Primary Biliary Cholangitis Cases Research Articles

The future risk of primary biliary cholangitis (PBC) is low among patients with incidental anti-mitochondrial antibodies but without baseline PBC.

Anti‐mitochondrial autoantibodies (AMAs) are highly specific for the diagnosis of primary biliary cholangitis (PBC) but are also occasionally found in other diseases. In the present study, we evaluated the incidence of and predictors for PBC development in AMA‐positive patients with other liver or non‐liver diseases at baseline. In this retrospective study, we screened patients who tested positive for AMA and/or anti‐mitochondrial M2 antibody (AMA‐M2) at Beijing Friendship Hospital, Capital Medical University, from October 2005 to January 2017. They were categorized by their diagnosis at the baseline as patients with PBC or non‐PBC cases. We followed up on the non‐PBC cases through telephone interviews and reviewing of medical records to obtain laboratory results and clinical outcomes. In total, 139 patients were AMA‐positive but did not fulfill the diagnostic criteria of PBC at baseline, including 51 patients with non‐PBC liver diseases and 88 cases with non‐liver diseases. The titers of AMA‐M2, alkaline phosphatase, gamma‐glutamyl transpeptidase, and immunoglobulin M were significantly higher in patients with PBC compared to those with non‐PBC liver diseases and non‐liver diseases. After a median follow‐up of 4.6 (interquartile range: 2.4–7.6) years, 4.3% (6 of 139) developed PBC, with an accumulative 5‐year incidence rate of 4.2%. None of the patients with non‐PBC liver diseases developed PBC, whereas the 5‐year incidence rate of PBC was 7.8% among 88 patients with non‐liver diseases. Lower alanine aminotransferase and higher immunoglobulin M were independent predictors for developing PBC. Conclusion: Our results suggest a low risk of developing PBC over time in AMA‐positive patients with other liver and non‐liver diseases.

Far From the Biliary Tree: A Case of Overlapping Autoimmune Liver Disease in a Patient Presenting With Sicca Symptoms

Primary biliary cholangitis (PBC) is a chronic autoimmune condition with many extrahepatic manifestations that are commonly encountered as a patient's primary presenting complaints. Rarely, PBC co-exists as an “overlapping syndrome” with other liver-related autoimmune conditions such as autoimmune hepatitis (AIH). Presented is a rare case of PBC with features of AIH diagnosed in a patient who initially presented with hemoptysis and worsened sicca symptoms due to advanced Sjögren’s syndrome. The patient had a three-year evolution of abnormal liver biochemistry and was found to be a heterozygous carrier for hereditary hemochromatosis (H63D mutation). Given that patients with PBC-AIH are at an increased risk of complications compared to isolated disease from either disorder, early diagnosis and prompt management can help spare patients from cirrhosis, liver failure and transplantation, or even death.

Diagnostic and Clinical Value of Specific Autoantibodies against Kelch-like 12 Peptide and Nuclear Envelope Proteins in Patients with Primary Biliary Cholangitis.

Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by the presence of antimitochondrial and antinuclear antibodies in patients’ serum. Here, we analyzed the reactivity of autoantibodies against a novel autoantigen, kelch-like 12 (KLHL12) protein, in a cohort of 138 PBC and 90 non-PBC patients. Additionally, we compared the reactivity of KLHL12 with antinuclear envelope antibodies: anti-gp210, anti-p62, and anti-LBR. Commercially available kits and an ‘in-house’ ELISA were used in the studies. Antinuclear envelope antibodies were detected in 65% of PBC patients and the presence of these antibodies was observed more frequently in patients diagnosed with later stages (III/IV) of PBC, according to Ludwig’s classification (p < 0.05) and were found to correlate with a higher concentration of bilirubin. Overall, anti-KLHL12 antibodies were found more frequently in PBC patients than in non-PBC controls (p < 0.001). Anti-KLHL12 antibodies were detected in 36% of the tested PBC cohort, including PBC patients negative for antimitochondrial antibodies. Presence of anti-KLHL12 was also associated with a higher concentration of bilirubin and correlated with fibrosis (p < 0.05). Anti-KLHL12 antibodies were detected in 30% of PBC individuals positive for antinuclear envelope antibodies, while anti-KLHL12 and antinuclear envelope antibodies were found in 17% of all PBC cases. Concluding, our data confirm that antibodies against the KLHL12 protein are highly specific for PBC and when used in combination with other markers, may significantly increase the diagnosis of PBC.

