Etiology and natural history of primary sclerosing cholangitis

K. V Narayanan Menon, Russell H. Wiesner

Research output: Contribution to journalArticle

31 Citations (Scopus)

Abstract

The etiology of primary sclerosing cholangitis remains unknown. Bacteria, toxins, viral infections, and immunological and genetic factors have all been proposed as etiological agents. Portal bacteremia, toxins absorbed from the diseased colon in inflammatory bowel disease, and cytomegalovirus and reovirus infections have been implicated by various investigators but there is little evidence to support these hypotheses. The close association between primary sclerosing cholangitis and various human leukocyte antigen haplotypes is now well established and lends support to the theory that immunologic and genetic mechanisms may be involved in its pathogenesis. Patients with primary sclerosing cholangitis may have elevated levels of circulating immune complexes, immunoglobulins, and non-organ specific autoantibodies. The association between ulcerative colitis and primary sclerosing cholangitis remains unexplained and both groups of patients have a high prevalence of antibodies to the perinuclear cytoplasmic antigen. The long-term prognosis in primary sclerosing cholangitis is tempered by the development of cholangiocarcinoma in 6%-30% of patients when followed over long periods of time. Detecting cholangiocarcinoma early in a patient with primary sclerosing cholangitis is one of the most frustrating problems faced by a clinician while caring for these patients. The long-term outlook for patients with primary sclerosing cholangitis and cholangiocarcinoma remains dismal, whatever the treatment modality employed. However, the development of a multivariate statistical survival model from long-term survival data from the Mayo Clinic and other centers has been a major step in identifying individual primary sclerosing cholangitis patients at low, moderate, and high risk of dying. Such models have been useful for stratifying patients in therapeutic trials, for in patient counseling, and in patient selection and timing of liver transplantation.

Original languageEnglish (US)
Pages (from-to)343-351
Number of pages9
JournalJournal of Hepato-Biliary-Pancreatic Surgery
Volume6
Issue number4
StatePublished - 1999

Fingerprint

Sclerosing Cholangitis
Cholangiocarcinoma
Reoviridae Infections
Survival
Cytomegalovirus Infections
Immunologic Factors
Statistical Models
Virus Diseases
HLA Antigens
Bacteremia
Antigen-Antibody Complex
Ulcerative Colitis
Inflammatory Bowel Diseases
Liver Transplantation
Autoantibodies
Haplotypes
Patient Selection
Immunoglobulins
Counseling
Colon

Keywords

  • Cholangiocarcinoma
  • Primary sclerosing cholangitis

ASJC Scopus subject areas

  • Surgery

Cite this

Etiology and natural history of primary sclerosing cholangitis. / Menon, K. V Narayanan; Wiesner, Russell H.

In: Journal of Hepato-Biliary-Pancreatic Surgery, Vol. 6, No. 4, 1999, p. 343-351.

Research output: Contribution to journalArticle

Menon, K. V Narayanan ; Wiesner, Russell H. / Etiology and natural history of primary sclerosing cholangitis. In: Journal of Hepato-Biliary-Pancreatic Surgery. 1999 ; Vol. 6, No. 4. pp. 343-351.
@article{1a8b87dcea8a4bc2989754a58033e1bf,
title = "Etiology and natural history of primary sclerosing cholangitis",
abstract = "The etiology of primary sclerosing cholangitis remains unknown. Bacteria, toxins, viral infections, and immunological and genetic factors have all been proposed as etiological agents. Portal bacteremia, toxins absorbed from the diseased colon in inflammatory bowel disease, and cytomegalovirus and reovirus infections have been implicated by various investigators but there is little evidence to support these hypotheses. The close association between primary sclerosing cholangitis and various human leukocyte antigen haplotypes is now well established and lends support to the theory that immunologic and genetic mechanisms may be involved in its pathogenesis. Patients with primary sclerosing cholangitis may have elevated levels of circulating immune complexes, immunoglobulins, and non-organ specific autoantibodies. The association between ulcerative colitis and primary sclerosing cholangitis remains unexplained and both groups of patients have a high prevalence of antibodies to the perinuclear cytoplasmic antigen. The long-term prognosis in primary sclerosing cholangitis is tempered by the development of cholangiocarcinoma in 6{\%}-30{\%} of patients when followed over long periods of time. Detecting cholangiocarcinoma early in a patient with primary sclerosing cholangitis is one of the most frustrating problems faced by a clinician while caring for these patients. The long-term outlook for patients with primary sclerosing cholangitis and cholangiocarcinoma remains dismal, whatever the treatment modality employed. However, the development of a multivariate statistical survival model from long-term survival data from the Mayo Clinic and other centers has been a major step in identifying individual primary sclerosing cholangitis patients at low, moderate, and high risk of dying. Such models have been useful for stratifying patients in therapeutic trials, for in patient counseling, and in patient selection and timing of liver transplantation.",
keywords = "Cholangiocarcinoma, Primary sclerosing cholangitis",
author = "Menon, {K. V Narayanan} and Wiesner, {Russell H.}",
year = "1999",
language = "English (US)",
volume = "6",
pages = "343--351",
journal = "Journal of Hepato-Biliary-Pancreatic Sciences",
issn = "1868-6974",
publisher = "Springer Verlag",
number = "4",

