Prevalence Of Primary Biliary Cirrhosis Research Articles

COMPARATIVE CHARACTERISTICS OF AMAPOSITIVE AND AMA-NEGATIVE PRIMARY BILIARY CHOLANGITIS

Primary biliary cholangitis (PBC, formerly known as primary biliary cirrhosis) is one of the most significant diseases that is predominantly verified in women. The prevalence of PBC is 1.9-40.2 per 100,000 population. Antimitochondrial antibodies (AMA) are of major importance for the diagnosis of primary biliary cirrhosis (PBC), and it has also been suggested that they may be involved in the pathogenesis of the disease. Objective: The aim of this study was to characterize the clinical, biochemical parameters and response to UDCA (Paris I criteria) PBC patients depending on AMA status in the Republic of Kazakhstan. Material and methods. The study was conducted on the basis of the Research Institute of Cardiology and Internal Diseases from 2014 to 2019. A total of 212 patients with primary biliary cholangitis were recorded. Results. Among 212 patients, 171 (80.7%) were AMA-positive and 41(19,3%) - AMA-negative. Vast majority of patients in both groups were Asian (179; 84.4%) and female (206; 97.2%). Severe disease (F3/4) was revealed in 108 (63.2%) AMA-positive and in 18 (43.9 %) AMA-negative patients (p>0.05). AMA-positive and AMA-negative PBC were associated with autoimmune hepatitis in 88 (51.5%) and 10 (24.4%) cases (p<0.01), rheumatoid arthritis in 26 (15.2%) and 5 (12.2%), autoimmune thyroiditis in 35 (20.5%) and 4 (9.8%), vitamin D deficiency in 89 (52.1%) and 19 (46.3%), osteoporosis in 48(28.1%) and 7 (17.1%), gallstone disease in 43 (25.4%) and 8 (21.2%) respectively (p >0.05). Response to ursodeoxycholic acid (UDCA) treatment according to Paris I criteria was noted in 24 out of 81 (29.6%) AMA-positive and in 9 out of 22 (40.9%) AMA-negative patients (p>0.05). Conclusion. In patients with AMA-positive PBC compared with AMA-negative, there is a statistically significant high value of autoimmune hepatitis and autoimmune thyroiditis. Whereas, response to UDCA treatment according to Paris I criteria is higher in AMA-negative PBC.

The new epidemiology of primary biliary cirrhosis.

Primary biliary cirrhosis (PBC) is an autoimmune cholestatic liver disease. Susceptibility to PBC probably arises from a combination of genetic and environmental factors. The prevalence of PBC varies both on an international and a regional level. This can be explained, in part, by differences in clinical practice and case-finding activity. It is likely, however, that substantive geographical differences exist both in terms of genetic susceptibility and environmental factors that potentially trigger the disease in genetically susceptible individuals. The study of the epidemiology of PBC has strongly supported the concept of an environmental triggering factor, but as yet no specific agent has been identified. Ongoing work to discover the environmental agent, as well as the mechanism that causes the disease will answer key questions as to the epidemiology of this complex autoimmune disease as well as providing useful information for other autoimmune conditions.

Geoepidemiology and space–time analysis of Primary biliary cirrhosis in Crete, Greece

The prevalence of Primary biliary cirrhosis varies in different geographical areas. This might reflect genetic or environmental risk factors. We aimed to define Primary biliary cirrhosis prevalence and incidence, describe patient's spatial distribution, generate prediction maps and detect any possible routing pattern of time-spatial appearance of the disease in Crete, Greece. From 1990-2010, 245 Primary biliary cirrhosis patients diagnosed and followed up at the Gastroenterology Department of the University Hospital and the District Hospitals of the island, were contacted and 222 were included in the time-spatial analysis. To map their spatial distribution per 5-year periods, geospatial models were applied in Gis-ArcMap 9.3 software. Kriging Interpolation methods were used to generate prediction maps for the disease in Crete. Areas of high and low probability of disease occurrence were estimated through multicriteria modelling. The disease route was defined by Gis-ArcMap's toolbox. Prevalence was found to be 365 cases per million, with a mean incidence of 20.88 (range 3.79-35.99). Prediction map estimates from 1.22 to 11 patients per 50 km2 all over Crete. Areas of high risk of disease occurrence are located in the Eastern part, while low risk in the Western part of the island. Prevalence and incidence of Primary biliary cirrhosis in Crete are among the higher published in Europe. Given the homogeneous and stable study population and the geopolitics of the island, the heterogeneity in the time-spatial distribution and the route of disease appearance strongly suggest a role for environmental causative agents.