Primary biliary cholangitis first demonstrated in pregnancy: a\xa0case report

BackgroundWe present a case of primary biliary cholangitis diagnosed during pregnancy. Diagnosis of this entity in pregnancy is infrequent, and when everything seemed to point to a simple obstetric cholestasis, close attention to the details of the clinical history was required to raise suspicion of the true diagnosis.Case presentationWe present a 37-year-old Portuguese Caucasian patient who complained of generalized pruritus and showed alteration in hepatic function tests with a cholestatic pattern. The first diagnostic hypothesis was intrahepatic cholestasis of pregnancy, and she began treatment with ursodeoxycholic acid, which resulted in slight improvement of cholestasis. Her pregnancy was also complicated with occlusive hemorrhagic placenta, and at 30 weeks she underwent emergency cesarean section due to heavy blood loss. However, careful observation of clinical and laboratory findings, postpartum evolution, and a multidisciplinary approach to the patient led to the probable diagnosis of primary biliary cholangitis.ConclusionsPhysiological changes during pregnancy can mimic chronic liver disease that can only be revealed at this stage, having an impact not only on the pregnancy but on the entire future of the woman.

Severe acute respiratory syndrome coronavirus-2-associated cholangiopathies.

Purpose of reviewSARS-CoV2 is a β-coronavirus, isolated for the first time in Wuhan in December 2019. Bilateral interstitial pneumonia is the hallmark of this disease. Liver is the second viral target for frequency and AST and ALT elevation is a common finding. From February 2020, two different cholangiopathies have been reported in COVID-19 patients. The aim of this article is to review the cases so far described in order to share information and awareness about these new clinical entities.Recent findingsSARS-CoV2 seems to trigger autoimmunity and two cases of primary biliary cholangitis (PBC) have been developed after viral infection while more than 30 patients have showed a rapidly progressing cholangiopathy with features of secondary sclerosing cholangitis (SSC). For what concerns SSC pathogenesis, a theory combining multiple hits is the most recognized.SummaryTwo different cholangiopathies have been reported in patients after severe-COVID-19. Attention should be paid to the development of cholestasis in ICU setting but above all after discharge and liver function tests should be, therefore, periodically performed. No treatment strategies are available and liver transplantation remains the last option in individuals with liver failure because of SSC. Other efforts are necessary to better understand the pathogenesis and to expand therapeutic options.

The Spectrum of Autoimmune Liver Disorders, Clinical Presentation, and Autoantibodies in Patients From a Tertiary Care Center in Pakistan.

