Are increased individual susceptibility and environmental factors both necessary for the development of primary biliary cirrhosis?

Douglas, Janice G.; Finlayson, N. D. C.

doi:10.1136/bmj.2.6187.419

Cited by 43 publications

(27 citation statements)

References 8 publications

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“…Antimitochondrial antibodies (AMA) have stood the test of time and remain the hallmark of this disease. The first observation of possible environmental and genetic factors involved in the pathogenesis of PBC was reported by Douglas and Finlayson in 1979 who described the development of PBC in the mother and a neighbor of an index case [5]. Subsequent studies confirmed a strong genetic background to PBC [6].…”

Section: Introductionmentioning

confidence: 84%

Primary Biliary Cirrhosis

Milkiewicz¹,

Heathcote²

2006

Diseases of the Gallbladder and Bile Ducts

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Section: Introductionmentioning

confidence: 84%

Primary Biliary Cirrhosis

Milkiewicz¹,

Heathcote²

2006

Diseases of the Gallbladder and Bile Ducts

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“…This latter notion is supported by studies reporting nonfamilial clustering of PBC. Indeed, an early study by Douglas and Finlayson [36] has described a daughter, her mother, and an unrelated close friend developing PBC. The mother and the close friend nursed the daughter through her terminal illness and developed PBC within 21 months after her death [36].…”

Section: Familial and Nonfamilial Clustering Of Pbcmentioning

confidence: 97%

“…Indeed, an early study by Douglas and Finlayson [36] has described a daughter, her mother, and an unrelated close friend developing PBC. The mother and the close friend nursed the daughter through her terminal illness and developed PBC within 21 months after her death [36]. An unusual clustering of six women with PBC living in two small cities in the state of Alaska amongst whom three had been working together as phone operators for several years has been also reported [35].…”

Section: Familial and Nonfamilial Clustering Of Pbcmentioning

confidence: 97%

Bacteria and Primary Biliary Cirrhosis

Bogdanos

Vergani

2008

Clinic Rev Allerg Immunol

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Infectious agents have been postulated to play a pathogenic role in the loss of immunological tolerance and the induction of primary biliary cirrhosis, an immune-mediated cholestatic liver disease characterized by progressive destruction of the small intrahepatic bile ducts and subsequent cirrhosis and liver failure. This review discusses emerging issues implicating infectious agents such as Escherichia coli, mycobacteria, chlamydia, helicobacter species, lactobacilli, Novosphingobium aromaticivorans, and betaretroviruses in the pathogenesis of primary biliary cirrhosis. We also review the immunopathological mechanisms responsible for the induction of the disease with special emphasis on the role of molecular mimicry and microbial/self immunological cross-reactivity.

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“…The disease has an annual incidence of approximately four patients per million population in a Northern European population [30, 85,86]. A prevalence of 90-150 cases per million population has been ascertained for Northern European populations [59]; there may be lower prevalence for other populations, Caucasian or Asian, suggesting environmental determinants [11]. It is the most female predominant of the autoimmune diseases, with a female to male sex ratio of as high as 12 to 1.…”

Section: Introductionmentioning

confidence: 99%