_epidemiology_and_demographics

  The incidence and prevalence of autoimmune hepatitis are generally higher among individuals of the whites of northern European ancestry with a high frequency of HLA-DR3 and HLA-DR4 markers. The incidence of autoimmune hepatitis approximately 1 to 2 per 100,000 individuals worldwide. […] The incidence of autoimmune hepatitis is estimated to be 0.9 to 2 cases per 100,000 population annually in Europe. […] The prevalence of autoimmune hepatitis is approximately 11 to 25 per 100,000 individuals worldwide. […] In Europe, the prevalence of autoimmune hepatitis ranges from 10 to 17 per 100000. […] In Japan, the prevalence of autoimmune hepatitis ranges from 0.08-0.015 cases per 100,000 persons. […] Autoimmune hepatitis has bimodal distribution usually presents around puberty and between 4th and 6th decade, although a significant proportion older people is usually above 65 years of age.

• #15

  https://journals.lww.com/ajg/fulltext/2017/10001/epidemiology,_clinical_characteristics_and.914.aspx

  Autoimmune hepatitis (AIH) is a chronic progressive immune-mediated liver disease whose incidence varies widely in different countries and regions within the same country. The incidence of AIH in the adult population of the Unites States is unknown, and the ability to evaluate population-based causative and prognostic factors and properly allocate medical resources has been compromised. Our goal was to determine the incidence, clinical presentations, and outcomes of AIH in Olmsted County, Minnesota over the past 25 years. […] Seventy patients were diagnosed with AIH from 1990 to 2015. The overall incidence was 2.88 per 100,000 (95% CI, 2.21 to 3.56) persons per year. The age-adjusted incidence of AIH (per 100,000) was 4.22 (95% CI, 3.08 to 5.35) in women and 1.46 (95% CI, 0.76 to 2.16) in men.

• #16

  https://journals.lww.com/ajg/fulltext/2017/10001/epidemiology,_clinical_characteristics_and.914.aspx

  In this first population-based study of adult AIH in the United States, the incidence of AIH in Olmsted County was higher than in all similar studies in Europe, Israel, and New Zealand. These findings should prompt regional comparisons to identify differences in genetic predispositions, environmental factors, and intestinal microbiome.

• #17 Overview of autoimmune hepatitis – UpToDate

  https://www.uptodate.com/contents/overview-of-autoimmune-hepatitis

  Autoimmune hepatitis can present at any age and in all ethnic groups, but it occurs predominantly in women. For type 1 autoimmune hepatitis, the female to male ratio is 4:1, but for type 2 autoimmune hepatitis, the ratio is 10:1. […] The worldwide incidence of autoimmune hepatitis ranges from 0.7 (southern Israel) to 2 (Canterbury, New Zealand) per 100,000 population, while the prevalence ranges from 4 (Singapore) to 25 (Canterbury, New Zealand) per 100,000. In studies from Europe, the incidence is 0.9 to 2 per 100,000 population per year, with a prevalence of 11 to 25 per 100,000 population. No prevalence data on autoimmune hepatitis exists for the United States.

• #18 Autoimmune hepatitis epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Autoimmune_hepatitis_epidemiology_and_demographics

  Autoimmune hepatitis usually affects individuals of the whites of northern European ancestry with a high frequency of HLA-DR3 and HLA-DR4 markers. […] Young women are more commonly affected by autoimmune hepatitis than men. […] The women to men ratio is approximately 3:1. […] Autoimmune hepatitis is more common in North American First Nations populations compared with predominantly Caucasian, Non-first Nations populations.

• #19 Epidemiology of Autoimmune Hepatitis (AIH) in the United States between 2014 and 2019: A Population Based National Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8050120/

  The overall prevalence of AIH was estimated as 31.2/100,000. […] The prevalence of AIH was higher in females at 45.0/100,000 vs. males at 13.9/100,000 [OR 3.21, 95% CI 3.07 to 3.36, p0.0001]. […] Based on age, AIH prevalence was highest in the 7th decade of life after which there was a decline in prevalence rates. […] Based on ethnicity, the AIH prevalence in Caucasians was 38.7/100,000 vs. African Americans at 33.1/100,000, Asians at 38.8/100,000 and Hispanics at 41.5/100,000 [OR 1.12, 95% CI 1.06 to 1.17, p0.0001]. […] We estimated the 5-year prevalence rate of AIH between 2014 and 2019 to be 31.2/100,000. […] Our study showed comparable results with AIH prevalence of 45.0 per 100,000 in females and 14.0 per 100,000 in males. […] Our study determined the prevalence in contrast to incidence and demonstrated the highest prevalence in elderly (65 years) population and an increase in AIH prevalence rates until the seventh decade of life.

• #20 Autoimmune Hepatitis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/172356-overview

  The prevalence of autoimmune hepatitis is estimated at 31.2 cases per 100,000 persons in the United States. This is comparable to prevalence rates that are reported in Europe. The prevalence of autoimmune hepatitis is as high as 43 cases per 100,000 persons in a native Alaskan population. Autoimmune hepatitis is more common in Whites, compared to Blacks, Asians, and Hispanics. […] Autoimmune hepatitis is more prevalent in women than men, although men may be affected more commonly than women in older age groups. Amongst adults, 71-95% of patients are women. Amongst children, 60-76% of patients are girls. In patients with type 2 autoimmune hepatitis, more than 90% of patients are female. […] Classically, the new onset of autoimmune hepatitis was most commonly reported in the 10-30 and 40-60 age ranges. A study from New Zealand, however, reported that 72% of patients presented after age 40, with a peak age of presentation in the sixth decade of life. Autoimmune hepatitis may occur in people of any age, including infants and older adults. The diagnosis should not be overlooked in individuals older than 70 years.

• #21 Autoimmune Hepatitis: 2019 Update

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl19261

  The male-to-female ratio of the population with AIH is considered to have changed over time, indicating a relative increase in the number of male patients. In Japan, the male-to-female ratio was 1:7 in 2004 and 1:4 in 2016. […] The point prevalence recently reported is similar to that of the rest of the world.

• #22 Changing epidemiology and treatment responses in autoimmune liver diseases: a 14-year retrospective analysis from a tertiary care center | Egyptian Liver Journal | Full Text

  https://eglj.springeropen.com/articles/10.1186/s43066-024-00395-8

  The male-to-female ratio increased from 1:7.3 to 1:2.6 for AIH, 1:15 to 1:3 for PBC, and 1:7 to 1:2.3 for overlap syndrome. […] The overall male-to-female ratio increased from 1:6.28 to 1:2.45 indicating a trend of increasing male diagnoses over the years. […] The presence of extrahepatic autoimmune diseases was observed in 40.8% of cases, with the highest occurrence rate of 46.4% noted in primary biliary cholangitis (PBC). […] Despite high treatment success rates, relapse rates in patients with AIH continue to pose a significant concern. […] An important finding of our study is the statistically significant association between the presence of portal plasma cell infiltration on liver biopsy at the time of diagnosis and relapse.

