Geographic Clusters of Primary Biliary Cirrhosis

Saif Abu-Mouch, Carlo Selmi, Gordon D. Benson, Thomas P. Kenny, Pietro Invernizzi, Massimo Zuin, Mauro Podda, Lorenzo Rossaro, M. Eric Gershwin

Research output: Contribution to journalArticle

40 Citations (Scopus)

Abstract

Genetic and environmental factors have been widely suggested to contribute to the pathogenesis of primary biliary cirrhosis (PBC), an autoimmune disease of unknown etiology leading to destruction of small bile ducts. Interestingly, epidemiologic data indicate a variable prevalence of the disease in different geographical areas. The study of clusters of PBC may provide clues as to possible triggers in the induction of immunopathology. We report herein four such unique PBC clusters that suggest the presence of both genetic and environmental factors in the induction of PBC. The first cluster is represented by a family of ten siblings of Palestinian origin that have an extraordinary frequency of PBC (with 5/8 sisters having the disease). Second, we describe the cases of a husband and wife, both having PBC. A family in which PBC was diagnosed in two genetically unrelated individuals, who lived in the same household, represents the third cluster. Fourth, we report a high prevalence of PBC cases in a very small area in Alaska. Although these data are anedoctal, the study of a large number of such clusters may provide a tool to estimate the roles of genetics and environment in the induction of autoimmunity.

Original languageEnglish (US)
Pages (from-to)127-131
Number of pages5
JournalClinical and Developmental Immunology
Volume10
Issue number2-4
DOIs
StatePublished - Jun 2003

Fingerprint

Biliary Liver Cirrhosis
Spouses
Siblings
Bile Ducts
Autoimmunity
Autoimmune Diseases

Keywords

  • Autoimmunity
  • Environment
  • Genetics
  • Geoepidemiology

ASJC Scopus subject areas

  • Immunology
  • Cell Biology
  • Developmental Biology

Cite this

Abu-Mouch, S., Selmi, C., Benson, G. D., Kenny, T. P., Invernizzi, P., Zuin, M., ... Gershwin, M. E. (2003). Geographic Clusters of Primary Biliary Cirrhosis. Clinical and Developmental Immunology, 10(2-4), 127-131. https://doi.org/10.1080/10446670310001626526

Geographic Clusters of Primary Biliary Cirrhosis. / Abu-Mouch, Saif; Selmi, Carlo; Benson, Gordon D.; Kenny, Thomas P.; Invernizzi, Pietro; Zuin, Massimo; Podda, Mauro; Rossaro, Lorenzo; Gershwin, M. Eric.

In: Clinical and Developmental Immunology, Vol. 10, No. 2-4, 06.2003, p. 127-131.

Research output: Contribution to journalArticle

Abu-Mouch, S, Selmi, C, Benson, GD, Kenny, TP, Invernizzi, P, Zuin, M, Podda, M, Rossaro, L & Gershwin, ME 2003, 'Geographic Clusters of Primary Biliary Cirrhosis', Clinical and Developmental Immunology, vol. 10, no. 2-4, pp. 127-131. https://doi.org/10.1080/10446670310001626526
Abu-Mouch S, Selmi C, Benson GD, Kenny TP, Invernizzi P, Zuin M et al. Geographic Clusters of Primary Biliary Cirrhosis. Clinical and Developmental Immunology. 2003 Jun;10(2-4):127-131. https://doi.org/10.1080/10446670310001626526
Abu-Mouch, Saif ; Selmi, Carlo ; Benson, Gordon D. ; Kenny, Thomas P. ; Invernizzi, Pietro ; Zuin, Massimo ; Podda, Mauro ; Rossaro, Lorenzo ; Gershwin, M. Eric. / Geographic Clusters of Primary Biliary Cirrhosis. In: Clinical and Developmental Immunology. 2003 ; Vol. 10, No. 2-4. pp. 127-131.
@article{db24115d53f84949ad5969bee1615bf5,
title = "Geographic Clusters of Primary Biliary Cirrhosis",
abstract = "Genetic and environmental factors have been widely suggested to contribute to the pathogenesis of primary biliary cirrhosis (PBC), an autoimmune disease of unknown etiology leading to destruction of small bile ducts. Interestingly, epidemiologic data indicate a variable prevalence of the disease in different geographical areas. The study of clusters of PBC may provide clues as to possible triggers in the induction of immunopathology. We report herein four such unique PBC clusters that suggest the presence of both genetic and environmental factors in the induction of PBC. The first cluster is represented by a family of ten siblings of Palestinian origin that have an extraordinary frequency of PBC (with 5/8 sisters having the disease). Second, we describe the cases of a husband and wife, both having PBC. A family in which PBC was diagnosed in two genetically unrelated individuals, who lived in the same household, represents the third cluster. Fourth, we report a high prevalence of PBC cases in a very small area in Alaska. Although these data are anedoctal, the study of a large number of such clusters may provide a tool to estimate the roles of genetics and environment in the induction of autoimmunity.",
keywords = "Autoimmunity, Environment, Genetics, Geoepidemiology",
author = "Saif Abu-Mouch and Carlo Selmi and Benson, {Gordon D.} and Kenny, {Thomas P.} and Pietro Invernizzi and Massimo Zuin and Mauro Podda and Lorenzo Rossaro and Gershwin, {M. Eric}",
year = "2003",
month = "6",
doi = "10.1080/10446670310001626526",
language = "English (US)",
volume = "10",
pages = "127--131",
journal = "Journal of Immunology Research",
issn = "2314-8861",
publisher = "Hindawi Publishing Corporation",
number = "2-4",