Rising incidence and prevalence of primary biliary cirrhosis: a large population‐based study

Large population-based studies are much needed to accurately establish the epidemiology of primary biliary cirrhosis (PBC). We aimed to collect all PBC patients in a geographically defined area to evaluate the epidemiology of PBC and examine the possible association of PBC with smoking, age at menarche, age at first pregnancy and number of pregnancies. All PBC patients between 2000 and 2008 were identified in a geographically defined area of the Netherlands, comprising 50% of the Dutch population. Four independent hospital databases were searched in 44 hospitals. Medical records were reviewed on site verifying diagnosis and for collection of clinical data. Age- and gender matched controls were recruited from the outpatient clinics of four participating hospitals. Patients and controls were asked to fill out a questionnaire regarding family history, previous and current smoking behaviour and fertility status. Nine hundred and ninety-two PBC patients fulfilled all inclusion criteria, resulting in a mean incidence of 1.1 per 100000; 0.3 in men and 1.9 in women. On January 1st 2008 the point prevalence was 13.2 per 100000 inhabitants. Incidence and prevalence rates were increasing over time (P<0.001). No geographical differences in disease distribution were observed. Smoking behaviour, age at menarche, age at first pregnancy, gravidity and number of children were not significantly different between cases and controls. Incidence and prevalence rates of PBC are increasing over time. PBC was not found to be associated with smoking, age at menarche, age at first pregnancy or number of pregnancies.

THU0190 Ultrasonagraphic assessment of submandibular glands in anti-centromere antibody positive and negative primary sjogren’s syndrome patients

BackgroundPrimary Sjogren’s Syndrome (pSS) is a chronic inflammatory autoimmune disease characterized by lymphocytic infiltration into exocrine glands. Previous reports have demonstrated that a subgroup of anti-centromere antibody (ACA) positive pSS...

Reply

We thank Dr. Shariff and colleagues for their interest in our study1 and for their helpful comments. We are more than happy to provide further detailed information on our cohorts and results in relation to the risk of hepatocellular carcinoma (HCC) development in patients with autoimmune liver diseases (AiLD) as requested. As described in our previous epidemiological study,2 36% of our autoimmune hepatitis (AIH) cohort had histological evidence of cirrhosis at diagnosis, a rate comparable to most other population-based epidemiological studies. In addition, 20% of the AIH cohort had Metavir stage 3 fibrosis, and 44% had stage 2 or lower degree of fibrosis at diagnosis. As reported in our current study, 3 out of 130 AIH patients developed HCC during total follow-up of 1,156 years. This is equivalent to a crude annual incidence of 259 per 100,000 years or 0.3% per year. Mean duration to a diagnosis of HCC from AIH diagnosis was 25 years, and 2 out of 3 of them were cirrhotic from diagnosis. Interestingly, our HCC annual incidence is much lower than that reported by Yeoman and colleagues3 (annual incidence of 1.1%). The discrepancy is likely due to the difference in the group of cohorts that were studied, as their cohort may have been affected by the referral pattern to tertiary specialist centers with a higher proportion of their cohort (56%) having cirrhosis. In our primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) cohorts, 12% and 9%, respectively, had cirrhosis at diagnosis. The prevalence of PBC in Canterbury is low compared to AIH and PSC, and that reported from Europe or North America.4 Our small PBC cohort is certainly a potential explanation for not detecting any HCC during the study period as acknowledged and discussed in our article. The aim of our study was to examine the survival and risk of malignancy in unselected population-based cohorts compared to that of the general population. Our results demonstrated increased mortality in patients with AiLD and increased risk of malignancy in AIH and PSC patients. Future studies should investigate for factors that could predict cirrhosis, malignancy, poor response to treatment, and death so that tailored management strategies and surveillance plans can be devised. Jing H. Ngu M.B.C.H.B., F.R.A.C.P.* , Richard B. Gearry M.B.C.H.B., F.R.A.C.P., Ph.D.* , Catherine A. M. Stedman M.B.C.H.B., F.R.A.C.P., Ph.D* , * Department of Gastroenterology, Christchurch Hospital, New Zealand, University of Otago, Christchurch, New Zealand.