• #23 Autoimmune hepatitis – Wikipedia

  https://en.wikipedia.org/wiki/Autoimmune_hepatitis

  Autoimmune hepatitis can develop in people of any race or age but occurs most frequently in women. Eighty percent of cases are the type 1 subtype with women being affected 4 times more often than men; for the type 2 subtype, women are affected 10 times more often than men. […] European studies suggest a disease incidence of 1 to 2 people affected per 100,000 population with a prevalence of 10 to 25 people per 100,000 population. […] The disease has a bimodal peak occurring between the ages of 10 and 20 and then later in life between the ages of 40 and 50.

• #24 Autoimmune hepatitis epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Autoimmune_hepatitis_epidemiology_and_demographics

  The incidence and prevalence of autoimmune hepatitis are generally higher among individuals of the whites of northern European ancestry with a high frequency of HLA-DR3 and HLA-DR4 markers. The incidence of autoimmune hepatitis approximately 1 to 2 per 100,000 individuals worldwide. […] The incidence of autoimmune hepatitis is estimated to be 0.9 to 2 cases per 100,000 population annually in Europe. […] The prevalence of autoimmune hepatitis is approximately 11 to 25 per 100,000 individuals worldwide. […] In Europe, the prevalence of autoimmune hepatitis ranges from 10 to 17 per 100000. […] In Japan, the prevalence of autoimmune hepatitis ranges from 0.08-0.015 cases per 100,000 persons. […] Autoimmune hepatitis has bimodal distribution usually presents around puberty and between 4th and 6th decade, although a significant proportion older people is usually above 65 years of age.

• #25 Autoimmune hepatitis – Wikipedia

  https://en.wikipedia.org/wiki/Autoimmune_hepatitis

  Autoimmune hepatitis can develop in people of any race or age but occurs most frequently in women. Eighty percent of cases are the type 1 subtype with women being affected 4 times more often than men; for the type 2 subtype, women are affected 10 times more often than men. […] European studies suggest a disease incidence of 1 to 2 people affected per 100,000 population with a prevalence of 10 to 25 people per 100,000 population. […] The disease has a bimodal peak occurring between the ages of 10 and 20 and then later in life between the ages of 40 and 50.

• #26 Epidemiology of Autoimmune Hepatitis (AIH) in the United States between 2014 and 2019: A Population Based National Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8050120/

  The overall prevalence of AIH was estimated as 31.2/100,000. […] The prevalence of AIH was higher in females at 45.0/100,000 vs. males at 13.9/100,000 [OR 3.21, 95% CI 3.07 to 3.36, p0.0001]. […] Based on age, AIH prevalence was highest in the 7th decade of life after which there was a decline in prevalence rates. […] Based on ethnicity, the AIH prevalence in Caucasians was 38.7/100,000 vs. African Americans at 33.1/100,000, Asians at 38.8/100,000 and Hispanics at 41.5/100,000 [OR 1.12, 95% CI 1.06 to 1.17, p0.0001]. […] We estimated the 5-year prevalence rate of AIH between 2014 and 2019 to be 31.2/100,000. […] Our study showed comparable results with AIH prevalence of 45.0 per 100,000 in females and 14.0 per 100,000 in males. […] Our study determined the prevalence in contrast to incidence and demonstrated the highest prevalence in elderly (65 years) population and an increase in AIH prevalence rates until the seventh decade of life.

• #27 Population-based epidemiology study of autoimmune hepatitis: A disease of older women? – University of Otago

  https://ourarchive.otago.ac.nz/esploro/outputs/journalArticle/Population-based-epidemiology-study-of-autoimmune-hepatitis/9926517687701891

  Background and Aim: The etiology of autoimmune hepatitis (AIH) is unknown, and limited epidemiological data are available. Our aim was to perform a population based epidemiological study of AIH in Canterbury, New Zealand. […] When the original revised criteria were used, 138 cases (123 definite and 14 probable AIH), were identified. Prospective incidence in 2008 was 2.0/100 000 (95% confidence interval [CI] 0.8-3.3/100 000). Point prevalence on 31 December 2008 was 24.5/100 000 (95% CI 20.1-28.9). Age-standardized (World Health Organization standard population) incidence and prevalence were 1.7 and 18.9 per 100 000, respectively. Gender-specific prevalence confirmed a female predominance, while ethnicity-specific prevalence showed higher prevalence in Caucasians. 72% of cases presented after 40 years of age and the peak age of presentation was in the sixth decade of life.

• #28 Epidemiology of Autoimmune Hepatitis (AIH) in the United States between 2014 and 2019: A Population Based National Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8050120/

  Autoimmune Hepatitis (AIH) is a chronic, inflammatory disease of the liver with increasing prevalence. However, limited epidemiological data exists for prevalence of AIH in the United States. We used a large database to describe the prevalence of AIH in the USA and the autoimmune diseases associated with it. […] We found that the estimated prevalence of AIH in the USA is 31.2/100,000, which is comparable to the reported prevalence of AIH in Europe. […] The prevalence of AIH was increased in females compared to males [OR 3.21, p0.0001], elderly (aged 65years) compared to adults (aged 1865years) and children (aged 18years) [OR 2.51, p0.0001] and Caucasians compared to African- Americans, Asians and Hispanics [OR 1.12, p0.0001]. […] Given the increasing disease burden, we sought to describe the prevalence of AIH in the United States and among various age, gender and race based subgroups utilizing a large population-based database.

• #29 Autoimmmune hepatitis | Cellular & Molecular Immunology

  https://www.nature.com/articles/s41423-021-00768-8

  AIH conforms to the definition of a rare disease, affecting less than 200,000 individuals in the US and less than 1 in 2000 inhabitants in the EU. It occurs worldwide and in all ethnicities, but the vast majority of the epidemiological studies stem from Western countries, and, more recently, from Asia. […] Early epidemiological studies, carried out before the publication of diagnostic criteria, report an incidence ranging from 0.1 to 1.9 cases/100,000 in European countries and Japan. More recent studies from Europe report higher disease frequency, with an incidence ranging from 1.1 to 2.56 and a prevalence ranging from 17.3 to 18.3/100,000 inhabitants. Studies in more recent years report an even higher incidence. […] A large primary care population-based study from the UK published recently reported a yearly incidence of AIH of 1.94/100,000 inhabitants from 2002 to 2016: the authors describe a higher incidence with higher latitude; in contrast to previous studies, this report did not find an increasing incidence in more recent years. The median age at disease onset in this study, which included only adults, increased from 2002 to 2015 from 52 to 58 years.

