Materiały źródłowe

• #1 Primary biliary cholangitis: Epidemiology, prognosis, and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10241503/

  Primary biliary cholangitis (PBC) is a chronic cholestatic autoimmune liver disease characterized by a destructive, small duct, and lymphocytic cholangitis, and marked by the presence of antimitochondrial antibodies. The incidence and prevalence of PBC vary widely in different regions and time periods, and although disproportionally more common among White non-Hispanic females, contemporary data show a higher prevalence in males and racial minorities than previously described. […] PBC affects people of all sexes, races, and ethnicities. Earlier reports from case-finding studies showed a median female-to-male ratio of 10:1. Modern literature has demonstrated that, although PBC remains a female-predominant disease, it is also more prevalent in males than previously thought, with a female-to-male ratio closer to 46:1. PBC in males is often diagnosed at an older age, once the disease is more advanced.

• #2

  https://journals.lww.com/hepcomm/fulltext/2023/06010/primary_biliary_cholangitis__epidemiology,.27.aspx

  Primary biliary cholangitis (PBC) is a chronic cholestatic autoimmune liver disease characterized by a destructive, small duct, and lymphocytic cholangitis, and marked by the presence of antimitochondrial antibodies. The incidence and prevalence of PBC vary widely in different regions and time periods, and although disproportionally more common among White non-Hispanic females, contemporary data show a higher prevalence in males and racial minorities than previously described. […] PBC affects people of all sexes, races, and ethnicities. Earlier reports from case-finding studies showed a median female-to-male ratio of 10:1. Modern literature has demonstrated that, although PBC remains a female-predominant disease, it is also more prevalent in males than previously thought, with a female-to-male ratio closer to 46:1. PBC in males is often diagnosed at an older age, once the disease is more advanced.

• #3 Primary biliary cholangitis: Epidemiology, prognosis, and treatment – PubMed

  https://pubmed.ncbi.nlm.nih.gov/37267215/

  Primary biliary cholangitis (PBC) is a chronic cholestatic autoimmune liver disease characterized by a destructive, small duct, and lymphocytic cholangitis, and marked by the presence of antimitochondrial antibodies. The incidence and prevalence of PBC vary widely in different regions and time periods, and although disproportionally more common among White non-Hispanic females, contemporary data show a higher prevalence in males and racial minorities than previously described. […] Outcomes largely depend on early recognition of the disease and prompt institution of treatment, which, in turn, are directly influenced by provider bias and socioeconomic factors. […] Here, we summarize the most up-to-date data regarding the epidemiology, prognosis, and treatment of PBC, providing clinically useful information for its holistic management.

• #4 Primary biliary cholangitis: Epidemiology, prognosis, and treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10241503/

  The pooled global incidence and prevalence of PBC are estimated to be 1.76 and 14.60 per 100,000 persons, respectively. However, these estimates vary significantly according to the search strategy utilized, the size of the study population, and the degree of scrutiny of case finding. […] Contemporary data show that the incidence of PBC is higher in North America (2.75 per 100,000 persons) relative to Europe (1.86 per 100,000 persons) and lowest in the Asian-Pacific region (0.84 per 100,000 persons). The pooled prevalence follows a similar trend, being highest in North America (21.81/1000,000), Europe (14.59/100,000), and then in the Asian-Pacific region (9.82/100,000). […] The incidence and prevalence of PBC increase with age, with a peak range between 60 and 79 years old. PBC is typically identified in middle-aged individuals (40-60 years of age) and is exceptionally rare under 25 years of age.

• #5

  https://journals.lww.com/hepcomm/fulltext/2023/06010/primary_biliary_cholangitis__epidemiology,.27.aspx

  The incidence and prevalence of PBC increase with age, with a peak range between 60 and 79 years old. PBC is typically identified in middle-aged individuals (40-60 years of age) and is exceptionally rare under 25 years of age. […] The pooled global incidence and prevalence of PBC are estimated to be 1.76 and 14.60 per 100,000 persons, respectively. However, these estimates vary significantly according to the search strategy utilized, the size of the study population, and the degree of scrutiny of case finding. […] Contemporary data show that the incidence of PBC is higher in North America (2.75 per 100,000 persons) relative to Europe (1.86 per 100,000 persons) and lowest in the Asian-Pacific region (0.84 per 100,000 persons). The pooled prevalence follows a similar trend, being highest in North America (21.81/1000,000), Europe (14.59/100,000), and then in the Asian-Pacific region (9.82/100,000).