Questionnaire based assessment of risk factors for primary biliary cirrhosis

Primary biliary cirrhosis is a cholestatic liver disease characterized by immune-mediated destruction of bile ducts. Its pathogenesis is largely unknown, although complex interactions between environment and genetic predisposition are proposed. Identify disease risk factors using a detailed patient questionnaire and compare study findings to 3 published reports. Questionnaire data were prospectively collected from 522 cases and 616 controls of the Mayo Clinic Primary Biliary Cirrhosis Genetic Epidemiology Registry. Case and control responses were compared using logistic regression, adjusting for recruitment age, sex, and education level. Cases reported ever regularly smoking cigarettes more frequently than controls (P < 0.001). History of urinary tract infection was similar between groups; however, cases reported multiple urinary tract infections more commonly than controls (P < 0.001). Frequency of other autoimmune disease was higher in cases than controls (P < 0.001). As well, prevalence of primary biliary cirrhosis among first-degree relatives was higher in case families than control families (P < 0.001). Our study confirms prior reported risk factors associated with disease risk. Given the potential importance of gene and environment interactions, further examination of environmental risk factors considering genetic background may provide new insight into primary biliary cirrhosis pathogenesis.

Y chromosome loss in male patients with primary biliary cirrhosis

Sex chromosome abnormalities have been advocated to be involved in the striking female prevalence of primary biliary cirrhosis (PBC) and women with PBC manifest an increased X chromosome loss in peripheral blood mononuclear cells compared to age-matched healthy women. Our knowledge of the etiopathogenesis of autoimmunity in male patients remains, however, limited. Next to the possible role of androgens and their imbalances, the Y chromosome appears as a potential candidate for influence of the immune function in men. Herein we analyzed a population of male patients with primary biliary cirrhosis (n = 26) and healthy controls (n = 88) to define a potential association of disease and the loss of the Y chromosome. We demonstrate that Y chromosome loss indeed is higher in PBC males compared to healthy controls, and this phenomenon increases with aging. We were, thus, able to confirm the existence of an analogous mechanism in the male population to previously identified X haploinsufficiency in female patients with organ-specific autoimmune disease. We propose that this commonality might represent a relevant feature in the etiopathogenesis of autoimmune diseases that should be further investigated.

High prevalence of primary biliary cirrhosis and disease-associated autoantibodies in Japanese patients with systemic sclerosis

Objective To investigate the prevalence of primary biliary cirrhosis (PBC) and PBC-associated autoantibodies in Japanese systemic sclerosis (SSc) patients.Methods Clinical data from 225 Japanese SSc patients were retrospectively obtained. Serum samples from these patients were examined for PBC-associated autoantibodies, anti-mitochondrial M2 antibodies (AMA), anti-sp100 antibodies (anti-sp100), and anti-gp210 antibodies (anti-gp210) by enzyme-linked immunosorbent assay.Results Of 225 patients, 37 (16.4%) had AMA, 13 (5.8%) had anti-sp100, and 3 (1.3%) had anti-gp210. Three patients were positive for both AMA and anti-sp100, and 2 were positive for both AMA and anti-gp210. PBC was found in 22 (9.8%) patients positive for AMA with or without anti-sp100 or anti-gp210, but not in those with anti-sp100 or anti-gp210 without AMA. Furthermore, 13 patients lacking these three antibodies were diagnosed with or suspected of PBC by liver biopsy and/or their clinical manifestation. Multivariable analysis revealed that AMA and anti-centromere antibodies were independently associated with PBC in SSc patients, while anti-sp100 and anti-gp210 were not.Conclusions This study has demonstrated even higher prevalence of both PBC-associated autoantibodies and PBC in the Japanese SSc population than in the Caucasian SSc population. AMA and anti-centromere antibodies are likely to indicate increasing risk of PBC in SSc patients.