• #30 Autoimmune Hepatitis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/172356-overview

  The prevalence of autoimmune hepatitis is estimated at 31.2 cases per 100,000 persons in the United States. This is comparable to prevalence rates that are reported in Europe. The prevalence of autoimmune hepatitis is as high as 43 cases per 100,000 persons in a native Alaskan population. Autoimmune hepatitis is more common in Whites, compared to Blacks, Asians, and Hispanics. […] Autoimmune hepatitis is more prevalent in women than men, although men may be affected more commonly than women in older age groups. Amongst adults, 71-95% of patients are women. Amongst children, 60-76% of patients are girls. In patients with type 2 autoimmune hepatitis, more than 90% of patients are female. […] Classically, the new onset of autoimmune hepatitis was most commonly reported in the 10-30 and 40-60 age ranges. A study from New Zealand, however, reported that 72% of patients presented after age 40, with a peak age of presentation in the sixth decade of life. Autoimmune hepatitis may occur in people of any age, including infants and older adults. The diagnosis should not be overlooked in individuals older than 70 years.

• #31 Overview of autoimmune hepatitis – UpToDate

  https://www.uptodate.com/contents/overview-of-autoimmune-hepatitis

  Autoimmune hepatitis can present at any age and in all ethnic groups, but it occurs predominantly in women. For type 1 autoimmune hepatitis, the female to male ratio is 4:1, but for type 2 autoimmune hepatitis, the ratio is 10:1. […] The worldwide incidence of autoimmune hepatitis ranges from 0.7 (southern Israel) to 2 (Canterbury, New Zealand) per 100,000 population, while the prevalence ranges from 4 (Singapore) to 25 (Canterbury, New Zealand) per 100,000. In studies from Europe, the incidence is 0.9 to 2 per 100,000 population per year, with a prevalence of 11 to 25 per 100,000 population. No prevalence data on autoimmune hepatitis exists for the United States.

• #32 Epidemiology of Autoimmune Hepatitis (AIH) in the United States between 2014 and 2019: A Population Based National Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8050120/

  The overall prevalence of AIH was estimated as 31.2/100,000. […] The prevalence of AIH was higher in females at 45.0/100,000 vs. males at 13.9/100,000 [OR 3.21, 95% CI 3.07 to 3.36, p0.0001]. […] Based on age, AIH prevalence was highest in the 7th decade of life after which there was a decline in prevalence rates. […] Based on ethnicity, the AIH prevalence in Caucasians was 38.7/100,000 vs. African Americans at 33.1/100,000, Asians at 38.8/100,000 and Hispanics at 41.5/100,000 [OR 1.12, 95% CI 1.06 to 1.17, p0.0001]. […] We estimated the 5-year prevalence rate of AIH between 2014 and 2019 to be 31.2/100,000. […] Our study showed comparable results with AIH prevalence of 45.0 per 100,000 in females and 14.0 per 100,000 in males. […] Our study determined the prevalence in contrast to incidence and demonstrated the highest prevalence in elderly (65 years) population and an increase in AIH prevalence rates until the seventh decade of life.

• #33 Autoimmune Hepatitis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/172356-overview

  The prevalence of autoimmune hepatitis is estimated at 31.2 cases per 100,000 persons in the United States. This is comparable to prevalence rates that are reported in Europe. The prevalence of autoimmune hepatitis is as high as 43 cases per 100,000 persons in a native Alaskan population. Autoimmune hepatitis is more common in Whites, compared to Blacks, Asians, and Hispanics. […] Autoimmune hepatitis is more prevalent in women than men, although men may be affected more commonly than women in older age groups. Amongst adults, 71-95% of patients are women. Amongst children, 60-76% of patients are girls. In patients with type 2 autoimmune hepatitis, more than 90% of patients are female. […] Classically, the new onset of autoimmune hepatitis was most commonly reported in the 10-30 and 40-60 age ranges. A study from New Zealand, however, reported that 72% of patients presented after age 40, with a peak age of presentation in the sixth decade of life. Autoimmune hepatitis may occur in people of any age, including infants and older adults. The diagnosis should not be overlooked in individuals older than 70 years.

• #34 Autoimmune hepatitis epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Autoimmune_hepatitis_epidemiology_and_demographics

  Autoimmune hepatitis usually affects individuals of the whites of northern European ancestry with a high frequency of HLA-DR3 and HLA-DR4 markers. […] Young women are more commonly affected by autoimmune hepatitis than men. […] The women to men ratio is approximately 3:1. […] Autoimmune hepatitis is more common in North American First Nations populations compared with predominantly Caucasian, Non-first Nations populations.

• #35 Autoimmune hepatitis epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Autoimmune_hepatitis_epidemiology_and_demographics

  Autoimmune hepatitis usually affects individuals of the whites of northern European ancestry with a high frequency of HLA-DR3 and HLA-DR4 markers. […] Young women are more commonly affected by autoimmune hepatitis than men. […] The women to men ratio is approximately 3:1. […] Autoimmune hepatitis is more common in North American First Nations populations compared with predominantly Caucasian, Non-first Nations populations.

• #36 The epidemiology of autoimmune liver disease varies with geographic latitude | medRxiv

  https://www.medrxiv.org/content/10.1101/2020.06.27.20141382.full

  The epidemiology of autoimmune liver disease (AILD) is challenging to study because of the diseases rarity and because of cohort selection bias. Increased incidence further from the Equator is reported for multiple sclerosis, another autoimmune. We assessed the incidence of primary biliary cholangitis (PBC), primary biliary cholangitis (PSC), and autoimmune hepatitis (AIH) in relation to latitude. […] There were 1314 incident cases of PBC, 396 of PSC, and 1034 of AIH. Crude incidences (95% confidence interval) was: PBC 2.47 (2.34-2.60), PSC 0.74 (0.67-0.82), and AIH 1.94 (1.83-2.06)/100 000/year. PBC incidence correlated with female sex, smoking, and deprivation; PSC incidence correlated with male sex and not smoking; AIH incidence correlated with female sex and deprivation. […] More northerly latitude was strongly associated with incidence of PBC: 2.16 (1.79-2.60) to 4.86 (3.93-6.00) from 50-57N (p=0.002) and AIH 2.00 (1.65-2.43) to 3.28 (2.53-4.24)(p=0.003), but not PSC 0.82 (0.60-1.11) to 1.02 (0.64-1.61)(p=0.473).

• #37 The epidemiology of autoimmune liver disease varies with geographic latitude | medRxiv

  https://www.medrxiv.org/content/10.1101/2020.06.27.20141382.full

  For AILDs, the crude incidence of PBC was markedly greater at more northerly latitudes. After adjustment for sex, age, smoking status, and Townsend deprivation quintile there remained a more than doubling in incidence from the 1.96 (1.57-2.34)/100 000/year in the 50 latitude band to 4.55 (3.52-5.58) in the 57 latitude band. […] In 2015, after adjustment for age, sex, smoking status and Townsend quintile, there was a significant increase in the prevalence of both PBC and AIH at more northerly latitudes; such a gradient was not apparent for PSC. […] Here we demonstrate for the first time a striking correlation between increased geographic latitude and disease risk for PBC; the same phenomenon is present to a lesser extent for AIH but absent for PSC.

