Clinical management of autoimmune biliary diseases

Mohamad H. Imam, Jayant A. Talwalkar, Keith Lindor

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Autoimmune biliary disease is an umbrella term that encompasses several distinct entities such as primary sclerosing cholangitis, primary biliary cirrhosis, autoimmune hepatitis and overlap syndromes. The general approach to the diagnosis of these disorders involves investigating symptomatic patients presenting with a cholestatic biochemical profile. Asymptomatic patients are often diagnosed during investigation of other accompanying or discrete diseases. The distinction between the various entities is necessary for directing clinical management in this group of patients with an underlying autoimmune pathophysiology. Goals of management comprise treating symptoms, preventing complications and suppressing the underlying pathogenetic processes. Liver transplantation plays a vital role in the management of this group of patients and has shown a dramatic improvement in outcomes. Medical therapies such as ursodeoxycholic acid have shown mixed effects with excellent outcomes in primary biliary cirrhosis and less impressive results in primary sclerosing cholangitis. In this manuscript we aim to discuss in detail the management of these autoimmune biliary disorders and describe the effects of different therapies on outcomes on the different subsets of patients.

Original languageEnglish (US)
Pages (from-to)88-96
Number of pages9
JournalJournal of Autoimmunity
Volume46
DOIs
StatePublished - Oct 2013

Fingerprint

Autoimmune Diseases
Sclerosing Cholangitis
Biliary Liver Cirrhosis
Ursodeoxycholic Acid
Autoimmune Hepatitis
Liver Transplantation
Therapeutics

Keywords

  • Autoimmune hepatitis
  • Clinical outcomes
  • Immunoglobulin g4
  • Overlap syndromes
  • Primary biliary cirrhosis
  • Primary sclerosing cholangitis

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

Cite this

Clinical management of autoimmune biliary diseases. / Imam, Mohamad H.; Talwalkar, Jayant A.; Lindor, Keith.

In: Journal of Autoimmunity, Vol. 46, 10.2013, p. 88-96.

Research output: Contribution to journalArticle

Imam, Mohamad H. ; Talwalkar, Jayant A. ; Lindor, Keith. / Clinical management of autoimmune biliary diseases. In: Journal of Autoimmunity. 2013 ; Vol. 46. pp. 88-96.
@article{12eb4b7768574617989a3c1dee1d352a,
title = "Clinical management of autoimmune biliary diseases",
abstract = "Autoimmune biliary disease is an umbrella term that encompasses several distinct entities such as primary sclerosing cholangitis, primary biliary cirrhosis, autoimmune hepatitis and overlap syndromes. The general approach to the diagnosis of these disorders involves investigating symptomatic patients presenting with a cholestatic biochemical profile. Asymptomatic patients are often diagnosed during investigation of other accompanying or discrete diseases. The distinction between the various entities is necessary for directing clinical management in this group of patients with an underlying autoimmune pathophysiology. Goals of management comprise treating symptoms, preventing complications and suppressing the underlying pathogenetic processes. Liver transplantation plays a vital role in the management of this group of patients and has shown a dramatic improvement in outcomes. Medical therapies such as ursodeoxycholic acid have shown mixed effects with excellent outcomes in primary biliary cirrhosis and less impressive results in primary sclerosing cholangitis. In this manuscript we aim to discuss in detail the management of these autoimmune biliary disorders and describe the effects of different therapies on outcomes on the different subsets of patients.",
keywords = "Autoimmune hepatitis, Clinical outcomes, Immunoglobulin g4, Overlap syndromes, Primary biliary cirrhosis, Primary sclerosing cholangitis",
author = "Imam, {Mohamad H.} and Talwalkar, {Jayant A.} and Keith Lindor",
year = "2013",
month = "10",
doi = "10.1016/j.jaut.2013.06.014",
language = "English (US)",
volume = "46",
pages = "88--96",
journal = "Journal of Autoimmunity",
issn = "0896-8411",
publisher = "Academic Press Inc.",

}

TY - JOUR

T1 - Clinical management of autoimmune biliary diseases

AU - Imam, Mohamad H.

AU - Talwalkar, Jayant A.

AU - Lindor, Keith

PY - 2013/10

Y1 - 2013/10

N2 - Autoimmune biliary disease is an umbrella term that encompasses several distinct entities such as primary sclerosing cholangitis, primary biliary cirrhosis, autoimmune hepatitis and overlap syndromes. The general approach to the diagnosis of these disorders involves investigating symptomatic patients presenting with a cholestatic biochemical profile. Asymptomatic patients are often diagnosed during investigation of other accompanying or discrete diseases. The distinction between the various entities is necessary for directing clinical management in this group of patients with an underlying autoimmune pathophysiology. Goals of management comprise treating symptoms, preventing complications and suppressing the underlying pathogenetic processes. Liver transplantation plays a vital role in the management of this group of patients and has shown a dramatic improvement in outcomes. Medical therapies such as ursodeoxycholic acid have shown mixed effects with excellent outcomes in primary biliary cirrhosis and less impressive results in primary sclerosing cholangitis. In this manuscript we aim to discuss in detail the management of these autoimmune biliary disorders and describe the effects of different therapies on outcomes on the different subsets of patients.

AB - Autoimmune biliary disease is an umbrella term that encompasses several distinct entities such as primary sclerosing cholangitis, primary biliary cirrhosis, autoimmune hepatitis and overlap syndromes. The general approach to the diagnosis of these disorders involves investigating symptomatic patients presenting with a cholestatic biochemical profile. Asymptomatic patients are often diagnosed during investigation of other accompanying or discrete diseases. The distinction between the various entities is necessary for directing clinical management in this group of patients with an underlying autoimmune pathophysiology. Goals of management comprise treating symptoms, preventing complications and suppressing the underlying pathogenetic processes. Liver transplantation plays a vital role in the management of this group of patients and has shown a dramatic improvement in outcomes. Medical therapies such as ursodeoxycholic acid have shown mixed effects with excellent outcomes in primary biliary cirrhosis and less impressive results in primary sclerosing cholangitis. In this manuscript we aim to discuss in detail the management of these autoimmune biliary disorders and describe the effects of different therapies on outcomes on the different subsets of patients.

KW - Autoimmune hepatitis

KW - Clinical outcomes

KW - Immunoglobulin g4

KW - Overlap syndromes

KW - Primary biliary cirrhosis

KW - Primary sclerosing cholangitis

UR - http://www.scopus.com/inward/record.url?scp=84886310297&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84886310297&partnerID=8YFLogxK

U2 - 10.1016/j.jaut.2013.06.014

DO - 10.1016/j.jaut.2013.06.014

M3 - Article

VL - 46

SP - 88

EP - 96

JO - Journal of Autoimmunity

JF - Journal of Autoimmunity

SN - 0896-8411

ER -