• #6 Primary biliary cholangitis – Wikipedia

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

  PBC is a relatively rare disease, affecting up to one in 3,0004,000 people. […] PBC is more common in women, with a female:male ratio of at least 9:1. The peak incidence of PBC is in the fifth decade of life. […] Epidemiologic studies report heterogeneous incidence rates of 0.33 to 5.8 per 100,000 inhabitants per year, and prevalence rates of 1.9 to 40.2 per 100,000 inhabitants. Such figures, in particular the prevalence, have shown some increase in the last decades. […] The disease has been described worldwide, though North America and Northern Europe have shown the highest incidence and prevalence rates. […] First-degree relatives may have as much as a 500 times increase in prevalence. […] PBC is considered a prime example of the female preponderance in autoimmunity with a female to male ratio of up to 9:1, confirmed by large cohort studies.

• #7 The disease

  https://www.globalpbc.com/the-disease

  Primary biliary cholangitis (PBC) – formerly known as primary biliary cirrhosis – is a chronic, slowly progressive autoimmune liver disease. PBC is a relatively rare disease and occurs worldwide. The incidence and prevalence in various countries and regions differ considerably and have been reported to vary from 0.33 to 5.8 and and 1.91 to 40.2 cases per 100 000 inhabitants, respectively. […] Patients without complete biochemical response to UDCA still benefit from UDCA, emphasizing its importance as first-line therapy for all patients with PBC.

• #8

  https://www.xiahepublishing.com/2310-8819/JCTH-2019-00049

  It is estimated that the incidence of PBC ranges from 0.33 to 5.8 per 100,000 persons, with the reported point prevalence ranging from 1.91 to 33.8 per 100,000 persons, with large differences seen in geographic region. […] Both genetic and environmental factors contribute widely to the pathogenesis of PBC, with epidemiologic data indicating variable prevalence rates of disease into distinct and different geographical areas, or clusters. […] Europe and North America have the highest reported prevalence of PBC worldwide, as reflected in the increasing rate of PBC-related hospitalizations over the last 30 years. […] Despite these limitations, data support the observation that global rates of PBC are rising, as many countries continue to improve diagnostic accuracy, reporting measures, and surveillance of PBC patients. […] Heterogeneity in prevalence can in part be explained by regional variation in diagnostic awareness or access to care; however, several studies have suggested that environmental exposures may play a role in the development of specific regional clusters of PBC.

• #9 Primary biliary cirrhosis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-3-1

  Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure. Affected individuals are usually in their fifth to seventh decades of life at time of diagnosis, and 90% are women. Annual incidence is estimated between 0.7 and 49 cases per million-population and prevalence between 6.7 and 940 cases per million-population (depending on age and sex). […] PBC have a female predominance with an 8:1 female-to-male ratio. PBC affects individuals of all ethnic origin and accounts for 0.6~2.0% of deaths from cirrhosis worldwide. Its prevalence is estimated to be between 6.7 and 940 cases per million-population (the latter in women 40 yrs old in United Kingdom), while its incidence is estimated to be between 0.7 and 49 cases per million-population per year. […] The highest incidence and prevalence rates come from the United Kingdom, Scandinavia, Canada and the United States, all in the northern hemisphere, whereas the lowest is from Australia.

• #10 New developments in the treatment of primary biliary cholangitis – rol | TCRM

  https://www.dovepress.com/new-developments-in-the-treatment-of-primary-biliary-cholangitis-role–peer-reviewed-fulltext-article-TCRM

  Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic liver disease that predominantly affects women in early to middle age. […] The worldwide prevalence rate of PBC is estimated to be between 67 and 940 cases per million population and the incidence is between 0.7 and 49 cases per million population per year. […] The prevalence rates of PBC are high in UK, Scandinavia, Canada and USA. […] The associations between PBC and extrahepatic autoimmune diseases have been well established. More than 50% of patients with PBC have other autoimmune disease. […] The main aim in the management of PBC is to reverse injury from bile duct inflammation, improve the symptoms, slow down the disease progression, improve liver biochemistries, and prevent the long-term consequences of chronic cholestasis, such as pruritus, fatigue, osteoporosis and fat-soluble vitamin deficiencies.

• #11 The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines | Gut

  https://gut.bmj.com/content/67/9/1568

  Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC) is an autoimmune liver disease in which a cycle of immune mediated biliary epithelial cell injury, cholestasis and progressive fibrosis can culminate over time in an end-stage biliary cirrhosis. […] PBC is most prevalent in women and those over the age of 50, but a spectrum of disease is recognised in adult patients globally; male sex, younger age at onset (45) and advanced disease at presentation are baseline predictors of poorer outcome. […] The epidemiology of PBC has been studied extensively. PBC meets the criteria for rare disease status (prevalence 50/100000) in all populations studied. […] Data from the largest UK study, in the north-east of England, suggest a prevalence of definite or probable disease of 35/100000, with an annual incidence of 23/100000.