}

TY - JOUR

T1 - Geographic Clusters of Primary Biliary Cirrhosis

AU - Abu-Mouch, Saif

AU - Selmi, Carlo

AU - Benson, Gordon D.

AU - Kenny, Thomas P.

AU - Invernizzi, Pietro

AU - Zuin, Massimo

AU - Podda, Mauro

AU - Rossaro, Lorenzo

AU - Gershwin, M. Eric

PY - 2003/6

Y1 - 2003/6

N2 - Genetic and environmental factors have been widely suggested to contribute to the pathogenesis of primary biliary cirrhosis (PBC), an autoimmune disease of unknown etiology leading to destruction of small bile ducts. Interestingly, epidemiologic data indicate a variable prevalence of the disease in different geographical areas. The study of clusters of PBC may provide clues as to possible triggers in the induction of immunopathology. We report herein four such unique PBC clusters that suggest the presence of both genetic and environmental factors in the induction of PBC. The first cluster is represented by a family of ten siblings of Palestinian origin that have an extraordinary frequency of PBC (with 5/8 sisters having the disease). Second, we describe the cases of a husband and wife, both having PBC. A family in which PBC was diagnosed in two genetically unrelated individuals, who lived in the same household, represents the third cluster. Fourth, we report a high prevalence of PBC cases in a very small area in Alaska. Although these data are anedoctal, the study of a large number of such clusters may provide a tool to estimate the roles of genetics and environment in the induction of autoimmunity.

AB - Genetic and environmental factors have been widely suggested to contribute to the pathogenesis of primary biliary cirrhosis (PBC), an autoimmune disease of unknown etiology leading to destruction of small bile ducts. Interestingly, epidemiologic data indicate a variable prevalence of the disease in different geographical areas. The study of clusters of PBC may provide clues as to possible triggers in the induction of immunopathology. We report herein four such unique PBC clusters that suggest the presence of both genetic and environmental factors in the induction of PBC. The first cluster is represented by a family of ten siblings of Palestinian origin that have an extraordinary frequency of PBC (with 5/8 sisters having the disease). Second, we describe the cases of a husband and wife, both having PBC. A family in which PBC was diagnosed in two genetically unrelated individuals, who lived in the same household, represents the third cluster. Fourth, we report a high prevalence of PBC cases in a very small area in Alaska. Although these data are anedoctal, the study of a large number of such clusters may provide a tool to estimate the roles of genetics and environment in the induction of autoimmunity.

KW - Autoimmunity

KW - Environment

KW - Genetics

KW - Geoepidemiology

UR - http://www.scopus.com/inward/record.url?scp=0347763479&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0347763479&partnerID=8YFLogxK

U2 - 10.1080/10446670310001626526

DO - 10.1080/10446670310001626526

M3 - Article

VL - 10

SP - 127

EP - 131

JO - Journal of Immunology Research

JF - Journal of Immunology Research

SN - 2314-8861

IS - 2-4

ER -