Primary biliary cirrhosis in Southern Israel: A 20 year follow up study

BackgroundThe epidemiology of primary biliary cirrhosis (PBC) in Israel is unknown. We aimed to determine the epidemiology, long-term survival and outcomes of PBC in Southern Israel from 1990 to 2010. MethodsCase-finding methods and population-based administrative data were used to estimate and evaluate the incidence, prevalence and prognostic factors of outcome in our PBC cohort. Results138 cases of PBC were identified. The average annual prevalence of PBC was 255 cases per million. The overall age/sex-adjusted annual incidence of PBC was 10 cases per million from 1990 through 1999 and 20 cases per million from 2000 to 2010.Among 138 incident cases with a total follow-up of 960 persons-years from diagnosis, 30 patients (21.7%) died. Survival in PBC patients was significantly lower than that of the age/sex-matched Israeli population. Mortality was significantly increased in patients with an initial MELD score greater than 8 (P<0.001), with portal hypertension (P<0.001), and in non-responders to ursodeoxycholic acid (UDCA) therapy according to Barcelona criteria (P=0.005).Out of 138 patients, 95 patients (68.0%) responded to UDCA therapy according to Barcelona and Paris criteria. None of the responders died during the follow-up period as opposed to 30 out of 43 (69.8%) of non-responders. In multivariate analysis the factors associated with response to UDCA were: albumin levels above 3.5g/dL (P<0.001) and lower degree of fibrosis per liver biopsy (P=0.003). ConclusionsThis study addresses the increasing burden of PBC in Israel and confirms the importance of some clinical and therapeutic factors as predictors of long-term prognosis.

High prevalence of primary biliary cirrhosis and disease-associated autoantibodies in Japanese patients with systemic sclerosis

To investigate the prevalence of primary biliary cirrhosis (PBC) and PBC-associated autoantibodies in Japanese systemic sclerosis (SSc) patients. Clinical data from 225 Japanese SSc patients were retrospectively obtained. Serum samples from these patients were examined for PBC-associated autoantibodies, anti-mitochondrial M2 antibodies (AMA), anti-sp100 antibodies (anti-sp100), and anti-gp210 antibodies (anti-gp210) by enzyme-linked immunosorbent assay. Of 225 patients, 37 (16.4%) had AMA, 13 (5.8%) had anti-sp100, and 3 (1.3%) had anti-gp210. Three patients were positive for both AMA and anti-sp100, and 2 were positive for both AMA and anti-gp210. PBC was found in 22 (9.8%) patients positive for AMA with or without anti-sp100 or anti-gp210, but not in those with anti-sp100 or anti-gp210 without AMA. Furthermore, 13 patients lacking these three antibodies were diagnosed with or suspected of PBC by liver biopsy and/or their clinical manifestation. Multivariable analysis revealed that AMA and anti-centromere antibodies were independently associated with PBC in SSc patients, while anti-sp100 and anti-gp210 were not. This study has demonstrated even higher prevalence of both PBC-associated autoantibodies and PBC in the Japanese SSc population than in the Caucasian SSc population. AMA and anti-centromere antibodies are likely to indicate increasing risk of PBC in SSc patients.

Low incidence and prevalence of primary biliary cirrhosis in Canterbury, New Zealand: a population-based study.

Epidemiological data on primary biliary cirrhosis (PBC) in the Southern Hemisphere is scarce. Our aim was to perform a population-based epidemiological study of PBC in Canterbury, New Zealand. Multiple case-finding methods were employed. All public and private, adult and pediatric outpatient clinics, hospital discharge summaries, and laboratory and pathology reports were searched to identify all cases in the region. Cases were included if at least two of the following criteria were fulfilled: (1) positive anti-mitochondrial antibodies, (2) elevated alkaline phosphatase for greater than 6months, and (3) compatible liver histology. A total of 71 cases of PBC were included. The incidence in 2008 was 0.8 (95% confidence interval (CI) 0.1-1.6) per 100,000. The point prevalence on December 31, 2008 was 9.9 (95% CI 7.1-12.7) per 100,000. Male to female ratio was 1:11. At presentation, 45% were asymptomatic. Age at diagnosis peaked at the seventh decade with mean age at diagnosis of 61 (95% CI 58-64). This is the first population-based epidemiological study of PBC conducted in New Zealand and only the second in the Southern Hemisphere. The incidence and prevalence are lower than the Northern Hemisphere, even though the majority of our population has shared genetic background with some of these countries. Our study has provided further support to the hypothesis that there may be a protective effect or lack of a risk factor for PBC in New Zealand.

