Prevalence Of Primary Biliary Cholangitis Research Articles

Incidence and prevalence of primary biliary cholangitis in the Netherlands – A nationwide cohort study

Background & AimsAlthough primary biliary cholangitis (PBC) is considered a rare disorder, accurate determination of its incidence and prevalence remains challenging due to limited comprehensive population-based registries. We aimed to assess the incidence and prevalence of PBC in the Netherlands over time through the nation-wide Dutch PBC Cohort Study (DPCS). MethodsDPCS included retrospectively every identifiable patient with PBC in the Netherlands from 1990 onwards in all 71 Dutch hospitals. Incidence and prevalence were assessed between 2008-2018 by Poisson regression between sex and age groups over time. ResultsOn January 1st 2008, there were 1458 patients with PBC in the Netherlands. Between 2008-2018, 2187 individuals were newly diagnosed, 46 were transplanted and 468 died. The yearly incidence of PBC in 2008 was 1.38, and increased to 1.74 per 100.000 persons in 2018. When compared to those aged <45 years, females aged 45-64 years (adjusted Incidence Rate Ratio [aIRR] 4.21, 95%CI 3.76-4.71, p<0.001) and males ≥65 years (aIRR 14.41, 95%CI 9.62-21.60, p<0.001) had the highest risk to be diagnosed with PBC. The male-to-female ratio of patients newly diagnosed with PBC during the study period was 1:14 in those <45 years, 1:10 in patients aged 45-64 years, and 1:4 in those ≥65 years. Point prevalence increased from 11.9 in 2008 to 21.5 per 100.000 persons in 2018. Average annual percent change (AAPC) in this time period was 5.94% (95%CI 5.77-6.15, p<0.05), and was the highest among the population ≥65 years (5.69%, 95%CI 5.32-6.36, p<0.001). ConclusionsIn this nationwide cohort study, we observed an increase in both incidence and prevalence of PBC in the Netherlands over the past decade, with marked age and sex differences. Impact And ImplicationsThis nationwide Dutch PBC cohort study, including all hospitals in the Netherlands, showed that the PBC incidence and prevalence has increased over the last decade. The age-dependent PBC incidence rate differed for males (highest risk ≥65 years) and females (highest risk between 45 and 65 years), which may be related to a difference in the timing of exposure to environmental triggers of PBC. The largest increase in PBC prevalence over time was observed in the population ≥65 years, which may have implications for the use of second line therapies. These results therefore indicate that further studies are needed to elaborate on the advantages and disadvantages of add-on therapies in the elderly population.

Primary biliary cholangitis: Epidemiology, prognosis, and treatment.

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. Ursodeoxycholic acid remains the initial treatment of choice for PBC, with obeticholic acid and fibrates (off-label therapy) reserved as add-on therapy for the management of inadequate responders or those with ursodeoxycholic acid intolerance. Novel and repurposed drugs are currently at different stages of clinical development not only for the treatment of PBC but also for its symptomatic management. 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.

Epidemiological trends and outcomes of primary biliary cholangitis in South Korea between 2009 and 2019.

There are no longitudinal studies on the epidemiology of primary biliary cholangitis (PBC) in Korea. This study aimed to elucidate the temporal trends in the epidemiology and outcomes of PBC in South Korea between 2009 and 2019. The epidemiology and outcomes of PBC were estimated using data from the Korean National Health Service database. Temporal trends in the PBC incidence and prevalence were analyzed using join-point regression. Transplant-free survival was analyzed according to age, sex, and ursodeoxycholic acid (UDCA) treatment using Kaplan-Meier and Cox regression analyses. The age and sex-standardized incidence between 2010 and 2019 (total patients, 4230) was 1.03 per 100,000 per year on average and increased from 0.71 to 1.14 per 100,000 with an annual percent change (APC) of 5.5. The age and sex-standardized prevalence between 2009 and 2019 was 8.21 per 100,000 on average and increased from 4.30 to 12.32 per 100,000 with an APC of 10.9. The increasing trend in prevalence was prominent in males and elderly individuals. Among patients with PBC, 98.2% received UDCA with 77.3% adherence. The 5-year transplant-free overall survival rate was 87.8%. Male sex and low adherence to UDCA were associated with all-cause death or transplantation (hazard ratios of 1.59 and 1.89, respectively), and liver-related death or transplantation (hazard ratios of 1.43 and 1.87, respectively). The incidence and prevalence of PBC in Korea increased significantly between 2009 and 2019. Male sex and low adherence to UDCA were poor prognostic factors for PBC.