• #38 The epidemiology of autoimmune liver disease varies with geographic latitude | medRxiv

  https://www.medrxiv.org/content/10.1101/2020.06.27.20141382.full

  Incidence after adjustment for age, sex, smoking, and deprivation status showed similar positive correlations for PBC and AIH with latitude, but not PSC. […] We describe a novel association between increased latitude and the incidence of PBC and AIH that requires both confirmation and explanation. […] In this study we investigate the relationship between latitude and incidence of AILD in a large UK primary care database with established generalisability to the national population. […] Overall, a total of 8 590 421 pseudonymised patient records with a total of approximately 53.3 million years follow-up from 694 practices were examined. First entry to follow-up was 2002-01-01 and the last data collection point 2016-05-10. Median follow-up was 5.2 years (IQR 1.9-10.2). 1314 incident cases of PBC, 396 incident cases of PSC, and 1034 incident cases of AIH were identified.

• #39 Autoimmune Hepatitis: 2019 Update

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl19261

  Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease, characterized by the elevation of aminotransferases, presence of anti-nuclear antibody or anti-smooth muscle antibody, elevated immunoglobulin G (IgG), and interface hepatitis/plasma-lymphocytic inflammation based on histology. Recent epidemiological studies have indicated an increasing trend in the prevalence of AIH worldwide, especially in male patients; this trend may suggest the alteration of environmental triggers of disease onset over time. […] The burden of AIH appears to be increasing worldwide, and the point prevalence reported in the published literature after 2000 is shown in Fig. 1. The point prevalence of AIH is reported to be 10 to 25 per 100,000 population in European countries, and 5 to 25 in the Asia-Pacific region.

• #40 Epidemiology of Autoimmune Liver Disease

  https://www.kjg.or.kr/journal/view.html?doi=10.4166/kjg.2023.007

  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. […] 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.

• #41 Autoimmmune hepatitis | Cellular & Molecular Immunology

  https://www.nature.com/articles/s41423-021-00768-8

  A recent study from the US, based on a commercial database including some 37,000,000 patients of all ages from 2014 to 2019, reported an AIH prevalence as high as 31.2/100,000. […] A population-based study from New Zealand published in 2021 reported an overall incidence of 1.93/100,000 from 2008 to 2016, with a significant increase of the incidence from the first 3 years of the observation period to the last three years (1.37-2.39). This is in line with recent European studies, and probably reflects a true increase in the disease frequency, as observed for other autoimmune diseases. […] AIH has long being considered to be rarer in Asia, although a recent systematic review of the literature published until April 2019 reported a similar yearly incidence per 100,000 inhabitants in Asia (1.31), Europe (1.37) and in America (1.00). The worldwide AIH prevalence in this paper was 17.44/100,000 inhabitants, being lower in Asia as compared to Europe and America. This finding may reflect a more recent awareness of AIH in Asia.

• #42 Incidence of autoimmune hepatitis may be rising | MDedge Internal Medicine

  https://www.mdedge9-ma1.mdedge.com/internalmedicine/article/235224/gastroenterology/incidence-autoimmune-hepatitis-may-be-rising

  The incidence of autoimmune hepatitis (AIH) may be rising, according to a prospective population-based study conducted in New Zealand. […] From 2008 to 2016, the rising incidence of AIH led to a 40% increase in point prevalence, reported lead author Mehul Lamba, MD, of Christchurch (New Zealand) Hospital and colleagues. […] The epidemiological trends of these autoimmune liver diseases therefore remain incompletely understood, wrote Dr. Lamba and colleagues. […] Incidence of AIH, PBC, and PSC was assessed at three time points: 2008-2010, 2011-2013, and 2014-2016. AIH had the highest overall incidence, at 1.93 cases per 100,000 people, followed by PSC (0.92) and PBC (0.51). […] While the rates of PBC and PSC did not change significantly over time, the incidence of AIH rose from 1.37 cases per 100,000 people in the period from 2008-2010 to 2.39 per 100,000 in 2014-2016 (P = .04), which computes to an incidence rate ratio of 1.69 (95% confidence interval, 1.02-2.84).

• #43 Epidemiology of autoimmune liver disease in Korea: evidence from a nationwide real-world database | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-024-03086-0

  The number of patients with autoimmune liver disease in South Korea is increasing over time. Further research on autoimmune liver disease is needed to fulfill unmet clinical needs. […] Diagnosis of AILD is difficult as its incidence is only 0.002% per year accounting for only 5% of liver diseases. […] The 10-year survival rates of AILD are 5090%, requiring regular hospital visits, lowering the quality of life, and generating psychological stress. […] The prevalence of AIH, PBC, and PSC were 18.4, 11.8, and 1.5 per 100,000 population, while the corresponding incidence was 2.3, 1.4, and 0.3 per 100,000 population, respectively, in 2019. […] Our results show that the number of newly diagnosed AILD patients in Korea has been increasing since 2010, as has the sex-age-standardized incidence of AIH and PBC. The total number of patients newly diagnosed with AIH was 507 in 2010 and 1,169 in 2019. In the case of PBC, the numbers were 271 and 724 for the respective years. […] The prevalence of AILD has also increased rapidly over time. The cumulative number of AILD patients has been rising.

• #44 Epidemiology of autoimmune liver disease in Korea: evidence from a nationwide real-world database | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-024-03086-0

  Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are all immune-mediated chronic inflammatory liver diseases. Autoimmune liver diseases are rare, making identification and treatment difficult. To improve clinical outcomes and enhance patient quality of life, we performed an epidemiological study of autoimmune liver diseases based on real-world comprehensive data. […] We used National Health Insurance Service claims data in Korea from 2005 to 2019. Patients were identified using the International Classification of Disease 10th Revision code, and rare intractable disease codes assigned according to the strict diagnostic criteria. […] During the study period, there were 10,212, 6,784, and 888 AIH, PBC, and PSC patients, respectively. The prevalence of AIH, PBC, and PSC in 2019 were 18.4, 11.8, and 1.5 per 100,000 population, while the corresponding incidences were 2.3, 1.4, and 0.3 per 100,000 population, respectively. Analysis of sex-age-standardized data showed that the annual prevalence of these diseases is increasing. The 10-year survival rates were 89.8%, 74.9%, and 73.4% for AIH, PBC, and PSC, respectively.

• #45 Clinical and Molecular Hepatology

  https://e-cmh.org/m/journal/view.php?number=1750

  Aberrant activation of autoimmunity may influence the bad impact on the liver. The global annual incidence rate of AIH was 1.37 per 100,000 persons, and similar among the Asian, European, and American populations. The global prevalence rate of AIH was 17.44 per 100,000 persons with a high level of regional variation. The average age of the onset of AIH is the mid-50s. […] In epidemiology, the prevalence and incidence of these rare autoimmune liver diseases presented an increasing trend. However, it is not definite whether this trend reflects a real increase of the disease or improved identification due to better awareness of the physician.