• #12 Primary biliary cholangitis: diagnosis and management – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/ld/primary-biliary-cholangitis-diagnosis-and-management

  Primarily affecting women, primary biliary cholangitis is a progressive condition that can result in end-stage liver disease. […] A UK study involving 770 individuals with definite or probable PBC demonstrated a prevalence of 35 cases per 100,000 and an annual incidence of 2–3 cases per 100,000. Currently, there are an estimated 20,000 patients living with PBC in the UK. […] Women are predominantly affected by PBC; 9 out of 10 patients diagnosed are women and the prevalence can be as high as 1 in 800 in women aged over 45 years. […] Patients with PBC have a higher mortality rate than that of the general population. […] Left untreated, PBC carries an average survival of 9–10 years from presentation; around 25% of patients develop liver failure during this time. […] Independent risk factors for poorer prognosis include age, hepatomegaly, and advanced fibrosis and cirrhosis.

• #13

  https://journals.lww.com/hepcomm/fulltext/2023/06010/primary_biliary_cholangitis__epidemiology,.27.aspx

  The incidence of PBC steadily rose until the year 2000 across the globe. Growth rates in North America and Europe have since plateaued, whereas the incidence of PBC in the Asian-Pacific region has continued to increase slowly, likely due to improved case reporting. The prevalence of PBC in all three regions has risen steadily over time, although at a faster pace in North America relative to Europe.

• #14 Incidence, prevalence, and outcome of primary biliary cholangitis in a nationwide Swedish population-based cohort | Scientific Reports

  https://www.nature.com/articles/s41598-019-47890-2

  Available epidemiological data on primary biliary cholangitis (PBC) in Sweden originate from regional studies in the 1980s and may not reflect modern day PBC. […] We aimed to estimate incidence and prevalence, survival and death causes, and gender differences in PBC. […] We identified 5,350 adults with PBC. Prevalence of PBC increased steadily from 5.0 (1987) to 34.6 (2014) per 100,000 inhabitants whereas the yearly incidence rate was relatively constant with a median of 2.6 per 100,000 person-years, with a female:male gender ratio of 4:1. […] In conclusion, we found that recorded prevalence of PBC in Sweden has increased substantially during the last 30 years although incidence has been stable. Patients diagnosed in young adulthood were at a 12.7-fold increased risk of death, and male PBC patients had worse prognosis.

• #15 Incidence, prevalence, and outcome of primary biliary cholangitis in a nationwide Swedish population-based cohort | Scientific Reports

  https://www.nature.com/articles/s41598-019-47890-2

  The PBC incidence rate was around 2.6 per 100,000 person-years with some variability that mainly can be explained by the start of different registers. […] There was a steady increase in the registered prevalence of PBC from 5.0 (1987) to 34.6 (2014) per 100,000 inhabitants. […] The female:male ratio in the whole study population was 4.0:1, with a higher proportion of women in the outpatient population 2001-2014. […] Patients with PBC were at increased risk of death. […] The major causes of death in PBC patients were digestive diseases related, most of them liver related. […] Patients with PBC compared to reference individuals had significantly more often a comorbidity diagnosis within all disease groups. […] UDCA was administered as the recommended first line treatment to 79.9% of PBC patients.

• #16 Incidence and prevalence of PBC is increasing | Nature Reviews Gastroenterology & Hepatology

  https://www.nature.com/articles/nrgastro.2014.3

  A population-based study of all patients with primary biliary cirrhosis (PBC) diagnosed from 2000-2008 within a geographically defined area of the Netherlands has found that the incidence and prevalence rates of PBC increased over time (P 0.001). 992 patients with PBC were identified resulting in a mean incidence of 1.1 per 100,000 individuals; prevalence of PBC on 1 January 2008 was 13.2 per 100,000 individuals. […] PBC was not associated with smoking, age of menarche, age at first pregnancy or number of pregnancies.

• #17

  https://journals.lww.com/ajg/fulltext/2019/01000/diagnosis_and_management_of_primary_biliary.13.aspx

  In a more recent analysis of data from 3488 patients receiving routine clinical care in Fibrotic Liver Disease Consortium health systems, investigators found that, from 2004 to 2014, the prevalence of PBC increased from 21.7 to 39.2 per 100,000 persons. This data suggests a possible increase in the prevalence of PBC between 2004 and 2014. […] There is increasing evidence that PBC can be seen in most regions of the world. The previous lower inclusion of minorities in the clinical trials of PBC and lower rates in Asians may have been due to under reporting. In fact, recent data from Japan and China report that the prevalence of PBC is 55/100,000 adults and 49/100,000 adults, respectively.