A population-based characterization study of anti-mitochondrial M2 antibodies and its consistency with anti-mitochondrial antibodies.

This study aims to estimate the prevalence of anti-mitochondrial antibody subtype M2 (AMA-M2) and assess its consistency with AMA in a general population. A total of 8954 volunteers were included to screen AMA-M2 using enzyme-linked immunosorbent assay. Sera with AMA-M2>50 RU/mL were further tested for AMA using an indirect immunofluorescence assay. The population frequency of AMA-M2 positivity was 9.67%, of which 48.04% were males and 51.96% were females. The AMA-M2 positivity in males had a peak and valley value of 7.81% and 16.88% in those aged 40 to 49 and ≥70years, respectively, whereas it showed a balanced age distribution in females. Transferrin and immunoglobulin M were the risk factors for AMA-M2 positivity and exercise was the only protective factor. Of 155 cases with AMA-M2>50 RU/mL, 25 cases were AMA-positive, with a female-to-male ratio of 5.25:1. Only 2 people, with very high AMA-M2 of 760 and >800 RU/mL, met the diagnostic criteria of primary biliary cholangitis (PBC), making the prevalence of PBC 223.36 per million in southern China. We found that AMA-M2 has a low coincidence rate with AMA in the general population. Anew decision-making point for AMA-M2 is needed to improve consistency with AMA and diagnostic accuracy.

Geographical Disparity in Primary Biliary Cholangitis Prevalence: A Mini Review

Primary biliary cholangitis (PBC) has been shown to occur more often in the West than in the East. The reason for this geographical disparity remains to be determined. Some researchers have considered that this was merely due to the lack of awareness on PBC in the East. Thus, the reported case and epidemiological studies on this disease remains scarce in the East. Other studies have suggested genetic predisposition and environmental factors proven to play important roles in the disease pathogenesis. In addition, these might also cause the disease to be more susceptible in some populations. The findings reported by multiple genome-wide association studies (GWAS) in recent years have not yet identified the specific genes responsible for the development of PBC, with different susceptible genes identified on each study in different regions. The present review describes some factors that might be associated with this geographical disparity.

Geographical region and clinical outcomes of patients with primary biliary cholangitis from Western Europe.

The are geographic variations in the incidence and prevalence of primary biliary cholangitis (PBC). The aim was to explore whether clinical outcomes of patients within Western Europe differ according to geographical region. Ursodeoxycholic acid-treated patients from European centers from the Global PBC database diagnosed from 1990 onwards were included. Patients with a time lag > 1 year from diagnosis to start of follow-up were excluded. Differences in baseline characteristics were studied according to North/South and East/West, whereas outcomes (transplant-free survival and decompensation) were studied with center latitude and longitude. Cox regression analyses were adjusted for age, sex, diagnosis year, biochemical markers, and cirrhosis as a time-dependent covariate. One thousand eight hundred seventy-eight patients were included, and there were no geographical differences in age or sex, with a mean age of 54 years and 89% female patients. Those in North Europe were more often of a moderately advanced/advanced Rotterdam biochemical stage (28.4%) compared with South Europe (20.6%). Additionally, they exhibited higher median alkaline phosphatase (2.0 ×ULN vs. 1.4 ×ULN) and transaminases. In multivariable analysis, there was a significant interaction between center latitude and longitude for decompensation (P < 0.001) and a trend for transplant-free survival, in which the Northwestern area demonstrated an increased risk for poor outcomes as compared to the reference (Paris). We describe geographic variations in outcomes for patients across Europe from specialist centers in the Global PBC Study Group. Further study is important to explore the potential individual, environmental, and healthcare-related factors that may be contributors.

