Incidence and Prevalence of Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis, and Autoimmune Hepatitis in a Norwegian Population

Boberg, Erik

doi:10.1080/00365529850166284

Cited by 443 publications

(69 citation statements)

References 14 publications

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“…The prevalence of PSC in the United States is reported to be 13.6 per 100000 inhabitants[4]. Norway has the highest incidence rate at 1.31 per 100000 inhabitants and a prevalence of 8.5 per 100000[17]. The true prevalence of PSC may be higher than the aforementioned estimates both nationally and internationally because cholangiography may not be widely available in many parts of the world and patients with PSC may have normal levels of serum alkaline phosphatase (ALP)[18].…”

Section: Epidemiologymentioning

confidence: 99%

Pathogenesis and clinical spectrum of primary sclerosing cholangitis

Gidwaney¹,

Pawa²,

Das³

2017

WJG

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Primary sclerosing cholangitis (PSC) is a disease of the biliary tract, which has been documented in the literature since 1867. This disease has a strong predilection for affecting men and can be seen in individuals as young as 2 years of age. PSC has a strong associated with inflammatory bowel disease, more commonly with ulcerative colitis, and is also part of the clinical spectrum of IgG4-related diseases. Small-duct PSC, a variant of PSC, also has an association with inflammatory bowel disease. The exact pathogenesis of PSC is not well understood at present, however, is likely a combination of a genetic predisposition with alteration of the molecular structure of the gut. Abnormal serum liver chemistry and presence of certain autoimmune markers are usually the first indicators leading to a diagnosis of PCS, however, these may often be normal in early stages of this disease. The diagnosis is made by cholangiography, which is now considered the gold standard. PSC is a known pre-malignant condition. Such patients have an increased risk of developing cholangiocarcinoma, gallbladder neoplasia, and colon cancer. Many new treatment modalities have emerged in the recent past, including anti-tumor necrosis factor- α and anti-integrins; however, liver transplantation is the only known cure for PSC. Despite past and present research, PSC remains an enigmatic biliary disease with few viable treatment options.

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Section: Epidemiologymentioning

confidence: 99%

Pathogenesis and clinical spectrum of primary sclerosing cholangitis

Gidwaney¹,

Pawa²,

Das³

2017

WJG

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“…Among the 43 enrolled studies, 22 reported the incidence of PBC [5–26], while 13 were relevant for PSC [13-14, 17, 27-36], and 14 provided information about epidemiology of AIH [4, 13-14, 17, 37-46]. No studies mentioned the population incidence of IgG4 related cholangitis, presumably because it is relatively rare as a new disease entity.…”

Section: Resultsmentioning

confidence: 99%

National incidence of autoimmune liver diseases and its relationship with the human development index

et al. 2016

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ObjectivePrimary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH) and immunoglobulin G4 related cholangitis represent the major autoimmune liver diseases (AILD). However, the relationship between AILD incidence and socioeconomic development levels is yet to be explored.ResultsA total of 43 studies were included. There was a positive but not significant correlation between the PBC incidence and HDI on a global level (r=0.348, P=0.082). However, in Europe, a significantly positive correlation existed between the PBC incidence and HDI (r=0.455, P=0.044). No statistical correlation between PSC incidence and HDI was observed (r=0.116, P=0.706). The incidence of AIH revealed a positive correlation with the national HDI both globally (r=0.638, P=0.014) and in Europe (r=0.644, P=0.045). Moreover, the PBC incidence demonstrated a positive correlation with the health index (r=0.422, P=0.036), but a negative correlation with the education index (r= −0.650, P<0.01). Moreover, the income index presented a positive correlation with both the PSC incidence (r=0.599, P=0.031) and the AIH incidence (r=0.649, P=0.012).MethodsPubMed was searched to identify relevant epidemiological studies on AILD. The human development index (HDI) was applied as an indicator for socioeconomic development. HDI data were obtained and calculated based on the 2014 Human Development Report. Pearson coefficient and linear regression analysis were conducted to estimate the correlation between incidence and HDI.ConclusionsThere is positive association between the national incidence of AILD and the socioeconomic status, as measured by HDI. In less-developed countries, the incidence of AILD, especially PBC and AIH, might be less common.

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“…84 Adult estimates vary, with the incidence and prevalence ranging from 1.7 to 1.9 per 100,000 per year and from 16.9 to 42.9 per 100,000, respectively. 85–87 …”

Section: Autoimmune and Cholestatic Liver Diseasesmentioning

confidence: 99%

Current and Future Burden of Chronic Nonmalignant Liver Disease

Udompap

Kim

2015

Clinical Gastroenterology and Hepatology

116

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Disease burden is an important indicator of the state of health of a population. It can be measured as the frequency (e.g. incidence and prevalence) of a condition or its effects including: fatal and non-fatal health loss from disease (e.g. disability-adjusted life years (DALYs)) as well as the financial costs (e.g. direct health care costs and indirect health care expenditures related to lost income due to premature death). Accurate disease burden information is essential for policy-making such as prioritization of health interventions and allocation of resources. Chronic Liver Disease (CLD) causes substantial health and economic burden in the US, where nearly two million deaths annually are attributable to CLD. In the recent past, overall mortality rate of CLD has been increasing. Viral hepatitis and alcoholic liver disease are thought to be the most common etiologies of chronic liver diseases. More recently, the prevalence of non-alcoholic steatohepatitis (NAFLD) is rapidly increasing and non-alcoholic steatohepatitis (NASH) has become a leading indication for liver transplantation. In this work, we assemble available data on the burden of CLD in the United States, focusing on non-malignant complications, whereas the impact on mortality and healthcare expenses of hepatocellular carcinoma (HCC), an important consequence of CLD is discussed elsewhere.

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Incidence and Prevalence of Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis, and Autoimmune Hepatitis in a Norwegian Population

Abstract: The prevalences of PBC and AIH are of the same order of magnitude and about twice as high as that of PSC. These epidemiologic data can be used to estimate the number of liver transplantations required due to autoimmune liver diseases.

Cited by 443 publications

References 14 publications

Pathogenesis and clinical spectrum of primary sclerosing cholangitis

Pathogenesis and clinical spectrum of primary sclerosing cholangitis

National incidence of autoimmune liver diseases and its relationship with the human development index

Current and Future Burden of Chronic Nonmalignant Liver Disease

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