• #18 Primary biliary cholangitis, a rising health burden

  https://www.explorationpub.com/Journals/ei/Article/1003165

  Primary biliary cholangitis (PBC) is a rare orphan disease with a prevalence of 19 to 402 cases per million persons depending on the geographic location. Earlier studies suggested that women were mainly affected (90 to 95 percent), mostly between the ages of 30 to 65 years, commonly in their 40s or 50s, with the least reported age as young as 15 years and as old as 93 years in women. The incidence and prevalence of the disease increases with age. Newer studies suggest that the female-male ratio may be closer to 4:1. Male PBC patients have a higher rate of progression to cirrhosis and HCC, are diagnosed at a later stage, and respond less well to ursodeoxycholic acid (UDCA). […] Geographically, PBC is present throughout the world and in all races and ethnicities. It is more common in North America (Northern Midwest regions of the US) and Northern Europe. The disease burden is increasing over time, particularly in Asian populations, possibly due to better diagnostic tools rather than a true rising incidence. PBC prevalence is 100 times higher in first-degree relatives than in the general population and occurs in twins, indicating genetic susceptibility. In a study of over 1,000 patients, a history of urinary tract infections, past smoking, or use of hormone replacement therapies were also significantly associated with an increased risk of having PBC. […] Adaptive innate immunity and allelic variations in major histocompatibility complex (MHC) class II (D.R., D.Q.) are associated with susceptibility to PBC.

• #19 CT and MR imaging of primary biliary cholangitis: a pictorial review | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1186/s13244-023-01517-3

  Primary biliary cholangitis (PBC) is a rare chronic autoimmune-mediated cholestatic liver disease involving medium and small bile ducts that can lead to liver fibrosis and cirrhosis. […] According to statistics from the 2022 AsiaPacific Society for the Study of the Liver (APASL) guidelines and the latest meta-analysis, the estimated global incidence and prevalence of PBC are 17.6 and 146 per million, respectively, with North America being the highest, followed by Europe, and the lowest in the AsiaPacific region. Notably, all three regions showed an upward trend in the incidence and prevalence of PBC, with North America showing the fastest growth in prevalence. […] PBC is a disease in which female involvement is overwhelmingly dominant (the female-to-male incidence ratio is approximately 3.910:1); however, the incidence in male patients has been gradually increasing (female-to-male ratio (3.96.2:1) in recent years.

• #20 Primary Biliary Cholangitis (Primary Biliary Cirrhosis): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/171117-overview

  Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic disease of the liver, presumably autoimmune in nature, that leads to progressive cholestasis through intrahepatic bile duct destruction and often end-stage liver disease. The name change reflects the fact that cirrhosis occurs only in the late stage and therefore does not correctly identify patients with early-stage disease. […] PBC is most frequently a disease of women and occurs between the fourth and sixth decades of life. […] Although the epidemiology of primary biliary cholangitis has not been studied systematically, the published prevalence is 65.4 cases for women and 12.1 cases for men (40.2 cases overall) per 100,000 population. The incidence of the disease has been estimated as 4.5 cases for women and 0.7 cases for men (2.7 cases overall) per 100,000 population.

• #21 Primary Biliary Cholangitis (Primary Biliary Cirrhosis): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/171117-overview

  Primary biliary cholangitis is reported to be more prevalent in the United Kingdom and Scandinavia. The prevalence of the disease has been estimated as 24 cases per 100,000 population in Newcastle, United Kingdom; 12.9 cases per 100,000 population in Northeast England; 1.9 cases per 100,000 population in Victoria, Australia; 2.2 cases per 100,000 population in Ontario, Canada; 2.7 cases per 100,000 population in Estonia; 9.2 cases per 100,000 in Malm, Sweden; and 15.1 cases per 100,000 population in Umea, Sweden. […] Primary biliary cholangitis is more common in Northern Europeans and is less common in populations of African descent. […] Women account for 75-90% of patients with primary biliary cholangitis. Males who are affected have a disease course similar to that of females; however, men appear to be more likely to develop hepatocellular carcinoma. […] Primary biliary cholangitis mostly affects middle-aged women, with a mean age of 39 years. Onset usually occurs in persons aged 30-65 years. However, patients as young as 22 years and as old as 93 years at the time of diagnosis have been reported.