BackgroundThe autoimmune illnesses that affect the liver include autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and overlap syndrome. In our patients, we aimed to address the complete spectrum of autoimmune liver disorders, clinical presentation, and autoantibodies.MethodsThe study included all the patients diagnosed with autoimmune liver disorder irrespective of age, gender, and ethnic background presented at the liver clinic of the hospital in the last two years. The diagnosis was based on characteristic clinical and laboratory findings, the presence of one or more characteristic autoantibodies, and/or histological abnormalities. The diagnosis of AIH was further validated by revised International AIH Group criteria using a scoring calculator. The diagnostic criteria for PBC required the presence of chronic elevation of alkaline phosphatase (ALP) with positive antimitochondrial antibody (AMA) or positive PBC-specific anti-nuclear antibodies (ANA) (sp-100, gp-210) tests and/or compatible histology. The patients of AIH-PBC overlap syndrome fulfilled the criteria for AIH in the setting of PBC. Patients having liver involvement in other autoimmune disorders were included in the study.ResultsThe total number of patients was 124; 83 (67%) were females; mean age ± standard error of mean (SEM) was 44.97 ± 1.47 years with a range of 09-84 years. Type-1 AIH was seen in 68 (54.8%) patients, type-2 AIH in 10 (8.1%) patients, PBC in 22 (17.7%) patients, overlap of PBC with AIH in 10 (8.1%) patients, IgG4 disease in four (3.2%) patients, psoriasis-specific immune hepatitis in four (3.2%) patients, celiac disease-related hepatitis in three (2.4%) patients, sarcoidosis in two (1.6%) patients, and ichthyosis-associated hepatitis in one (0.8%) patient. There was a high prevalence of cirrhosis (50%) at the time of presentation; 19% of patients had decompensated liver disease. ANA was positive in 52/68 cases of AIH type-1, but anti-smooth muscle antibody (ASMA) was reactive only in nine cases and anti-soluble liver antigen (SLA) in five cases. There was no female preponderance in type-2 AIH (M:F = 6:4). AMA was reactive in 25 (78%) cases of PBC and overlap syndrome. Antibodies prevalent in PBC (AMA-M2, AMA-M2-3E, sp-100, gp-210, anti-Ro52) were also seen in some cases of AIH, though they did not fulfill the criteria of the overlap syndrome.ConclusionThere is an unmet need for the early diagnosis of autoimmune liver diseases and the initiation of appropriate management to prevent complications.

The liver steatosis severity and lipid characteristics in primary biliary cholangitis

BackgroundPatients with primary biliary cholangitis (PBC) often have comorbid dyslipidemia, and determining the degree of hepatic steatosis can help predict the risk of cardiovascular events in PBC patients. The aim of our study was to analyze the characteristics of lipid distribution and the degree of hepatic steatosis in PBC.MethodsWe retrospectively analyzed 479 cases of PBC, chronic hepatitis B (CHB), chronic hepatitis C (CHC), non-alcoholic fatty liver disease (NAFLD), and healthy subjects (Normal) diagnosed by liver biopsy or definitive clinical diagnosis. Controlled attenuation parameter (CAP) values were applied to assess the degree of steatosis of the liver, and lipid levels were also compared in the five cohorts.ResultsWe found that among the five groups of subjects, the PBC group had the lowest CAP values (P < 0.001), and the high-density lipoprotein cholesterol (HDL-C) level in the PBC group was higher than normal, CHC and CHB group (P = 0.004, P = 0.033, P < 0.001, respectively).In the multivariate linear analysis, only BMI (β = 1.280, P = 0.028), ALP (β = − 0.064, P = 0.012), TBA (β = − 0.126, P = 0.020), TG (β = 12.520, P = 0.000), HDL-C (β = − 11.338, P = 0.001) and LDL-C (β = 7.012, P = 0.002) were independent predictors of CAP.ConclusionsAmong PBC, CHB, CHC, NAFLD and healthy subjects, PBC had the lowest degree of hepatic steatosis and higher HDL-C levels, all of which were found to be protective factors against atherosclerosis and cardiovascular risk and would provide a valuable reference for the risk of developing cardiovascular events in PBC patients.

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.

From the Editor’s Desk…

From the Editor’s Desk…

Primary Biliary Cholangitis: Predictors of Poor Response to Ursodeoxycholic Acid after 1 Year of Treatment in Moroccan Patients