• #46 Epidemiology and updated management for autoimmune liver disease

  https://www.e-cmh.org/journal/view.php?doi=10.3350/cmh.2022.0387

  Epidemiology and updated management for autoimmune liver disease […] The global annual incidence rate of AIH was 1.37 per 100,000 persons, and similar among the Asian, European, and American populations. The global prevalence rate of AIH was 17.44 per 100,000 persons with a high level of regional variation. The average age of the onset of AIH is the mid-50s. […] In epidemiology, the prevalence and incidence of these rare autoimmune liver diseases presented an increasing trend. However, it is not definite whether this trend reflects a real increase of the disease or improved identification due to better awareness of the physician.

• #47

  https://journals.lww.com/jcge/fulltext/2021/11000/epidemiology_of_autoimmune_hepatitis__aih__in_the.15.aspx

  Autoimmune hepatitis (AIH) is a chronic, inflammatory disease of the liver with increasing prevalence. However, limited epidemiological data exist for the prevalence of AIH in the United States. We used a large database to describe the prevalence of AIH in the United States and the autoimmune diseases associated with it. […] Of the 37,161,280 individuals active in the database from April 2014 to 2019, we identified 11,600 individuals with a diagnosis of AIH with an overall prevalence rate of 31.2/100,000. The prevalence of AIH was increased in females compared with males [odds ratio (OR)=3.21, P0.0001], elderly (aged above 65y) compared with adults (aged 18 to 65y) and children (aged below 18y) (OR=2.51, P0.0001) and whites compared with African Americans, Asians, and Hispanics (OR=1.12, P0.0001). […] We found that the estimated prevalence of AIH in the United States is 31.2/100,000, which is comparable to the reported prevalence of AIH in Europe. We confirmed that AIH has a strong association with other autoimmune diseases studied in the literature.

• #48 Changing epidemiology and treatment responses in autoimmune liver diseases: a 14-year retrospective analysis from a tertiary care center | Egyptian Liver Journal | Full Text

  https://eglj.springeropen.com/articles/10.1186/s43066-024-00395-8

  The male-to-female ratio increased from 1:7.3 to 1:2.6 for AIH, 1:15 to 1:3 for PBC, and 1:7 to 1:2.3 for overlap syndrome. […] The overall male-to-female ratio increased from 1:6.28 to 1:2.45 indicating a trend of increasing male diagnoses over the years. […] The presence of extrahepatic autoimmune diseases was observed in 40.8% of cases, with the highest occurrence rate of 46.4% noted in primary biliary cholangitis (PBC). […] Despite high treatment success rates, relapse rates in patients with AIH continue to pose a significant concern. […] An important finding of our study is the statistically significant association between the presence of portal plasma cell infiltration on liver biopsy at the time of diagnosis and relapse.

• #49 Autoimmune Hepatitis – almostadoctor

  https://almostadoctor.co.uk/encyclopedia/autoimmune-hepatitis

  Autoimmune hepatitis is rare. In Western Europe and the US there are 1 case per 100 000. The M:F ratio is 1:3. Peaks of incidence: Age 10-20, and 45-70. The lower peak is more common and: Accounts for about half of cases, Typically premenstrual girls, 80% of AIH-2 patients are children. Other autoimmune disease in the patient, or first degree relatives: 20% of patients have another autoimmune disease, 40% of patients have a first degree relative with an autoimmune disease.

• #50

  https://samajournals.co.za/index.php/samj/article/view/1646

  Autoimmune hepatitis (AIH) has scarcely been reported on in patients of black African descent. […] We aim to describe the presenting features of AIH from a single referral centre in a Sub-Sahara African setting. […] This study was a retrospective chart review. […] Forty cases were included, of which 33 (82.5%) were female and 33 (82.5%) were black African. […] A diagnosis of a coexistent autoimmune disease was made in 22.5% of patients, with Systemic Lupus Erythematosus (SLE) being the most common (12.5%). […] Sixteen patients were HIV-infected, all of whom were female (p =0.03), with a significantly older age of disease onset as compared to their HIV-uninfected counterparts (median age 38 vs 17.5 years, p 0.001). […] AIH is a disease most commonly affecting young females. Female sex and older age of onset is associated with AIH in HIV-infected individuals.

• #51

  https://smw.ch/index.php/smw/article/view/3474?articlesBySameAuthorPage=3

  BACKGROUND AND AIMS: The Swiss Autoimmune Hepatitis Cohort Study is a nationwide registry, initiated in 2017, that collects retrospective and prospective clinical data and biological samples from patients of all ages with autoimmune hepatitis treated at Swiss hepatology centres. Here, we report the analysis of the first 5 years of registry data. […] A total of 291 patients with autoimmune hepatitis have been enrolled, 30 of whom were diagnosed before 18 years of age and composed the paediatric cohort. […] Adult cohort: median age at diagnosis 54 years (range 4264, IQR 1881), 185 (71%) women, 51 (20%) with primary biliary cholangitis variant syndrome, 22 (8%) with primary sclerosing cholangitis variant syndrome, 9 (4%) with inflammatory bowel disease and 66 (32%) with advanced liver fibrosis at diagnosis.

• #52

  https://smw.ch/index.php/smw/article/view/3474?articlesBySameAuthorPage=3

  BACKGROUND AND AIMS: The Swiss Autoimmune Hepatitis Cohort Study is a nationwide registry, initiated in 2017, that collects retrospective and prospective clinical data and biological samples from patients of all ages with autoimmune hepatitis treated at Swiss hepatology centres. Here, we report the analysis of the first 5 years of registry data. […] A total of 291 patients with autoimmune hepatitis have been enrolled, 30 of whom were diagnosed before 18 years of age and composed the paediatric cohort. […] Adult cohort: median age at diagnosis 54 years (range 4264, IQR 1881), 185 (71%) women, 51 (20%) with primary biliary cholangitis variant syndrome, 22 (8%) with primary sclerosing cholangitis variant syndrome, 9 (4%) with inflammatory bowel disease and 66 (32%) with advanced liver fibrosis at diagnosis.

• #53

  https://smw.ch/index.php/smw/article/view/3474?articlesBySameAuthorPage=3

  BACKGROUND AND AIMS: The Swiss Autoimmune Hepatitis Cohort Study is a nationwide registry, initiated in 2017, that collects retrospective and prospective clinical data and biological samples from patients of all ages with autoimmune hepatitis treated at Swiss hepatology centres. Here, we report the analysis of the first 5 years of registry data. […] A total of 291 patients with autoimmune hepatitis have been enrolled, 30 of whom were diagnosed before 18 years of age and composed the paediatric cohort. […] Adult cohort: median age at diagnosis 54 years (range 4264, IQR 1881), 185 (71%) women, 51 (20%) with primary biliary cholangitis variant syndrome, 22 (8%) with primary sclerosing cholangitis variant syndrome, 9 (4%) with inflammatory bowel disease and 66 (32%) with advanced liver fibrosis at diagnosis.