}

TY - JOUR

T1 - Etiology and natural history of primary sclerosing cholangitis

AU - Menon, K. V Narayanan

AU - Wiesner, Russell H.

PY - 1999

Y1 - 1999

N2 - The etiology of primary sclerosing cholangitis remains unknown. Bacteria, toxins, viral infections, and immunological and genetic factors have all been proposed as etiological agents. Portal bacteremia, toxins absorbed from the diseased colon in inflammatory bowel disease, and cytomegalovirus and reovirus infections have been implicated by various investigators but there is little evidence to support these hypotheses. The close association between primary sclerosing cholangitis and various human leukocyte antigen haplotypes is now well established and lends support to the theory that immunologic and genetic mechanisms may be involved in its pathogenesis. Patients with primary sclerosing cholangitis may have elevated levels of circulating immune complexes, immunoglobulins, and non-organ specific autoantibodies. The association between ulcerative colitis and primary sclerosing cholangitis remains unexplained and both groups of patients have a high prevalence of antibodies to the perinuclear cytoplasmic antigen. The long-term prognosis in primary sclerosing cholangitis is tempered by the development of cholangiocarcinoma in 6%-30% of patients when followed over long periods of time. Detecting cholangiocarcinoma early in a patient with primary sclerosing cholangitis is one of the most frustrating problems faced by a clinician while caring for these patients. The long-term outlook for patients with primary sclerosing cholangitis and cholangiocarcinoma remains dismal, whatever the treatment modality employed. However, the development of a multivariate statistical survival model from long-term survival data from the Mayo Clinic and other centers has been a major step in identifying individual primary sclerosing cholangitis patients at low, moderate, and high risk of dying. Such models have been useful for stratifying patients in therapeutic trials, for in patient counseling, and in patient selection and timing of liver transplantation.

AB - The etiology of primary sclerosing cholangitis remains unknown. Bacteria, toxins, viral infections, and immunological and genetic factors have all been proposed as etiological agents. Portal bacteremia, toxins absorbed from the diseased colon in inflammatory bowel disease, and cytomegalovirus and reovirus infections have been implicated by various investigators but there is little evidence to support these hypotheses. The close association between primary sclerosing cholangitis and various human leukocyte antigen haplotypes is now well established and lends support to the theory that immunologic and genetic mechanisms may be involved in its pathogenesis. Patients with primary sclerosing cholangitis may have elevated levels of circulating immune complexes, immunoglobulins, and non-organ specific autoantibodies. The association between ulcerative colitis and primary sclerosing cholangitis remains unexplained and both groups of patients have a high prevalence of antibodies to the perinuclear cytoplasmic antigen. The long-term prognosis in primary sclerosing cholangitis is tempered by the development of cholangiocarcinoma in 6%-30% of patients when followed over long periods of time. Detecting cholangiocarcinoma early in a patient with primary sclerosing cholangitis is one of the most frustrating problems faced by a clinician while caring for these patients. The long-term outlook for patients with primary sclerosing cholangitis and cholangiocarcinoma remains dismal, whatever the treatment modality employed. However, the development of a multivariate statistical survival model from long-term survival data from the Mayo Clinic and other centers has been a major step in identifying individual primary sclerosing cholangitis patients at low, moderate, and high risk of dying. Such models have been useful for stratifying patients in therapeutic trials, for in patient counseling, and in patient selection and timing of liver transplantation.

KW - Cholangiocarcinoma

KW - Primary sclerosing cholangitis

UR - http://www.scopus.com/inward/record.url?scp=0033250073&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0033250073&partnerID=8YFLogxK

M3 - Article

VL - 6

SP - 343

EP - 351

JO - Journal of Hepato-Biliary-Pancreatic Sciences

JF - Journal of Hepato-Biliary-Pancreatic Sciences

SN - 1868-6974

IS - 4

ER -