Introduction: Primary biliary cholangitis (PBC), the new dominance of primary biliary cirrhosis, is a cholestatic disease of autoimmune etiology and represents the leading cause of intra-hepatic cholestasis. Treatment is mainly based on ursodeoxycholic acid. The biological response to treatment is the main predictor of survival without liver transplantation. The Globe-score has been recently validated as the main prognostic factor. Materials and methods: This is a retrospective study carried out in our department collating all cases of PBC followed in consultation. The aim of our work is to research the predictors of poor response to UDCA. Results: 46 patients were collected. The mean age of the patients was 58.82 years, with a predominance of women (n = 43, 93.5%). 34.78% of patients were in the stage of cirrhosis. Anti-M2 mitochondria antibodies were positive in 44 patients (95.65%). An overlap syndrome was found in 11 patients (23.9%). Treatment was based on UDCA combined with corticosteroid therapy and immunosuppressant for overlap syndrome. A biochemical response at 1 year of treatment according to the Paris II criteria was found in 47.8%. The average value of the globe score was 1.35. A score greater than 0.30 was objectified in 20 cases (43.47%). Nineteen cirrhotic patients (41.30%) had a globe score&gt; 0.30. Factors associated with poor response to therapy were: stage of decompensated cirrhosis, elevated pre-therapy total bilirubin greater than 30 g / l and hypoalbunemia less than 35 g / l. The study of the correlation between Globe score and Paris II showed a strong and significant association with a correlation coefficient estimated at 67%. The Paris II score was significantly correlated with the response to treatment (p = 0.001). Conclusion: In accordance with the data in the literature, the globe-score and Paris II are two similar predictive means for evaluating the response at 1 year of treatment in Moroccan context. Keywords: Morocco, Predictors of response, Primary biliary cholangitis, Ursodeoxycholic acid

Promyelocytic Leukemia Antigen Expression: a Histological Marker for Primary Biliary Cholangitis Diagnosis?

Background and ObjectivesDistinguishing primary biliary cholangitis (PBC) from other cholestatic diseases at the histological level could be assisted by new methods, such as immunohistochemical staining of specific antigens.MethodsWe evaluated whether the detection of promyelocytic leukemia protein (PML) can serve as a specific and sensitive marker for PBC diagnosis. Liver biopsies from 26 PBC patients, 20 primary sclerosing cholangitis (PSC), 37 viral hepatitis, 11 non-alcoholic steatohepatitis (NASH) and 5 normal patients were investigated after immunostaining with the anti-PML monoclonal PG-M3, IgG1 antibody.ResultsImmunoreactivity in bile ducts was expressed by the PML-score (quotient of positive ducts to the total number of portal tracts multiplied by 2). PML-score was higher in PBC as compared to controls (P < 0.001). Using a cutoff of 0.18, PML-score proved highly sensitive (84.6%) and specific (89.7%) for confirming PBC as compared to only 5% of PSC, 9.1% of NASH and 13.5% of viral hepatitis patients (P < 0.001). Irrespective of the underlying disease, patients with PML-score > 0.18 were older (P = 0.007), more often females (P < 0.001) with higher ALP (P < 0.001), γ-GT (P = 0.001) and IgM (P < 0.001) compared to the patients with PML-score < 0.18.ConclusionsWe postulate that a simple PML immunohistochemical test could be sufficient for histopathological discrimination of PBC in problematic cases of undefined cholestatic disorders, including small-duct PSC and AMA-negative PBC cases.

Two cases of primary biliary cholangitis associated with autoimmune hemolytic anemia

1例目は71歳，女性．動悸を主訴に当科を紹介受診した．血色素7.7 g/dl，ハプトグロビン10 mg/dl以下，Coombs試験陽性から自己免疫性溶血性貧血（AIHA）と診断された．プレドニゾロン（prednisolone，PSL）投与が著効したが，AIHAの診断後1年8カ月経過した頃よりALP，γGTPの上昇ならびに抗ミトコンドリアM2抗体の高値を示し，肝組織検査で中沼分類stage 2の原発性胆汁性胆管炎（PBC）と診断された．2例目は67歳，男性．褐色尿を主訴に当科を紹介受診した．血色素が10.3 g/dl，ハプトグロビンが10 mg/dl以下，Coombs試験が陽性であり，AIHAと診断された．1例目同様にPSLが著効したが，初診時からの持続的なALP，γGTPの上昇ならびに抗ミトコンドリアM2抗体の高値を示し，肝組織検査で中沼分類stage 2のPBCと診断された．2症例ともにウルソデオキシコール酸が奏効し，肝胆道系酵素は正常化した．自己免疫性疾患であるPBC，AIHAの合併例は稀であり，発症機序，治療について考察した．