Liver transplantation in PBC and PSC: Indications and disease recurrence

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) represent major indications for liver transplantation (LT). Despite the steady increase in the incidence and prevalence of PBC, the number of liver transplants for PBC has fallen in recent years, whereas the number of transplants for PSC has remained stable. Indications for LT for PBC and PSC are no different from those of other causes of chronic liver disease, apart from some disease-specific indications. PBC and PSC have more favourable outcomes after LT, compared to viral hepatitis and alcohol-associated liver disease. Numerous studies have clearly demonstrated that PBC and PSC recur after LT. The diagnosis of recurrent disease should be made on agreed criteria. The impact of recurrent disease on survival is unclear. Study of recurrent PBC and PSC may provide a better understanding of the mechanisms of these diseases in the native liver.

Prevalence of Primary Biliary Cirrhosis–Autoimmune Hepatitis Overlap Syndrome

The prevalence of and the most appropriate way to diagnose the primary biliary cirrhosis (PBC)-chronic autoimmune hepatitis (AIH) overlap syndrome are uncertain. We investigated the prevalence of PBC and AIH and their level of overlap at a tertiary referral center, along with clinical, biochemical, and serologic characteristics. We reviewed data from all patients with PBC (n = 609) and/or AIH (n = 15) examined at the Tufts Medical Center (Boston, MA) from January 1, 2000, to June 20, 2006. PBC was diagnosed based on 2 of the following 3 results: 6 months of positive results in tests for cholestatic liver enzymes, a positive result in a test for antimitochondrial antibodies, or a liver biopsy that indicated PBC. AIH was defined as an alanine aminotransferase level of 200 U/L or greater (≥ 5-fold above normal), a liver biopsy that indicated severe interface hepatitis, and levels of immunoglobulin G 2-fold or greater than that of normal. Only 6 patients with PBC (1%) met the Paris criteria for the overlap syndrome. If we included 9 patients with PBC who did not meet the Paris criteria, but had results from liver enzyme tests and liver biopsy analyses that indicated improvement after treatment with prednisone, the prevalence was 15 (2.8%). This is at the low end of previously reported prevalence values for overlap of PBC and AIH (2%-20%). The prevalence of the PBC-AIH overlap syndrome varies among medical centers. We propose that if the definition of PBC-AIH overlap syndrome be modified to include patients with unequivocal responses to prednisone despite not meeting the Paris criteria, this would improve treatment of patients.

Lessons Learned from Liver Transplantation with the Canadian First Nations

Lessons Learned from Liver Transplantation with the Canadian First Nations

Incidence and risk factors of hepatocellular carcinoma in patients with primary biliary cirrhosis

We read with great interest the article by Cavazza et al.,1 who demonstrated that an advanced histological stage was the only risk factor associated with the development of hepatocellular carcinoma (HCC) in patients with primary biliary cirrhosis (PBC) from two European centers. Similar results were described in a Japanese multicenter study by Shibuya et al.2 Although these studies suggest screening for HCC in patients with advanced-stage PBC, it is still uncertain whether there is a significantly increased risk of HCC development in patients with stage IV PBC cirrhosis versus patients with cirrhosis of other etiologies. We assessed in a single-center study the incidence of HCC in North American patients with stage IV PBC or autoimmune hepatitis (AIH) cirrhosis and compared it to the incidence of HCC in patients with hepatitis C virus (HCV) cirrhosis. Three hundred fifteen patients with HCV cirrhosis, 49 patients with AIH cirrhosis, and 52 patients with stage IV PBC were evaluated at the Cleveland Clinic between 2001 and 2007. Stage IV PBC cirrhosis was diagnosed when patients had positive serological and histological findings of cirrhosis or biochemical and radiological evidence of portal hypertension. Cirrhosis due to AIH was defined by positive serological and histological findings of cirrhosis or biochemical and radiological evidence of portal hypertension. HCC-free survival was analyzed from the moment of the diagnosis of cirrhosis in HCV, AIH, and PBC patients until death or transplantation. During a median follow-up of 3.6 years (with 25th and 75th percentiles of 1.8 and 6.3, respectively), 64 of 315 patients (20.3%) with HCV cirrhosis, 2 of 49 patients (4.1%) with AIH cirrhosis, and 4 of 52 patients (7.7%) with PBC cirrhosis developed HCC. The annual cumulative incidence of HCC was 1.1% in patients with AIH cirrhosis, 1.5% in patients with PBC cirrhosis, and 4.0% in patients with HCV cirrhosis (Fig. 1). Incidence of HCC in patients with AIH, PBC, or HCV. This study has shown that although patients with stage IV PBC cirrhosis develop liver cancer, the risk is significantly lower in comparison with the risk for patients with HCV cirrhosis. The results of our study are discordant with a previously reported Spanish series in which the risks of HCC were similar in patients with late-stage PBC and in patients with HCV cirrhosis.3 We agree with Cavazza et al.1 that the low prevalence of PBC and the possible influence of geography on disease progression are confounding factors that may explain the divergent results in the literature. Future multicenter studies in North America with a longer follow-up period are necessary to validate these findings and better estimate the risk of HCC in PBC patients at an advanced histological stage. Carole Macaron M.D.*, Ibrahim A. Hanouneh M.D. , Nizar N. Zein M.D. , * Department of Internal Medicine, Cleveland Clinic, Cleveland, OH, Department of Gastroenterology and Hepatology, Cleveland Clinic, Cleveland, OH.