• #54 Autoimmmune hepatitis | Cellular & Molecular Immunology

  https://www.nature.com/articles/s41423-021-00768-8

  A recent study from the US, based on a commercial database including some 37,000,000 patients of all ages from 2014 to 2019, reported an AIH prevalence as high as 31.2/100,000. […] A population-based study from New Zealand published in 2021 reported an overall incidence of 1.93/100,000 from 2008 to 2016, with a significant increase of the incidence from the first 3 years of the observation period to the last three years (1.37-2.39). This is in line with recent European studies, and probably reflects a true increase in the disease frequency, as observed for other autoimmune diseases. […] AIH has long being considered to be rarer in Asia, although a recent systematic review of the literature published until April 2019 reported a similar yearly incidence per 100,000 inhabitants in Asia (1.31), Europe (1.37) and in America (1.00). The worldwide AIH prevalence in this paper was 17.44/100,000 inhabitants, being lower in Asia as compared to Europe and America. This finding may reflect a more recent awareness of AIH in Asia.

• #55 Epidemiology of autoimmune liver disease in Korea: evidence from a nationwide real-world database | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-024-03086-0

  Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are all immune-mediated chronic inflammatory liver diseases. Autoimmune liver diseases are rare, making identification and treatment difficult. To improve clinical outcomes and enhance patient quality of life, we performed an epidemiological study of autoimmune liver diseases based on real-world comprehensive data. […] We used National Health Insurance Service claims data in Korea from 2005 to 2019. Patients were identified using the International Classification of Disease 10th Revision code, and rare intractable disease codes assigned according to the strict diagnostic criteria. […] During the study period, there were 10,212, 6,784, and 888 AIH, PBC, and PSC patients, respectively. The prevalence of AIH, PBC, and PSC in 2019 were 18.4, 11.8, and 1.5 per 100,000 population, while the corresponding incidences were 2.3, 1.4, and 0.3 per 100,000 population, respectively. Analysis of sex-age-standardized data showed that the annual prevalence of these diseases is increasing. The 10-year survival rates were 89.8%, 74.9%, and 73.4% for AIH, PBC, and PSC, respectively.

• #56 The epidemiology of autoimmune liver disease varies with geographic latitude | medRxiv

  https://www.medrxiv.org/content/10.1101/2020.06.27.20141382v1

  The epidemiology of autoimmune liver disease (AILD) is challenging to study because of the diseases rarity and because of cohort selection bias. We assessed the incidence of primary biliary cholangitis (PBC), primary biliary cholangitis (PSC), and autoimmune hepatitis (AIH) in relation to latitude. There were 1314 incident cases of PBC, 396 of PSC, and 1034 of AIH. Crude incidences (95% confidence interval) was: PBC 2.47 (2.34-2.60), PSC 0.74 (0.67-0.82), and AIH 1.94 (1.83-2.06)/100 000/year. PBC incidence correlated with female sex, smoking, and deprivation; PSC incidence correlated with male sex and not smoking; AIH incidence correlated with female sex and deprivation. More northerly latitude was strongly associated with incidence of PBC: 2.16 (1.79-2.60) to 4.86 (3.93-6.00) from 50-57N (p=0.002) and AIH 2.00 (1.65-2.43) to 3.28 (2.53-4.24)(p=0.003), but not PSC 0.82 (0.60-1.11) to 1.02 (0.64-1.61)(p=0.473). Incidence after adjustment for age, sex, smoking, and deprivation status showed similar positive correlations for PBC and AIH with latitude, but not PSC. We describe a novel association between increased latitude and the incidence of PBC and AIH that requires both confirmation and explanation.

• #57 The epidemiology of autoimmune liver disease varies with geographic latitude | medRxiv

  https://www.medrxiv.org/content/10.1101/2020.06.27.20141382v1

  The epidemiology of autoimmune liver disease (AILD) is challenging to study because of the diseases rarity and because of cohort selection bias. We assessed the incidence of primary biliary cholangitis (PBC), primary biliary cholangitis (PSC), and autoimmune hepatitis (AIH) in relation to latitude. There were 1314 incident cases of PBC, 396 of PSC, and 1034 of AIH. Crude incidences (95% confidence interval) was: PBC 2.47 (2.34-2.60), PSC 0.74 (0.67-0.82), and AIH 1.94 (1.83-2.06)/100 000/year. PBC incidence correlated with female sex, smoking, and deprivation; PSC incidence correlated with male sex and not smoking; AIH incidence correlated with female sex and deprivation. More northerly latitude was strongly associated with incidence of PBC: 2.16 (1.79-2.60) to 4.86 (3.93-6.00) from 50-57N (p=0.002) and AIH 2.00 (1.65-2.43) to 3.28 (2.53-4.24)(p=0.003), but not PSC 0.82 (0.60-1.11) to 1.02 (0.64-1.61)(p=0.473). Incidence after adjustment for age, sex, smoking, and deprivation status showed similar positive correlations for PBC and AIH with latitude, but not PSC. We describe a novel association between increased latitude and the incidence of PBC and AIH that requires both confirmation and explanation.

• #58 Autoimmune Hepatitis Management: Recent Advances and Future Prospects

  https://www.mdpi.com/2673-4389/4/2/17

  The initial diagnosis may uncover that up to 40% of AIH patients are asymptomatic, but the majority eventually develop symptoms throughout the disease. […] The acute onset of autoimmune hepatitis has become increasingly common worldwide, affecting not only adults but also children and adolescents. […] AIH is frequently overlooked as a cause of ACLF, necessitating a high index of suspicion for its diagnosis. […] There is no single specific biomarker for AIH diagnosis. […] Consequently, there is a pressing demand for noninvasive blood-based biomarkers that can accurately identify patients at a heightened risk of relapse or function as early indicators of relapse. […] The process poses a clinical challenge, as diagnostic criteria rely on scoring systems developed by national societies.

• #59 Autoimmune Hepatitis Management: Recent Advances and Future Prospects

  https://www.mdpi.com/2673-4389/4/2/17

  The original scoring system, the first standardized system for AIH, initially aimed to recruit prospective AIH research trials rather than diagnosing AIH. […] The simplified scoring system was specifically designed for diagnosing AIH, in contrast to the first two scoring systems used primarily for research purposes. […] The maximum score in the simplified scoring system is eight points. […] Diverse studies have validated the score’s performance across diverse populations. […] The guidelines from the American Association for the Study of Liver Diseases (AASLD) outline three essential parameters for comparing the revised and simplified scoring systems. […] The current pathogenesis of AIH is believed to initiate with the presence of autoantigenic peptides, which are detected by dendritic cells.

• #60 Autoimmune Hepatitis Diagnosis: What It Means For You

  https://rmggastroenterology.com/blog/autoimmune-hepatitis-diagnosis-explained/

  While autoimmune hepatitis can affect anyone of any age, its more common in women. Cases seem to be increasing across the globe. […] AIH is fairly rare. Occurrence varies by region. In the United States, the estimated prevalence is 31.2 out of 100,000. […] Researchers believe that this condition could be underdiagnosed because its symptoms are similar to other types of liver diseases. […] Autoimmune hepatitis is not contagious. […] Most people with autoimmune hepatitis will need lifelong treatment, and regular check-ups with our expert gastroenterologists in Raleigh who can help monitor your liver and improve your quality of life. […] The key to treating autoimmune hepatitis is to keep your immune system from attacking your liver.