S1252 Prevalence of Diagnosed Primary Biliary Cholangitis and Associated Conditions in the United States: Results From a National Electronic Patient Database

Introduction: Primary biliary Cholangitis (PBC) is an autoimmune disease that is associated with a number of autoimmune disorders. PBC is associated with liver and non-liver organ co-morbidities that contribute to clinical outcomes. Several studies have suggested an increase in the incidence and prevalence of PBC, although this has not been assessed in a large cohort and has not been consistent across all epidemiologic studies. We determined the nationwide prevalence of PBC and its association with other autoimmune and non-liver related conditions in the United States (US). Methods: A database (Explorys Inc, Cleveland, OH) of electronic health record data from 26 major integrated US healthcare systems was used. Based on Systematized Nomenclature of Medicine – Clinical Terms (SNOMED-CT), patients (age >18 years) with a diagnosis of PBC between 1999 to present were identified. Data were analyzed in patients with/without a diagnosis of PBC. The prevalence of associated diseases was compared in in the 2 groups, and statistical univariate binary logistic model was performed. Results: Of the 70,383,890 individuals in the database, we identified 11,070 cases of PBC, with a prevalence of 15.7/100,000 persons (0.02%). Prevalence was higher in females than males [odds ratio (OR) 32.84; 95% Confidence Interval (CI) 30.50-35.37, p < 0.0001], adults (≥ 65 years) versus adult (18-65 years) (OR) 2.18; 95% CI 2.07-2.30, p < 0.0001) and whites versus non-whites (OR 14.54; 95% CI 13.63-15.51, p < 0.0001) (Table). PBC patients were at higher odds (Table) of co-morbidities including ischemic heart disease, cerebrovascular accident, congestive heart failure and sarcopenia when compared to non-PBC patients (p < 0.0001). PBC patients were more likely than non-PBC patients to have primary sclerosing cholangitis ( OR 1061.43) autoimmune hepatitis (OR 537.93), Type 1 diabetes mellitus (OR 240.58), ulcerative colitis (OR 15.25), Crohn’s disease (OR 7.84), celiac disease (OR 8.99), autoimmune thyroiditis (OR 7.69), systemic lupus erythematosus (OR 14.66) and other autoimmune disorders (Figure). Conclusion: Our large nationwide analysis study demonstrates higher prevalence of PBC in the US than previously reported, which is likely a result of diagnosis at an early stage and prolonged survival that may be related to improved management. The high association between PBC and other autoimmune conditions suggest shared common pathogenesis of immune-mediated destruction of end organs in genetically susceptible individuals. Table 1. - Baseline Characteristic of Patients with Primary Biliary Cholangitis and Control Group PBC n=11,070 (%) No PBC n=70,372,820 (%) OR (95%CI) p-value Demographics Age 18-65 4,490(41%) 47,940,720(68%) 0.32(0.31-0.33) <0.0001 Age > 65 6,620(60%) 21,251,790(30%) 3.44(3.31-3.57) <0.0001 Male 1,610(15%) 31,420,380(45%) 0.21(0.20-0.22) <0.0001 Female 9,390(85%) 38,453,360(55%) 4.64(4.41-4.89) <0.0001 Caucasian 8,770(79%) 37,743,080(54%) 3.30(3.15-3.45) <0.0001 Comorbidities T2DM 2,810(25%) 5,650,420(8%) 3.90(3.73-4.07) <0.0001 HTN 1,950(18%) 3,493,560(5%) 4.09(3.89-4.29) <0.0001 HLD 5,940(54%) 11,779,380(17%) 5.76(5.55-5.98) <0.0001 Tobacco use 2,220(20%) 6,486,950(9%) 2.47(2.36-2.59) <0.0001 Hypothyroidism 3,300(30%) 4,049,030(6%) 6.96(6.68-7.25) <0.0001 Ischemic Heart Disease 1,240(11%) 2,496,690(4%) 3.43(3.23-3.64) <0.0001 CHF 1,070(10%) 1,834,510(3%) 4.00(3.75-4.26) <0.0001 Sarcopenia 4,910(44%) 5,480,850(8%) 9.44(9.09-9.80) <0.0001 Alcohol abuse 320(3%) 1,090,250(2%) 1.89(1.69-2.11) <0.0001 Univariate analysis used to calculate OR, OR; odds ratio, CI; confidence interval, T2DM;Type 2 Diabetes Miletus, HTN; Hypertension, HLD; Hyperlipidemia, CHF; Congestive Heart Failure. PBC; Primary Biliary Cholangitis. Figure 1.: Primary Biliary Cholangitis Associated Autoimmune Disorders. Univariate analysis used to calculate OR, OR; odds ratio, CI; confidence interval, PSC; Primary Sclerosing Cholangitis, AIH; Autoimmune Hepatitis, T1DM; Type 1 Diabetes Miletus, AIHA; Autoimmune Hemolytic Anemia, UC; Ulcerative Colitis, CD; Crohn's Disease, SLE; Systemic Lupus Erythematosus, ITP; Idiopathic Thrombocytopenia Purpura, AIT; Autoimmune Thyroiditis, RA; Rheumatoid Arthritis, MG; Myasthenia Gravis.