• #22 Primary Biliary Cholangitis: Symptoms & Causes

  https://liverfoundation.org/liver-diseases/autoimmune-liver-diseases/primary-biliary-cholangitis-pbc/

  Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic liver disease resulting from progressive destruction of the bile ducts in the liver called the intrahepatic bile ducts. […] Primary Biliary Cholangitis (PBC) affects mostly women, but more men are now being diagnosed. […] Researchers estimate that in the United States, about 65 out of every 100,000 women have PBC. […] The disorder usually becomes apparent during middle age, initially affecting most individuals between the ages of 45 to 65 years. […] There is no cure for Primary Biliary Cholangitis (PBC), but there is medication available to manage the disease and slow down the progression of liver damage. […] Women are nine times more likely than men to develop Primary Biliary Cholangitis (PBC), meaning that women make up about 90% of Primary Biliary Cholangitis (PBC) cases.

• #23 The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines | Gut

  https://gut.bmj.com/content/67/9/1568

  Comparison with other Northern European and North American cohorts suggest these rates are broadly typical. […] Reported prevalence appears stable following several years of increase. This may reflect a now fully evolved change in diagnostic activity and practice linked to increased awareness of the disease. […] PBC prevalence is asymmetrical within the population with markedly higher rates being seen in women than men (the difference is 10-fold). […] UK data suggest that PBC is diagnosed at a later stage in men, potentially reflecting perception bias among clinicians. […] PBC is also typically a disease of older patients with the median age at diagnosis being 65 years. […] The dual effects of age and sex mean that PBC can reach a prevalence of as high as 1 in 800 in women over the age of 45 years.

• #24 Primary biliary cholangitis – Wikipedia

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

  An association of a greater incidence of PBC at latitudes more distant from the Equator is similar to the pattern seen in multiple sclerosis. […] Typical disease onset is between 30 and 60 years, though cases have been reported of patients diagnosed at the ages of 15 and 93. Prevalence of PBC in women over the age of 45 years could exceed one in an estimated 800 individuals.

• #25

  https://link.springer.com/article/10.1007/s12072-021-10276-6

  Primary biliary cholangitis (PBC) is a chronic intrahepatic cholestatic disease with not fully elucidated pathogenesis. PBC typically affects middle-aged women, commonly presents with fatigue and pruritus, or with an asymptomatic elevation of serum alkaline phosphatase (ALP)/glutamyl transpeptidase (GGT). The reported incidence and prevalence of PBC in the AsiaPacific region (8.4, and 98.2118.8 per million, respectively) were lower than that in North America (27.5 and 218.1 per million, respectively) and Europe (18.6 and 145.9 per million, respectively). Of note, the geographical differences in PBC epidemiology exist even within the AsiaPacific region, with a higher reported prevalence in Japan and China (191.18 per million) and a much lower reported prevalence in South Korea and Australia (39.09 per million). The prevalence of PBC in the AsiaPacific region has become higher than once deemed and increased quickly. A recent study in Japan demonstrated that the point prevalence of PBC was 338 per million, which was comparable to that in Europe and North America. Another Japanese study reported that PBC was diagnosed in 5.7% of the women with asymptomatic serum GGT elevation at the annual health check-up among a large population, yielding an estimated PBC prevalence of 3400 per million in women over 40 years old and 840 per million in the whole population in Okinawa Prefecture. […] Overall, the estimated global incidence and prevalence were 17.6 per million persons/year and 146 per million, respectively.

• #26 The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines | Gut

  https://gut.bmj.com/content/67/9/1568

  Familial PBC is clearly recognised, with familial rates similar to those seen in other autoimmune conditions. The reported sibling relative risk for PBC is 10. […] The relative risk for familial disease is greatest, at 35, for the daughters of mothers with PBC, reflecting in part the disease demographics.

• #27 Primary biliary cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

  Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed. It previously was called primary biliary cirrhosis. […] Although it affects both sexes, primary biliary cholangitis mostly affects women. […] Researchers think that genetic factors combined with certain environmental factors trigger primary biliary cholangitis. […] The following factors may increase your risk of primary biliary cholangitis: Most people with primary biliary cholangitis are women. It’s most likely to occur in people 30 to 60 years old. You’re more likely to get the condition if you have a family member who has or had it. It’s most common in people of northern European descent, but primary biliary cholangitis can affect all ethnicities and races. […] As liver damage worsens, primary biliary cholangitis can cause serious health problems, including liver scarring, called cirrhosis. Cirrhosis makes it difficult for your liver to work and may lead to liver failure. […] Primary biliary cholangitis is associated with other disorders, including those that affect the thyroid, skin and joints. It also can be associated with dry eyes and mouth, a disorder called Sjogren’s syndrome.