Prevalence of primary biliary cirrhosis in adults referring hospital for annual health check-up in Southern China

BackgroundPrimary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by the presence of anti-mitocondrial autoantibodies (AMA) which has an essential role also for diagnosis. In addition, also some anti-nuclear antibodies (ANA) have been shown to be highly specific PBC. The purpose of this study was to assess the prevalence of PBC among the adults referring hospital for annual health check-up in Southern China by screening sera for PBC-specific autoantibodies.MethodsAMA and ANA were screened in 8,126 adults (mean age 44 ± 15 years, 48% females) by indirect immunofluorenscence (IIF). Positive sera were tested by ELISA/immunoblotting for AMA-M2, anti-sp100 and anti-gp210. A diagnosis of PBC was re-assessed six months after the initial testing.ResultsOut of 8,126 individuals 35 were positive for AMA and 79 positive for ANA. Nineteen, 4, and 3 of the subjects positive for AMA and/or ANA showed reactivity for AMA-M2, anti-sp100 or gp210, respectively, further tested with ELISA/immunoblotting. Fourteen in the 39 individuals positive for AMA at IIF, AMA-M2, anti-gp210, or anti-sp100 had abnormal cholestatic liver functional indices. One definite and 3 probable PBC diagnosis could be made in 4 cases including 3 females and 1 male after half a year.ConclusionsWe found a point prevalence rate of PBC among Southern Chinese adults attending for yearly health check-up of 492 cases per million (95% CI, 128 to 1,093) and 1,558 cases per million (95% CI, 294 to 3,815) for women over 40, a finding similar to prevalence reported in other geographical areas.