Alcohol intake is associated with a decreased risk of developing primary biliary cholangitis.

BACKGROUNDPrimary biliary cholangitis (PBC) is a chronic progressive liver disease of unknown aetiology characterised by immune-mediated destruction of small and medium-sized intrahepatic bile ducts. There are few well-established risk factors and epidemiological studies are needed to further evaluate the pathogenesis of the disease. AIMTo evaluate the relationship between alcohol intake, smoking and marijuana use with PBC development. METHODSWe conducted a prevalent case control study of 200 cases and 200 age (within a five year age band) and sex-matched controls, identified from the Victorian PBC prevalence study. We assessed lifetime alcohol intake and smoking behaviour (both tobacco and marijuana) prior to PBC onset and used conditional logistic regression for analyses. RESULTSAlcohol intake consistently showed a dose-dependent inverse association with case status, and this was most substantial for 21-30 years and 31-40 years (Ptrend < 0.001). Smoking was associated with PBC, with a stronger association with a longer duration of smoking [e.g., adjusted OR 2.27 (95%CI: 1.12- 4.62) for those who had smoked for 20-35 years]. There was no association between marijuana use and PBC. CONCLUSIONAlcohol appears to have an inverse relationship with PBC. Smoking has been confirmed as an environmental risk factor for PBC. There was no association between marijuana use and PBC.

THU463 - The prevalence of primary biliary cholangitis (PBC) is on the rise: a canadian population-based study

THU463 - The prevalence of primary biliary cholangitis (PBC) is on the rise: a canadian population-based study

Prevalence of Primary Biliary Cholangitis in a Cohort of Primary Sjögren's Syndrome Patients.