The Epidemiology of UK Autoimmune Liver Disease Varies With Geographic Latitude

Background & AimsThe epidemiology of autoimmune liver disease (AILD) is challenging to study because of the diseases’ rarity and because of cohort selection bias. Increased incidence farther from the Equator has been reported for multiple sclerosis, another autoimmune disease. We assessed the incidence of primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and autoimmune hepatitis (AIH) in relation to latitude.MethodsWe performed a retrospective cohort study using anonymized UK primary care records from January 1, 2002, to May 10, 2016. All adults without a baseline diagnosis of AILD were included and followed up until the first occurrence of an AILD diagnosis, death, or they left the database. Latitude was measured as registered general practice rounded down to whole degrees.ResultsThe cohort included 8,590,421 records with 53.3 × 107 years of follow-up evaluation from 694 practices. There were 1314 incident cases of PBC, 396 of PSC, and 1034 of AIH. Crude incidences were as follows: PBC, 2.47 (95% CI, 2.34–2.60); PSC, 0.74 (95% CI, 0.67–0.82); and AIH, 1.94 (95% CI, 1.83–2.06) per 100,000 per year. PBC incidence correlated with female sex, smoking, and deprivation; PSC incidence correlated with male sex and non-smoking; AIH incidence correlated with female sex and deprivation. A more northerly latitude was associated strongly with incidence of PBC: 2.16 (95% CI, 1.79–2.60) to 4.86 (95% CI, 3.93–6.00) from 50°N to 57°N (P = .002) and incidence of AIH: 2.00 (95% CI, 1.65–2.43) to 3.28 (95% CI, 2.53–4.24) (P = .003), but not incidence of PSC: 0.82 (95% CI, 0.60–1.11) to 1.02 (95% CI, 0.64–1.61) (P = .473). Incidence after adjustment for age, sex, smoking, and deprivation status showed similar positive correlations for PBC and AIH with latitude, but not PSC. Incident AIH cases were younger at more northerly latitude.ConclusionsWe describe an association in the United Kingdom between more northerly latitude and the incidence of PBC and AIH that requires both confirmation and explanation.

Cases of liver disease lost in the health system: a call to action.

We recently reported our experience searching for patients with primary biliary cholangitis (PBC) who were lost in the health system. Our search of databases in 4 hospitals, 3 of which were tertiary institutions, revealed that sufficient data were available to ensure a reliable diagnosis (i.e., cholestasis, positive antimitochondrial antibody titer, and no other liver disease) in 14.3 % (100/697) of PBC cases. However, patients remained undiagnosed, thus missing an opportunity to start early treatment and follow-up. Even more strikingly, of 30 rescued patients, 27 % had an elastography result indicating a poorer prognosis (> 9.5 kPa).

Primary biliary cholangitis (PBC) - autoimmune hepatitis (HAI) overlap syndrome (OS)

The AIH-PBC overlap syndrome is the most common form of OS. The aim: to study the clinical and paraclinical characteristics of the overlap syndrome and to specify the therapeutic and evolutionary characteristics. Methods: The medical data of 42 PBC patients were evaluated for associated autoimmune hepatitis. Results: Among the 42 cases of PBC, we retained 16 cases of OS (37%). All female the association of diagnostic criteria for PBC and HAI was simultaneous in 80%. Treatment with AUDC, corticosteroid and Azathioprine was started in the majority of our patients. The course was marked by a complete (10 cases) or incomplete (n = 6) response. Conclusion: OS should be considered in the presence of any mixed cytolytic and cholestatic liver disease. Its recognition is essential in order to adapt treatment and prevent progression to cirrhosis and its complications.