• #61 Autoimmune Hepatitis Management: Recent Advances and Future Prospects

  https://www.mdpi.com/2673-4389/4/2/17

  The initial diagnosis may uncover that up to 40% of AIH patients are asymptomatic, but the majority eventually develop symptoms throughout the disease. […] The acute onset of autoimmune hepatitis has become increasingly common worldwide, affecting not only adults but also children and adolescents. […] AIH is frequently overlooked as a cause of ACLF, necessitating a high index of suspicion for its diagnosis. […] There is no single specific biomarker for AIH diagnosis. […] Consequently, there is a pressing demand for noninvasive blood-based biomarkers that can accurately identify patients at a heightened risk of relapse or function as early indicators of relapse. […] The process poses a clinical challenge, as diagnostic criteria rely on scoring systems developed by national societies.

• #62 Autoimmune Hepatitis: 2019 Update

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl19261

  Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease, characterized by the elevation of aminotransferases, presence of anti-nuclear antibody or anti-smooth muscle antibody, elevated immunoglobulin G (IgG), and interface hepatitis/plasma-lymphocytic inflammation based on histology. Recent epidemiological studies have indicated an increasing trend in the prevalence of AIH worldwide, especially in male patients; this trend may suggest the alteration of environmental triggers of disease onset over time. […] The burden of AIH appears to be increasing worldwide, and the point prevalence reported in the published literature after 2000 is shown in Fig. 1. The point prevalence of AIH is reported to be 10 to 25 per 100,000 population in European countries, and 5 to 25 in the Asia-Pacific region.

• #63 Recent advances in the diagnosis and management of autoimmune hepatitis – Polish Archives of Internal Medicine

  https://www.mp.pl/paim/issue/article/16334/

  The need for identifying new biomarkers for the diagnosis of AIH is of particular importance. […] A meta-analysis by Zhang et al showed that ANA have moderate specificity and sensitivity, while SMAs offer high specificity but moderate sensitivity. […] IgG antibodies with a high capacity to bind to several proteins were recently discovered using a protein microarray and verified using ELISAs in patients with AIH. […] AIH is characterized by the same complications (cirrhosis, liver failure, hepatocellular carcinoma, and finally liver-related death) as any other liver disease. […] All AIH patients with at least more than mild disease should receive immunosuppression to achieve CBR and histological remission. […] The therapeutic aim is to achieve remission of the disease at the histological level with as few drug-induced complications as possible.

• #64 Epidemiology and clinical characteristics of autoimmune hepatitis in the Netherlands

  https://lirias.kuleuven.be/2352161

  Epidemiological data on autoimmune hepatitis (AIH) are scarce. In this study, we determined the clinical and epidemiological characteristics of AIH patients in the Netherlands (16.7 million inhabitants). The prevalence of AIH was 18.3 (95% confidential interval [CI]: 17.3-19.4) per 100,000 with an annual incidence of 1.1 (95% CI: 0.5-2) in adults. An incidence peak was found in middle-aged women. This is the largest epidemiological study of AIH in a geographically defined region and demonstrates that the prevalence of AIH in the Netherlands is uncommon. The high percentage of patients with cirrhosis or fibrosis at diagnosis urges the need of more awareness for AIH.

• #65

  http://www.diva-portal.org/smash/record.jsf?pid=diva2:160364

  CONCLUSIONS: The incidence and prevalence figures confirm that AIH is a fairly uncommon disease in the Swedish population. Symptoms at presentation were unspecific, but almost half of the patients were jaundiced, with around 30% having liver cirrhosis. The majority of Swedish AIH patients had AIH type 1. […] Autoimmune hepatitis (AIH) was identified as an entity by the Swedish professor Jan Waldenström in the 1950s. […] Studies of epidemiological data and long term prognosis have been scarce in the literature. […] The aim of the thesis was to explore epidemiological and clinical outcome of AIH. The onset of AIH may be at any age, but the incidence seems to increase after 50 years of age; 75% are females, the overall incidence (0.85/ 100,000 inhabitants and year) and prevalence (11/100,000 inhabitants) are figures that are within the range of another but smaller Scandinavian study. Approximately 30 % had cirrhosis already at diagnosis and 87% displayed at some time positive auto-antibodies indicating AIH (Smooth muscle ab and or antinuclear ab). […] Conclusion: Our epidemiological results confirm that AIH is a fairly uncommon disease, and that many already at time of diagnosis have an advanced disease with liver cirrhosis. There is a clear overrisk for HCC and lymphoma.

• #66 Recent advances in clinical practice: epidemiology of autoimmune liver diseases | Gut

  https://gut.bmj.com/content/70/10/1989.abstract

  Population-based studies and multicentre observational cohort series provide improved understanding of the clinical course that patients experience, highlighting variations in presenting phenotypes geographically and temporally. […] Collectively, while autoimmune liver diseases are rare, the clinical burden is disproportionately high relative to population incidence and prevalence. […] Age, sex and race also impact clinical outcomes, and patient morbidity and mortality are reflected by high need for gastroenterology, hepatology and organ transplant services.

• #67 Epidemiology of autoimmune liver disease in Korea: evidence from a nationwide real-world database | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-024-03086-0

  The number of patients with autoimmune liver disease in South Korea is increasing over time. Further research on autoimmune liver disease is needed to fulfill unmet clinical needs. […] Diagnosis of AILD is difficult as its incidence is only 0.002% per year accounting for only 5% of liver diseases. […] The 10-year survival rates of AILD are 5090%, requiring regular hospital visits, lowering the quality of life, and generating psychological stress. […] The prevalence of AIH, PBC, and PSC were 18.4, 11.8, and 1.5 per 100,000 population, while the corresponding incidence was 2.3, 1.4, and 0.3 per 100,000 population, respectively, in 2019. […] Our results show that the number of newly diagnosed AILD patients in Korea has been increasing since 2010, as has the sex-age-standardized incidence of AIH and PBC. The total number of patients newly diagnosed with AIH was 507 in 2010 and 1,169 in 2019. In the case of PBC, the numbers were 271 and 724 for the respective years. […] The prevalence of AILD has also increased rapidly over time. The cumulative number of AILD patients has been rising.

• #68 Epidemiology of Autoimmune Liver Disease

  https://www.kjg.or.kr/journal/view.html?doi=10.4166/kjg.2023.007

  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. […] 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.

• #69 Genetic risk factors for autoimmune hepatitis: implications for phenotypic heterogeneity and biomarkers for drug response | Human Genomics | Full Text

  https://humgenomics.biomedcentral.com/articles/10.1186/s40246-020-00301-4

  Although the influence of many genetic and environmental factors on the pathogenesis of AIH has been investigated, only some have been confirmed as risk factors for AIH. DRB1 is the sole established genetic risk factor. […] The results from several GWASs should be reported, to estimate the similarity of the genetic factors. There has been only one GWAS on AIH, although many GWASs have been published on other diseases. […] A multi-ethnic, large-scale case-control study is needed to resolve the problem.