ObjectiveTo analyze the prevalence and clinical progression of primary biliary cholangitis (PBC) in patients with primary Sjögren's syndrome (pSS) and possible associations between biochemical and immunological features and the development of PBC.MethodsWe retrospectively reviewed a cohort of 115 pSS patients followed up in an outpatient clinic from 1987 to 2020, without a history of liver disease, and looked for the presence of PBC through analysis of several biochemical, immunological, and histologic characteristics.ResultsTwenty patients (17.4%) had chronic cholestatic liver biochemistry. After exclusion of extrahepatic liver disease by abdominal ultrasound, 13 of them were tested for antimitochondrial autoantibodies (AMA) detected by indirect immunofluorescence (IF), of which five tested positive, fulfilling the diagnostic criteria for PBC. Three of the five PBC patients and three of the eight chronic cholestasis AMA-negative patients were further investigated with liver biopsy, which showed features of PBC in all three PBC patients and in one of the chronic cholestasis AMA-negative patients, allowing for the diagnosis of AMA-negative PBC in the latter. The remaining two AMA-negative patients had liver histology compatible with autoimmune hepatitis and unspecific findings, respectively. Overall, six (5.2%) patients with pSS had AMA-positive PBC (n=5) or AMA-negative PBC (n=1). Comparing immunological characteristics between PBC and non-PBC patients, we found that PBC patients had a higher mean maximum erythrocyte sedimentation rate (ESR) during follow-up than patients without PBC. All PBC patients were treated with ursodeoxycholic acid (UDCA) and after treatment with UDCA, only one patient showed biochemical and clinical progression of PBC, with increasing alkaline phosphatase and total bilirubin levels, eventually progressing to cirrhosis.ConclusionsAmong patients with pSS, PBC had an overall prevalence of six of 115 (5.2%). Higher ESR was a feature associated with PBC patients. In our cohort, after initiation of UDCA treatment, PBC showed predominantly slow progress, with only one patient progressing to cirrhosis during follow-up.

99Increasing prevalence of primary biliary cholangitis in Victoria, Australia

Abstract Background The prevalence of primary biliary cholangitis (PBC) reported in different countries varies significantly and in some parts of the world appears to be increasing. The aim of this study was to determine the 2013 prevalence of PBC in Victoria, Australia, and to determine the time trend by comparing it with previous studies undertaken in 1991 and 2002. Methods Four case-finding methods were used to identify cases of PBC in Victoria: (1) physicians’ survey; (2) tertiary hospital search; (3) liver transplant database search; and (4) private pathology antimitochondrial antibody search. Results The prevalence of PBC in Victoria, Australia, is 189.0 per million using all four methods. The average annual increase in prevalence from 1991 to 2013 was 7.7 per million per year. Using the same case-finding methods as the 1991 Victorian prevalence study (methods 1 and 2), the prevalence of PBC increased from 19.1 per million in 1991 to 49.4 per million in 2002 (P &amp;lt; 0.001) and to 80.7 per million in 2013 (P &amp;lt; 0.001). Conclusions The current prevalence of PBC in Victoria is significantly higher than previously reported. The use of private pathology-based case-finding methods is important in identifying the maximum number of PBC cases. Key messages PBC prevalence in Victoria increased significantly since previous surveys, to 189 per million persons. Including pathology-based case ascertainment yields improved identification of cases.

Usefulness of Clinical and Laboratory Criteria for Diagnosing Autoimmune Liver Disease among Patients with Systemic Lupus Erythematosus: An Observational Study.

Abnormal liver function tests are frequently observed during follow-up of patients with systemic lupus erythematosus (SLE) but data on co-existence with autoimmune liver diseases (AILD) are scarce. This retrospective study aimed to describe the prevalence of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) among well-characterized subjects with SLE. We also evaluated whether the presence of autoantibodies to complement protein 1q (C1q) and/or ribosomal P protein (anti-ribP) are, directly or inversely, associated with AIH, as proposed in some reports. The number of screened patients was 287 (86% females), and all cases were included in a regional Swedish cohort. Each subject of the study population met the 1982 American College of Rheumatology classification criteria and/or the Fries’ diagnostic principle. By applying the simplified diagnostic AIH criteria combined with persistent transaminasemia, 40 (13.9%) cases reached at least “probable AIH”. However, merely 8 of these had been diagnosed with AIH (overall AIH prevalence 2.8%). Neither anti-C1q nor anti-ribP associated significantly with AIH. By applying the recent PBC guidelines, 6 (2.1%) cases were found, but only 3 of them had actually been diagnosed with PBC and one additional subject was not identified by the guidelines (overall PBC prevalence 1.4%). Compared to prevalence data from the general Swedish population, both AIH and PBC were highly overrepresented in our study population. The sensitivity of the diagnostic AIH criteria was impeccable but the specificity was less impressive, mainly due to positive ANA and hypergammaglobulinemia. Based on our findings, among subjects with SLE, the AIH criteria are less useful and liver biopsy combined with detection of other AILD-associated autoantibodies should be performed.