Clinical characteristics of patients with autoimmune liver disease complicated with gallbladder stone

Objective To explore the clinical characteristics of liver function of patients with autoimmune liver disease (AILD) complicated with gallbladder stone (GS), so as to guide clinical practice. Methods From November 2009 to October 2018, at General Hospital of Tianjin Medical University, the clinical data of 386 patients with AILD were retrospectively analyzed. According to the relevant diagnostic criteria, 208 cases of autoimmune hepatitis (AIH), 129 cases of primary biliary cholangitis (PBC) and 49 cases of PBC-AIH overlap syndrome were screened out. The incidence, clinical characteristics and the changes of laboratory indicators including albumin, alkaline phosphatase (ALP) and γ-glutamyl transferase (GGT) of AILD patients complicated with GS were analyzed. Chi-square test, t test and rank sum test were performed for statistical analysis. Results There was no significant difference in the incidence between AILD, AIH, PBC and PBC-AIH overlap syndrome patients complicated with GS (32.9%, 127/386; 28.8%, 60/208; 36.4%, 47/129 and 40.8%, 20/49; respectively; P>0.05). Gallstones of AILD patients complicated with GS mostly were multiple and small stones with maximum diameter <1 cm (45.7%, 58/127 and 57.7%, 60/104, respectively). The age of initial diagnosis, the proportion of liver cirrhosis at inital diagnosis and the levels of ALP and GGT were higher in AILD patients complicated with GS than those of AILD patients without GS ((60.5±11.5) years vs. (57.6±11.5) years; 53.5%, 68/127 vs. 42.1%, 109/259; 154.00 U/L (89.00 U/L, 257.00 U/L) vs. 125.00 U/L (86.00 U/L, 212.00 U/L); 169.00 U/L (79.00 U/L, 343.00 U/L) vs. 128.60 U/L (48.00 U/L, 284.00 U/L); respectively); however the albumin level was lower than that of AILD patients without GS ((36.46±7.30) g/L vs. (38.34±7.58) g/L), and the differences were statistically significant (t=-2.361, χ2=4.506, Z=-2.192, -2.443, t=2.322; all P<0.05). The incidence of GS in AILD patients≥60 years old was higher than that AILD patients<60 years old (37.6%, 73/194 vs. 28.1%, 54/192), and the difference was statistically significant (χ2=3.948, P=0.047). The incidence of GS in AILD patients and AIH patients complicated with liver cirrhosis was higher than that in patients without liver cirrhosis (38.4%, 68/177 vs. 28.2%, 59/209; 35.7%, 35/98 vs. 22.7%, 25/110; respectively), and the differences were statistically significant (χ2=4.506 and 4.259, P=0.034 and 0.039). Conclusions AILD patients complicated with GS are common, most are multiple and small stones. When complicated with GS, the initial diagnosis may be delayed and the rate of liver cirrhosis at initial diagnosis may increase. The incidence of GS is high in AILD patients with older age and liver cirrhosis. Key words: Liver cirrhosis; Autoimmune liver disease; Gallbladder stone; Liver function; Age

Cost of Illness of Primary Biliary Cholangitis in Lombardia

The burden of Primary Biliary Cholangitis (PBC) has never been characterised at population-level. We aimed to estimate the cost of illness of PBC in Lombardy Region, Italy. PBC cases were defined as individuals with at least a single episode of hospitalization attributed to code 571.6 (ICD–9 – Biliary Cirrhosis) or with a disease exemption code 008.571.6, (Biliary Cirrhosis) from year 2007 to year 2017. Case finding was further refined excluding patients not receiving ursodeoxycholic acid and those treated with antivirals for hepatitis B and C. Data are representative of the context of Lombardia Region (located in Northern Italy, with a population of about 10 million inhabitants) and costs are calculated assuming the perspective of Regional Health Service. Only health services considered clinically related to the PBC diagnose were considered, and direct costs were estimated only for services related to PBC in 2017 and subdivided as outpatient and inpatient activities. The point of view assumed in the analysis is that of the Regional Health Service of Lombardia Region. We identified 970 adult patients, 810 (83.5 %) were females, with a mean age of 61 years. Direct medical costs in 2017 were equal to € 916,763, with € 459,506 (50.3 %) deriving from inpatient activities. These were mostly due to liver transplant (30.5 %) and cirrhosis complication (20.6 %). Costs from outpatient activities were equal to € 109,090 (11.9 %). Of note, only 52.8 % were prescribed to perform biochemicals tests: of those 72 % had alkaline phosphatase checked. Cost of drugs used to manage was € 345,167 (37.7 %). The main direct costs component for PBC are inpatient activities due to the progression of disease, which might be potentially reduced as a result of the adoption of new treatments. The analysis of outpatient's activities suggests an inadequate monitoring activity of patients affected by PBC.