Clinical features of chronic C virus hepatitis in patients with celiac disease

The association between celiac disease (CD) and several liver disorders has long been documented. About 40% of adult celiac patients have been reported to have mild to moderate hypertransaminasemia (up to five times the upper limit of normal) at the time of diagnosis of CD [1, 2]. In addition, CD has been found in roughly 10% of patients with unexplained hypertransaminasemia, and the majority of them have had their liver enzyme levels normalized after one year of following a strict gluten-free diet [3, 4]. In addition, an increased prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis among CD patients has been reported [5, 6]. CD might also be linked to very severe liver conditions such as end-stage liver failure and hepatocellular carcinoma [7]. There is also evidence, even contrasting reports, about the association of CD with nonalcoholic steatohepatitis and fatty liver disease [8]. In contrast, no definitive evidence is available about the association between chronic hepatitis C (hepatitis C virus [HCV]) and CD. Fine et al. described a three-fold increase of CD prevalence among HCV patients compared to noninfected celiac individuals [9]. It has also been reported the activation of silent CD during the antiviral treatment for HCV with interferon-α and ribavirin, both alone and in combination [10]. Consequently, a routine serological screening for CD has been proposed in HCV patients before starting antiviral therapy. In case of HCV positivity, the achievement of the histological normalization of the intestinal mucosa after following a gluten-free diet has been advised before starting the therapy [10]. On the contrary, some recent prospective studies have not shown increased prevalence of CD among HCV patients and reported that the link between these two conditions is biased by the route of transmission [11, 12]. Among the 3,725 celiac patients included in the Italian Registry of the Complication of Celiac Disease, we identified 34 individuals (0.91%) that had an HCV chronic hepatitis at the time of diagnosis of CD. For the diagnosis of HCV, we considered the serological positivity of antiHCV antibodies. Some of the patients had the diagnosis made in the early 1980s, when the molecular tests for the detection of the viral antigens were not yet available. The demographic and clinical features of the patients with both CD and HCV with respect to those of patients with CD only are listed in Table 1. The prevalence of HCV among our celiac series is lower than the overall prevalence of HCV among the general population in Italy, matched for age and gender, which is estimated to be around 2% [13]. This finding does not support the hypothesis of a potential correlation between these two disorders. It has been assumed that antiviral therapy with INF-α and ribavirin may precipitate the onset of CD in susceptible individuals, promoting a Th1-specific response in the small intestine [14]. However, in our series, only 12 of the 34 celiac patients with HCV had antiviral therapy before CD diagnosis. Looking at our series, it seems more likely that an overall increased risk of CD in HCV patients exists, due to the predisposition for autoimmune diseases related to Eur J Clin Microbiol Infect Dis (2009) 28:1267–1269 DOI 10.1007/s10096-009-0769-6

Incidence, prevalence and clinical course of primary biliary cirrhosis in a Spanish community

Primary biliary cirrhosis (PBC) is characterized by the autoimmune inflammatory response of small intrahepatic bile ducts. Prevalence in Spain is estimated as 61.9 cases per million inhabitants, whereas Northern Europe rates over 200 cases/million. Our objective was to determine the incidence and prevalence of PBC in our health area. PBC was defined by the presence of abnormal liver tests (dissociated cholestasis) with positive antimitochondrial antibodies and/or compatible liver histology. Medical records from patients diagnosed between 1990 and 2002 were reviewed retrospectively. The following data were collected: diagnostic data, demographic and analytic data, liver histology and stage and treatment and disease outcome. In a population of 389 758 inhabitants, 87 patients were diagnosed with PBC. Mean age at diagnosis was 63.9+/-12.6 years. Eighty-four (96.6%) were women. Mean annual incidence was 17.2 per 10 inhabitants and the prevalence at the end of study was 195 per 10. Biopsy was performed in 71 (81.6%) patients, 61 of whom (86%) did not have fibrosis. Time of follow-up was 63.6+/-43.2 (2.28-153.9) months. Incidence and prevalence in our reference area are higher than in some Spanish areas, as per the results previously published; however, they are comparable with those obtained in Northern Europe and the US.

Prevalence and incidence of primary biliary cirrhosis are increasing in Finland

Objective. To examine the epidemiology of primary biliary cirrhosis (PBC) in Finland and to evaluate whether the possible increase in prevalence was attributable to the increasing incidence, better survival, or both. Material and methods. The Hospital Discharge Register, pathology registers, and death certificates for the years 1988–99 were scrutinized, and the patients identified were followed-up for survival until 31 October 2004. The study area covered four university hospital districts: a total of 25 hospitals. The diagnosis of PBC was regarded as definite (or probable) if three (or two) of the following criteria were fulfilled: positive antimitochondrial antibodies, constantly elevated alkaline phosphatase, and compatible liver histology. Results. In the total population of the study areas, the age-standardized prevalence of PBC increased during the study period from 103 (95% CI: 97–110) to 180 (172–189) per million inhabitants. Incidence increased from 12 (10–14) to 17 (15–20) per million inhabitants per year. The annual average increase in prevalence was 5.1% (4.2–5.9%, p<0.0001) and in incidence 3.5% (0.9%–6.0%, p=0.008). In gender-specific analyses among women, the prevalence of PBC increased from 161 (151–171) to 292 (277–207) per million during the study period and the incidence from 20 (16–24) to 27 (23–32) per million per year. The death rate was 4% per year and half the deaths were from liver-related causes. Survival after diagnosis during the study period lengthened. Conclusions. The prevalence of PBC increased in Finland during 1988–99, owing to both the increased incidence and the prolonged survival.