The Epidemiology of Primary Biliary Cholangitis in European Countries: A Systematic Review and Meta-Analysis.

Background Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic liver disease with wide ranges of reported incidence and prevalence. Aim To map the incidence and prevalence of PBC in European countries from 2000 through 2020. Methods Following PRISMA recommendations, we searched the Medline and Scopus databases for studies with information on either the incidence or prevalence of PBC. After data extraction, we used a random-effects model to estimate both the pooled annual incidence rate and pooled point-prevalence rate and performed subgroup analyses to identify components contributing to between-study heterogeneity. Results We performed a qualitative and quantitative analysis of 18 studies. The pooled point-prevalence rate was 22.27 cases per 100,000 inhabitants (95% CI: 17.98–27.01), and the pooled annual incidence rate was 1.87 new cases per 100,000 inhabitants (95% CI: 1.46–2.34). In the subgroup analyses, we proved that a small part of the between-study heterogeneity is significantly associated with a history of being part of the Eastern Bloc.

Identification of a Latitude Gradient in the Prevalence of Primary Biliary Cholangitis.

INTRODUCTION:The prevalence of primary biliary cholangitis (PBC) reported in different countries varies significantly and seems to have a latitudinal gradient with the highest prevalence reported in higher latitudes, as has been observed with other autoimmune diseases. This study aimed to determine whether there is a latitudinal gradient of PBC prevalence in Australia using 2 methods of case ascertainment.METHODS:We investigated the latitudinal variation of PBC prevalence across the states and territories of Australia (latitudinal range 18.0°–42.7°S) using pathology-based (private pathology antimitochondrial antibody results and PBC-specific prescription databases (prescriptions for ursodeoxycholic acid, the only publicly subsidized treatment for this disease).RESULTS:PBC prevalence was significantly positively associated with latitude, and the postcodes in the highest quintile of latitude (encompassing the south coastal areas of the Australian mainland and Tasmania; latitude range −37.75° to −42.72°) had a prevalence estimate that was 1.78 times higher using the pathology-based prevalence estimation than those in the lowest quintile (encompassing tropical and southern Queensland; latitude range −18.02° to −27.59°). Comparing prevalence estimates between states/territories, the result was 2.53 and 2.21 times higher in Tasmania compared with Queensland when using the pathology-based and prescription-based methods, respectively.DISCUSSION:Using 2 different case-ascertainment methods, we have demonstrated that prevalence estimates of PBC vary significantly with latitude in Australia. Further studies are needed to determine whether factors such as variations in ultraviolet radiation exposure and/or vitamin D levels are responsible for this observation and to investigate the latitudinal prevalence of PBC in other populations.