• #28 Primary biliary cholangitis: diagnosis and management – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/ld/primary-biliary-cholangitis-diagnosis-and-management

  A cohort study demonstrated that 61.2% of PBC patients had a comorbid autoimmune condition. […] There is a strong association between PBC and other autoimmune conditions including Sjögren’s syndrome, autoimmune thyroid disease, CREST syndrome, scleroderma, and Raynaud’s disease. […] Owing to increased awareness and earlier diagnosis, as well as the introduction of effective medical treatments, the number of liver transplantations for PBC in Europe and North America is falling.

• #29 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 mean ages at diagnosis were 40.9715.93 years for AIH, 49.4311.87 years for PBC, and 44.6117.17 years for patients with overlap syndrome. […] 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). […] In primary biliary cholangitis (PBC), treatment responses were evaluated using the Paris-II criteria. […] In our study, the relapse rate was determined to be 48.5%. […] The relationship between portal plasma cell infiltration on liver biopsy and relapse also warrants further investigation through more extensive and multicenter studies, which could inform long-term treatment or alternative treatment regimens for these patients.

• #30 Extrahepatic Manifestations of Primary Biliary Cholangitis

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl16365

  Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogrens syndrome, thyroid dysfunction and systemic sclerosis. […] A systematic review of population-based epidemiological studies reported that PBC incidence rates range from 0.9 to 5.8 per 100,000 inhabitants/year and prevalence rates range from 1.9 to 40.2 per 100,000 inhabitants/year. […] Given the high concordance rate among monozygotic twins and high level of PBC aggregates in families, there appears to be a genetic predisposition towards this disease. […] Extrahepatic manifestations of PBC are generally autoimmune in nature and occur between 32% and 63% of patients.

• #31 Extrahepatic Manifestations of Primary Biliary Cholangitis

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl16365

  It is well known that people with one autoimmune disease are more prone to developing other autoimmune diseases. These patients are thought to have an underlying immunological dysfunction and the interplay between genetic, immunological, environmental and hormonal factors plays a role in the development of disease.

• #32 Primary biliary cholangitis: Epidemiology, prognosis, and treatment – PubMed

  https://pubmed.ncbi.nlm.nih.gov/37267215/

  Liver disease progression is inevitable in most untreated individuals, with fibrosis and cirrhosis ensuing as a consequence of the inflammatory and cholestatic processes. […] The cumulative incidence of major non-neoplastic hepatic complications has been reported as 15% after 15 years of follow-up. […] PBC will recur in 20%40% of post-transplant patients. […] The frequency of post-transplant PBC is highly variable among studies (9%61%).

• #33 The epidemiology of primary biliary cirrhosis

  https://www.periodicos.capes.gov.br/index.php/acervo/buscador.html?task=detalhes&id=W2004437574

  Patients usually receive a diagnosis of primary biliary cholangitis (PBC) at an early stage, based on biochemical analyses. […] We investigated the proportion of patients who progress to moderate or advanced PBC and factors associated with progression and patient survival. […] Approximately half of patients with early stage PBC progress to a more severe stage within 5 years. Progression is associated with an increased risk of a clinical event, so surveillance is important for patients with early stage PBC.

• #34 Early-Stage Primary Biliary Cholangitis: Progression and Outcomes – Gastroenterology Advisor

  https://www.gastroenterologyadvisor.com/news/early-stage-primary-biliary-cholangitis-progression-and-outcomes/

  Nearly half of patients with early-stage PBC progress to a more severe stage within 5 years. […] Progression is associated with increased risk of a clinical event, so surveillance is important for patients with early-stage PBC. […] Study investigators concluded, our internationally representative study provides a comprehensive overview of the natural history of PBC patients with early disease stage, showing that almost one out of two patients with early biochemical disease will transit to moderately advanced disease and, approximately one-sixth of them can progress to advanced stage. These transitions are associated with an increased probability of clinical events. The findings underline the importance of clinical surveillance in PBC patients with early biochemical disease stage.

• #35 Primary Biliary Cirrhosis: New Options | Borland Groover

  https://borlandgroover.com/patient-resources/gastroenterology/gi-education/primary-biliary-cirrhosis-new-options

  Primary biliary cirrhosis (PBC) is a cholestatic liver disease in which 90% of patients are women, usually between age 30 and 65 at the time of diagnosis. […] In both Europe and North America there is a decreasing incidence of liver transplant secondary to PBC. […] Without therapy, the 10 year survival of asymptomatic patients ranges from 50%-70%, while the median survival for symptomatic patients ranges from 5-8 years. […] The presence of both an elevated serum alkaline phosphatase and a positive AMA is predictive of PBC in most cases. […] Due to the slow pace of liver injury in PBC, surveillance for progression may include liver biopsy, serum markers of fibrosis or non-invasive testing such as liver elastography. […] PBC is currently the sixth leading cause of liver transplant in the U.S. (down from the most common cause in the mid 1980s).