Increasing prevalence of primary biliary cholangitis in Victoria, Australia.

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. 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. 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<0.001) and to 80.7 per million in 2013 (P<0.001). 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.

Low rate of new-onset primary biliary cholangitis in a cohort of anti-mitochondrial antibody-positive subjects over six years of follow-up.

Background and AimsAnti‐mitochondrial antibodies (AMA) are closely linked to primary biliary cholangitis (PBC). The prevalence of AMA in the general population is low, and AMA positivity may precede PBC. We aimed to determine the natural history of subjects with positive AMA.MethodsIn total, 302 patients were tested AMA‐positive over a ten‐year period. Of these, immunoblotting confirmed specific AMA in 184 (29 male, 155 female, age 59.6 ± 14.1 years). These subjects were invited to our liver outpatient clinic for clinical and biochemical re‐evaluation. Detailed clinical history data were additionally collected from the hospital computer system and by telephone. The subsequent course with regard to mortality, liver‐related morbidity, extrahepatic co‐morbidities and effectiveness of PBC treatment was determined in 150 subjects (81.5%).ResultsAfter 5.8 ± 5.6 years of follow‐up (FU), of 184 AMA‐positive subjects, 28 subjects (15.2%; liver‐related mortality n = 5) were deceased, and 122 subjects (66.3%) completed FU while 34 subjects (18.5%) were not available for FU. The 122 patients who completed FU were 63 patients with established PBC, six de novo cases of PBC (10.2% of 59 initially at risk), 42 (34.4%) subjects were still AMA‐positive without PBC, and 11 (9.0%) subjects were AMA‐negative at FU.ConclusionsAnti‐mitochondrial antibodies‐positive patients without PBC at baseline infrequently developed PBC over six years of FU. AMA positivity represented a transient serological autoimmune phenomenon in a significant proportion of subjects.

Situation analysis of timing of first visit of anti-mitochondrial antibody-positive patients

Objective: To understand the basic information of anti-mitochondrial antibody (anti-AMA)-positive patients after initial diagnosis, and to set groundwork for further exploring the clinical significance of AMA in various diseases. Methods: Demographic data and related clinical information recorded through the Information System of Peking University People's Hospital from January 2013 to December 2016 were collected. Patients whose AMA and/or AMA-M2 first- tested as positive were recorded. Complications were classified according to the International Classification of Diseases. Results: A total of 1323 AMA positive cases were discovered for the first time. Among them, 78.0% were women, and the age of initial diagnosis was 56.8 ± 16.0 years. The first three initially diagnosed departments were rheumatology and immunology (37.4%), liver Disease (15.9%) and hematology (15.9%) relevant to musculoskeletal and connective tissue diseases (45.2%), hematology and hematopoietic organs and immune diseases (30.6%) and circulatory system diseases (29.7%). There were 297 newly confirmed cases of primary biliary cholangitis (PBC); accounting for 89.2% of women, and the age of initial diagnosis was 60.1 ± 12.4 years. The top three departments of initially diagnosed as PBC were liver disease (37.7%), rheumatology (33.0%) and gastroenterology (15.2%), of which 39.7% had musculoskeletal and connective tissue diseases, 27.9% had circulatory diseases, and 24.9 % were combined with endocrine and metabolic diseases. Conclusion: Besides PBC and other autoimmune diseases, AMA and / or AMA-M2 positivity can be observed in a variety of diseases in several clinical departments, and its clinical significance remains to be further clarified.