Primary biliary cholangitis

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an organ-specific autoimmune disease predominantly affecting middle-aged women. It does not occur in children. PBC prevalence varies depending on the geographic location of the country. Over the past 30 years, there has been an increased incidence of PBC, while significant progress has been made in understanding the pathogenesis of PBC due to the development of innovative technologies in molecular biology, immunology and genetics. The presence of antimitochondrial antibodies and cholestasis on biochemical analysis is sufficient to make a diagnosis, without the need for liver biopsy. Small- and medium-sized bile ducts are the targets of PBC. In the first stage of the disease, granulomatous destruction of the bile ducts occurs; in the second stage, loss of bile ducts, their proliferation, increased size of the portal tracts with chronic inflammation; in the third stage - fibrosis with septal formation, loss of bile ducts and cholestasis; in the fourth stage - liver cirrhosis. Previously, the survival rate of PBC patients ranged from 7.5 to 16 years. However, it has improved significantly with ursodeoxycholic acid and obeticholic acid treatment. If there is no effect from treatment and end-stage liver failure sets in, liver transplantation is performed.

Anti-mitochondrial Antibodies in Patients with Dilated Cardiomyopathy.

Objective It has been reported that anti-mitochondrial antibodies (AMAs) recognize mitochondrial antigens and are associated with some diseases involving multiple organs, such as primary biliary cholangitis, Sjögren syndrome, Hashimoto's thyroiditis, systemic sclerosis, interstitial pneumoniae, dilated cardiomyopathy, and tubulointerstitial nephritis. In the current study, we examined the prevalence of AMAs in patients with dilated cardiomyopathy (DCM) and their clinical characteristics. Methods We enrolled 270 patients with DCM. We measured serum AMAs and analyzed the associated factors. Out of the 270 patients, positive AMAs were detected in 3 patients (1.1%; mean age, 68 years old; 2 men). These three patients had a significantly higher prevalence of primary biliary cholangitis and myopathy and levels of alanine alkaline phosphatase than those who were negative for said antibodies. There were no significant differences in the levels of B-type natriuretic peptide, aspartate transaminase, and left ventricular ejection fraction between these groups of patients. During the follow-up period, two of the three patients died due to respiratory failure. The other patient survived but experienced type II respiratory failure. Conclusion The prevalence of AMAs in 270 DCM patients was only 1.1%, and these patients suffered from respiratory failure.

Primary Biliary Cholangitis and Type 1 Diabetes in A Male Patient: A Case Report

Primary Biliary Cholangitis (PBC) isa cholestatic disease of autoimmune origin characterized by the destruction of small to medium size intrahepatic bile ducts due to lymphocytic infiltration. The diagnosis is made by the presence of Anti-Mitochondrial Antibodies (AMAs) specific for the disease. PBC typically affects middle-aged women and is often associated with other autoimmune pathologies which are in decreasing order of frequency: Sjogren's syndrome, autoimmune dysthyroidism, scleroderma, rheumatoid arthritis, lupus and celiac diseasewhich are also more common in women. Few clinical studies evaluate the presentation of PBC in men with concomitant type 1 diabetes due to its low incidence compared to the prevalence of PBC in women.This case report presents that of a male patient with type 1 diabetes, admitted for cholestatic jaundice with pruritus, and in whom the diagnosis of PBC was only made late in the cirrhosis stage

Regional variation and temporal trend of primary biliary cholangitis epidemiology: A systematic review and meta-analysis.

We aimed to estimate the worldwide incidence and prevalence, with focus on the geographical differences and temporal trends. Studies on epidemiology of primary biliary cholangitis (PBC) in PubMed, Embase, and Cochrane Library were systematically retrieved from inception to October 2, 2020. Random-effect model was applied to estimate the pooled PBC incidence and prevalence rates. Subgroup analysis, meta-regression, and sensitivity analysis were conducted to find out the cause for heterogeneity. Out of 3974 records identified through database searching, 47 population-based studies were finally included. The pooled global incidence and prevalence of PBC were 1.76 and 14.60 per 100000 persons, respectively. Both the PBC incidence and prevalence were lower in the Asia-Pacific region (0.84, 9.82 per 100000 persons) than that in North America (2.75, 21.81 per 100000 persons) and Europe (1.86, 14.59 per 100000 persons) (P<0.05). The incidence and prevalence showed an increasing tendency in all three regions, with the fastest growth of prevalence in North America (P<0.05). We found a similar incidence and a lower prevalence of PBC in Northern Europe than that in Southern Europe. A higher incidence and prevalence were observed in female individuals and in the elderly (60-79). The PBC incidence and prevalence varied widely across regions, with North America being the highest, followed by Europe, and the lowest in the Asia-Pacific region. Both the incidence and prevalence showed an increasing tendency worldwide, especially in North America.