• #36 Risk of HCC Warrants Careful Surveillance in People With Primary Biliary Cholangitis

  https://www.ajmc.com/view/risk-of-hcc-warrants-careful-surveillance-in-people-with-primary-biliary-cholangitis

  People with primary biliary cholangitis (PBC) and cirrhosis should be aggressively screened for hepatocellular carcinoma (HCC), using twice-annual liver screening, according to a new report in Clinics in Liver Disease. […] The authors wrote about the latest evidence related to the risks and outcomes of HCC in people with PBC. They said such cases warrant vigilance. […] Advanced fibrosis is the most important risk factor for the development of HCC, but there are other reports showing the development of HCC in PBC without evidence of advanced disease, the investigators wrote. […] Among autoimmune diseases, PBC appears to be associated with the greatest HCC risk. […] The best chance of survival for patients is liver transplant, they added. […] At present, the authors added, cirrhosis is the only indication for which all scientific societies recommend HCC screening. They said cirrhosis in PBC warrants screening every 6 months given the high risks associated with HCC, and they added that more studies are needed to better understand the ways in which treatment response for PBC might affect the risk of developing HCC.

• #37 Predicting outcome in primary biliary cirrhosis | Annals of Hepatology

  https://www.elsevier.es/en-revista-annals-hepatology-16-articulo-predicting-outcome-in-primary-biliary-S1665268119308385

  The current AASLD guideline on PBC recommends surveillance for esophageal varices of patients with a platelet count of 3 or Mayo risk score 4.1.6. […] A recent systematic review and meta-analysis demonstrated a pooled relative risk of the development of HCC of 18.80 (95% CI, 10-81-26.79) for PBC patients compared with a general population, which makes HCC the most prevalent cancer in PBC. […] Surveillance strategies resulting in early diagnosis of HCC may improve outcome.

• #38 Incidence, prevalence, and outcome of primary biliary cholangitis in a nationwide Swedish population-based cohort | Scientific Reports

  https://www.nature.com/articles/s41598-019-47890-2

  The disease predominantly affects women and is typically diagnosed between 50 and 60 years of age. […] The majority of patients are asymptomatic at diagnosis and often PBC is suspected because of abnormal laboratory tests, particularly a raised serum alkaline phosphatase (AP) level. […] In recent years, much focus has been on finding optimal criteria to define UDCA responders and non-responders. […] To underscore the need of novel treatment options, updated and robust data on the epidemiology and current management of PBC are needed also for Sweden as available information dates from regional studies in the 1980s. […] This paper aims to provide these data by two complimentary approaches. […] We identified 5,350 adults (15 years of age and older) with a PBC diagnosis and 50,145 reference individuals.

• #39 Diagnosis, Treatment, and Monitoring of Patients With Primary Biliary Cholangitis – Gastroenterology & Hepatology

  https://www.gastroenterologyandhepatology.net/archives/september-2016/diagnosis-treatment-and-monitoring-of-patients-with-primary-biliary-cholangitis/

  Women are affected much more often than men, at a ratio of approximately 9:1. […] This is a progressive disease; most people are unaware that, after hepatitis C virus infection, PBC is one of the more common indications for liver transplantation in women. […] Early diagnosis and initiation of treatment as soon as possible are crucial for maximizing outcomes in patients with PBC. […] Patients who start treatment early have similar survival over a 10- to 15-year period as the general population. […] In contrast, late initiation of therapy is associated with very poor survival. […] Current treatment works in approximately 60% to 70% of patients. Therefore, up to 30% to 40% of patients do not respond to UDCA, and there is still a need for new therapeutic strategies for patients with PBC.

• #40 Current Landscape and Evolving Therapies for Primary Biliary Cholangitis

  https://www.mdpi.com/2073-4409/13/18/1580

  Primary Biliary Cholangitis (PBC), formerly Primary Biliary Cirrhosis, is a progressive cholestatic disease that if untreated might progress to cirrhosis and liver decompensation requiring liver transplant. […] The incidence of PBC in the European Union (EU), the USA and Canada, Asia and Australia range between 0.9 and 5.8 per 100,000 population per year, while the prevalence is 1.9–40.2 per 100,000. In Western countries, it is estimated that 1 in 1000 women over the age of 40 live with PBC. […] The prevalence of PBC has witnessed a constant increase in the last two decades in the EU, North America and the Asia-Pacific regions while the quality of life is improving along with a reduction in liver transplants and mortality. […] The introduction of ursodeoxycholic acid (UDCA), a secondary bile acid normally present in human bile at low concentrations, in the treatment of PBC patients has changed the disease course, improving transplant-free survival rates to up to 60% of patients, thereby reducing mortality.