• #70 Recent advances in the diagnosis and management of autoimmune hepatitis – Polish Archives of Internal Medicine

  https://www.mp.pl/paim/issue/article/16334/

  The need for identifying new biomarkers for the diagnosis of AIH is of particular importance. […] A meta-analysis by Zhang et al showed that ANA have moderate specificity and sensitivity, while SMAs offer high specificity but moderate sensitivity. […] IgG antibodies with a high capacity to bind to several proteins were recently discovered using a protein microarray and verified using ELISAs in patients with AIH. […] AIH is characterized by the same complications (cirrhosis, liver failure, hepatocellular carcinoma, and finally liver-related death) as any other liver disease. […] All AIH patients with at least more than mild disease should receive immunosuppression to achieve CBR and histological remission. […] The therapeutic aim is to achieve remission of the disease at the histological level with as few drug-induced complications as possible.

• #71 Autoimmune Hepatitis Management: Recent Advances and Future Prospects

  https://www.mdpi.com/2673-4389/4/2/17

  The initial diagnosis may uncover that up to 40% of AIH patients are asymptomatic, but the majority eventually develop symptoms throughout the disease. […] The acute onset of autoimmune hepatitis has become increasingly common worldwide, affecting not only adults but also children and adolescents. […] AIH is frequently overlooked as a cause of ACLF, necessitating a high index of suspicion for its diagnosis. […] There is no single specific biomarker for AIH diagnosis. […] Consequently, there is a pressing demand for noninvasive blood-based biomarkers that can accurately identify patients at a heightened risk of relapse or function as early indicators of relapse. […] The process poses a clinical challenge, as diagnostic criteria rely on scoring systems developed by national societies.

• #72 Autoimmune Hepatitis and Fibrosis

  https://www.mdpi.com/2077-0383/12/5/1979

  […] […] In conclusion, TE has been demonstrated to be a clever non-invasive method for fibrosis assessment in AIH. It is reproducible, rapid and easy to perform, minimally invasive and well tolerated by patients. Moreover, cost-effectiveness analysis has indicated TE as a valid alternative to liver biopsy, which allows a saving of up to $14.000/year for patients. For all these reasons, EASL recommends the use of TE (together with transaminases and IgG) to monitor AIH course and stage liver fibrosis in patients on at least 6 months of therapy.

• #73 Changing epidemiology and treatment responses in autoimmune liver diseases: a 14-year retrospective analysis from a tertiary care center | Egyptian Liver Journal | Full Text

  https://eglj.springeropen.com/articles/10.1186/s43066-024-00395-8

  The male-to-female ratio increased from 1:7.3 to 1:2.6 for AIH, 1:15 to 1:3 for PBC, and 1:7 to 1:2.3 for overlap syndrome. […] The overall male-to-female ratio increased from 1:6.28 to 1:2.45 indicating a trend of increasing male diagnoses over the years. […] The presence of extrahepatic autoimmune diseases was observed in 40.8% of cases, with the highest occurrence rate of 46.4% noted in primary biliary cholangitis (PBC). […] Despite high treatment success rates, relapse rates in patients with AIH continue to pose a significant concern. […] An important finding of our study is the statistically significant association between the presence of portal plasma cell infiltration on liver biopsy at the time of diagnosis and relapse.

• #74 Autoimmune Hepatitis Management: Recent Advances and Future Prospects

  https://www.mdpi.com/2673-4389/4/2/17

  Identifying AIH patients at greater risk for metabolic syndrome enables personalized management to mitigate its long-term consequences in this population. […] Accurate and timely diagnosis, along with effective management, are crucial in improving AIH symptoms and achieving biochemical remission, potentially reducing the risk of disease progression and the need for a liver transplant.

• #75 Epidemiology of Autoimmune Hepatitis (AIH) in the United States between 2014 and 2019: A Population Based National Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8050120/

  Similar to these studies, our patient cohort had majority of Caucasians (75%) and, the prevalence rate was significantly higher in Caucasians when compared to Asians, Hispanics and African Americans. […] In conclusion, this is the largest study to date that evaluates the epidemiology of autoimmune hepatitis in the US. We estimated the 5-year prevalence rate of AIH at 31.2 per 100,000 persons. We found the prevalence of AIH to be higher in females, elderly and Caucasian patients.

• #76 Epidemiology of Autoimmune Hepatitis (AIH) in the United States between 2014 and 2019: A Population Based National Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8050120/

  Similar to these studies, our patient cohort had majority of Caucasians (75%) and, the prevalence rate was significantly higher in Caucasians when compared to Asians, Hispanics and African Americans. […] In conclusion, this is the largest study to date that evaluates the epidemiology of autoimmune hepatitis in the US. We estimated the 5-year prevalence rate of AIH at 31.2 per 100,000 persons. We found the prevalence of AIH to be higher in females, elderly and Caucasian patients.

• #77 Clinical and Molecular Hepatology

  https://e-cmh.org/m/journal/view.php?number=1750

  Aberrant activation of autoimmunity may influence the bad impact on the liver. The global annual incidence rate of AIH was 1.37 per 100,000 persons, and similar among the Asian, European, and American populations. The global prevalence rate of AIH was 17.44 per 100,000 persons with a high level of regional variation. The average age of the onset of AIH is the mid-50s. […] In epidemiology, the prevalence and incidence of these rare autoimmune liver diseases presented an increasing trend. However, it is not definite whether this trend reflects a real increase of the disease or improved identification due to better awareness of the physician.

• #78 The epidemiology of autoimmune liver disease varies with geographic latitude | medRxiv

  https://www.medrxiv.org/content/10.1101/2020.06.27.20141382.full

  For AILDs, the crude incidence of PBC was markedly greater at more northerly latitudes. After adjustment for sex, age, smoking status, and Townsend deprivation quintile there remained a more than doubling in incidence from the 1.96 (1.57-2.34)/100 000/year in the 50 latitude band to 4.55 (3.52-5.58) in the 57 latitude band. […] In 2015, after adjustment for age, sex, smoking status and Townsend quintile, there was a significant increase in the prevalence of both PBC and AIH at more northerly latitudes; such a gradient was not apparent for PSC. […] Here we demonstrate for the first time a striking correlation between increased geographic latitude and disease risk for PBC; the same phenomenon is present to a lesser extent for AIH but absent for PSC.

• #79 Autoimmune Hepatitis Management: Recent Advances and Future Prospects

  https://www.mdpi.com/2673-4389/4/2/17

  Identifying AIH patients at greater risk for metabolic syndrome enables personalized management to mitigate its long-term consequences in this population. […] Accurate and timely diagnosis, along with effective management, are crucial in improving AIH symptoms and achieving biochemical remission, potentially reducing the risk of disease progression and the need for a liver transplant.

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