Geo-epidemiology and environmental co-variate mapping of primary biliary cholangitis and primary sclerosing cholangitis

Background & AimsAutoimmune liver disease (AILD) is thought to result from a complex interplay between genetics and the environment. Studies to date have focussed on primary biliary cholangitis (PBC) and demonstrated higher disease prevalence in more urban, polluted, and socially deprived areas. This study utilises a large cohort of patients with PBC and primary sclerosing cholangitis (PSC) to investigate potential environmental contributors to disease and to explore whether the geo-epidemiology of PBC and PSC are disease-specific or pertain to cholestatic AILD in general.MethodsAll adult patients with PBC and PSC in a tightly defined geographical area within the UK were identified. Point- and area-based analyses and structural equation modelling (SEM) were used to investigate for disease clustering and examine for relationships between prevalence, distribution of environmental contaminants, and socio-economic status.ResultsWe identified 2,150 patients with PBC and 472 with PSC. Significant spatial clustering was seen for each disease. A high prevalence of PBC was found in urban, post-industrial areas with a strong coal-mining heritage and increased environmental cadmium levels, whereas a high PSC prevalence was found in rural areas and inversely associated with social deprivation.ConclusionsThis study demonstrates spatial clustering of PBC and PSC and adds to our understanding of potential environmental co-variates for both diseases. Disease clustering, within the same geographical area but over different scales, is confirmed for each disease with distinct risk profiles identified and associations with separate putative environmental factors and socio-economic status. This suggests that different triggers and alternative pathways determine phenotypic expression of autoimmunity in the affected population. Co-variate analysis points towards the existence of specific disease triggers.Lay summaryThis study looked for potential environmental triggers in patients with primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) living in the north-east of England and north Cumbria. We found that PBC was more common in urban areas with a history of coal mining and high levels of cadmium whereas PSC was more common in rural areas with lower levels of social deprivation.

Epidemiology and clinical course of primary biliary cholangitis in Eastern Slovakia.

To determine both the incidence and the prevalence of primary biliary cholangitis (PBC) in Eastern Slovakia and to describe its clinical course and the response to ursodeoxycholic acid (UDCA). We recorded data of patients with PBC, who were followed up in gastroenterology and hepatology centers in Eastern Slovakia during the period from June 30, 1999, through June 30, 2019. The annual incidence of PBC varied from 0.7 to 1.5 cases per 100,000 inhabitants between 2014 and 2018. PBC prevalence steadily increased from initial 10.2 cases per 100,000 inhabitants in 2014 to 14.9 cases per 100,000 inhabitants in June 2019. The mean age at the time of diagnosis was 56.3 ± 10.9years. 95.7% of patients were females, and female/male gender ratio was 22.3:1. In June 2019, prevalence in the female population was 28 cases per 100,000 women. At the time of diagnosis, three-quarters of patients were symptomatic and 10% of patients had liver cirrhosis. The mean follow-up was 7.3 ± 5.2years. Response to UDCA was observed in 72.1% of patients. Patients with higher baseline alkaline phosphatase (ALP) levels, liver cirrhosis at entry or at the end of follow-up and women younger than 45years responded worse to UDCA. One-quarter of patients had liver cirrhosis at the end of follow-up. During follow-up, 1.6% of patients underwent liver transplantation and 5.5% of patients died. Five-year and 10-year liver-related mortalities were 2.7% and 4.3%, respectively. PBC prevalence in Eastern Slovakia is increasing, and most of the patients respond to therapy with UDCA.