• #41 Current Landscape and Evolving Therapies for Primary Biliary Cholangitis

  https://www.mdpi.com/2073-4409/13/18/1580

  Currently, life expectancy in PBC patients under UDCA therapy is similar to non-PBC patients. […] The diagnosis of PBC, in these early stages, is confirmed when at least two out of the following three criteria are satisfied: (1) biochemical indicators of cholestasis, particularly elevated levels of alkaline phosphatase (ALP); (2) detection of antimitochondrial antibodies (AMA) or other PBC-specific autoantibodies, such as sp100 or sp210, if AMA is not detected; and, (3) typical histopathology features at liver biopsy. […] The introduction of ursodeoxycholic acid (UDCA), a secondary bile acid normally present in human bile at low concentrations, in the treatment of PBC patients has changed the disease course, improving transplant-free survival rates to up to 60% of patients, thereby reducing mortality. […] PBC is a progressive hepatic disease and the early stages of the disease manifest with fatigue, pruritus, right upper quadrant abdominal discomfort and jaundice.

• #42 Incidence, prevalence, and outcome of primary biliary cholangitis in a nationwide Swedish population-based cohort | Scientific Reports

  https://www.nature.com/articles/s41598-019-47890-2

  Liver transplantations for PBC were in Sweden performed from 1997 on, at a median number of 9.5 per year after PBC diagnosis. […] This nationwide population-based study found a prevalence rate of PBC in Sweden of about 35 per 100,000 inhabitants at the end of the observation period. […] Our study included 5,350 adult PBC cases and the prevalence is among the highest reported worldwide. […] Our data do not show significantly increased overall survival since the introduction of UDCA in the Swedish health care in 1998. […] Indeed, our registry study confirms a strong association of PBC with other immune-mediated endocrine, musculoskeletal and skin diseases. […] PBC has a substantial morbidity and mortality, especially from liver related causes.

• #43 About PBC | PBC Foundation

  https://www.pbcfoundation.org.uk/what-is-pbc/about-pbc/

  PBC is an autoimmune liver condition. PBC is becoming more commonly diagnosed. PBC affects nine women to every one man. The common age of diagnosis is between 35 55. There are an estimated 22,000 PBC patients in the UK. […] PBC was formerly known as Primary Biliary Cirrhosis. In 2015, the name was changed to Primary Biliary Cholangitis. […] One of the key aspects of living with PBC is lifelong surveillance, including monitoring of symptoms, or of liver biochemistry, and taking into account Urso-response and the most appropriate care pathway for each patient.

• #44 Osteoporosis in Primary Biliary Cholangitis: Prevalence, Impact and Ma | CEG

  https://www.dovepress.com/osteoporosis-in-primary-biliary-cholangitis-prevalence-impact-and-mana-peer-reviewed-fulltext-article-CEG

  Management of osteoporosis in PBC remains challenging. […] The optimal strategy for the diagnosis, prevention and treatment of osteoporosis in PBC is not well established. […] Preventing the reduction of bone density is important to decrease the risk of fractures and improve morbidity and mortality. […] The future for osteoporosis therapy in PBC remains uncertain.

• #45 Osteoporosis in Primary Biliary Cholangitis: Prevalence, Impact and Ma | CEG

  https://www.dovepress.com/osteoporosis-in-primary-biliary-cholangitis-prevalence-impact-and-mana-peer-reviewed-fulltext-article-CEG

  Primary biliary cholangitis (PBC) is a chronic, cholestatic condition associated with symptoms that directly impact the quality of life in those afflicted with the disease. […] The prevalence of osteoporosis in PBC is expected to increase in conjunction with the rising prevalence of PBC as a whole. […] Osteoporosis is a known complication in multiple etiologies of liver disease, but its epidemiology has been studied most extensively in the context of cholestatic liver disease. The prevalence of osteoporosis in PBC ranges from 20% to 45% with the highest prevalence in those with cirrhosis on the liver transplant list. […] The incidence and prevalence of PBC-related osteoporosis is expected to rise as the prevalence of the disease grows overall. […] The optimal timing for diagnosis and monitoring of osteoporosis in PBC is not well established. However, expert consensus recommends screening with DEXA in all patients with PBC at the time of diagnosis.

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