Primary biliary cirrhosis, primary sclerosing cholangitis, and adult cholangiopathies:
Gespeichert in:
Format: | Buch |
---|---|
Sprache: | English |
Veröffentlicht: |
Philadelphia [u.a.]
Saunders
1998
|
Schriftenreihe: | Clinics in liver disease
2,2 |
Schlagworte: | |
Online-Zugang: | Inhaltsverzeichnis |
Beschreibung: | NST: PBC, PSC, and adult cholangiopathies |
Beschreibung: | XII S., S. 217 - 455 |
Internformat
MARC
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Datensatz im Suchindex
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adam_text | I PRIMARY HIl.IAR^ CIRRHOSIS PRIMARY SCI l ROSJNC:
UIOI ANiilFIS AND ADLl I CHOI.AVIIOI ATIHlS
CONTENTS
Preface xiii
Keith D. Lindor and E. Rolland Dickson
Overview of Chronic Cholestatic Conditions in Adults:
Terminology and Definitions 217
Sheila Sherlock
The cholangiopathies represent diseases and syndromes affecting
the biliary system at any site between the canals of Hering and
the ampulla of Vater. Hepato canalicular cholestasis reflects bili¬
ary secretory failure of the hepatocyte caused by disturbances of
intracellular organelles or damage to the bile canalicular excretory
functions. Drug reactions are related especially to antibiotics, phe
nothiazine derivates and carbamazepine. Immune mediated cho¬
langiopathies cause destruction and reduction of interlobular bile
ducts, and are sometimes called vanishing bile duct diseases.
They include primary biliary cirrhosis, primary sclerosing cholan
gitis, autoimmune cholangitis, chronic hepatic allograft rejection,
graft versus host disease and chronic cholestatic sarcoidosis. Is
chemic (vascular) cholangiopathies include traumatic, hepatic ar
teritis and mechanical causes. Infectious cholangiopathies usually
are associated with the immunosuppressed patient.
Pathogenesis of Primary Biliary Cirrhosis, Primary
Sclerosing Cholangitis, and Autoimmune Cholangiopathy 235
Marion G. Peters
Autoimmune liver diseases have much in common with each
other, and there are clear associations with genetic haplotypes.
Elegant studies have shown autoimmune liver disease induced
by viruses and drugs. Although there is evidence for nonimmuno
} CLINICS IN LIVER DISEASE
VOLUME 2 • NUMBER 2 • MAY 1998 vii
logical events precipitating immune disease, especially in primary
sclerosing cholangitis, the precise pathways, what is bystander
and what is essential, have not been determined. This article
reviews some of the mechanisms involved in pathogenesis.
Histopathology of Chronic Cholestasis and Adult
Ductopenic Syndrome 249
Valeer J. Desmet
This article reviews the histopathologic liver alterations in chronic
cholestatic diseases and parenchymal changes associated with
periportal ductular reaction and fibrosis, which may result in
end stage biliary cirrhosis. Adult ductopenic diseases are possible
causes of such chronic cholestasis. Ductopenia is defined, and
guidelines are given for its quantitation. The last section summa¬
rizes the specific histologic lesions of primary biliary cirrhosis,
primary sclerosing cholangitis, their histologic mimics, and the
differential diagnosis with drug induced cholestasis and ducto¬
penia.
Primary Biliary Cirrhosis: Clinical and Associated
Autoimmune Features and Natural History 265
David E. J. Jones, Oliver F. W. James, Margaret F. Bassendine
Primary biliary cirrhosis, a chronic liver disease, predominately
affects middle aged women. The diagnosis is established by the
presence of disease specific autoantibodies and compatible liver
histology showing focal immune mediated damage to the intrahe
patic bile ducts. Patients now are detected prior to the onset of
symptoms typical of cholestasis with abnormal liver function
tests, or even prior to the onset of abnormal liver function tests,
with positive antimitochondrial antibodies. Earlier diagnosis is
changing not only our appreciation of the prevalence of this
condition, but also of the natural history. The disease appears to
be heterogeneous with some patients having a slow progression
and a normal life expectancy, although other patients have a more
aggressive course developing symptoms and end stage disease
that leads to death or liver transplantation.
Clinical Features in Primary Sclerosing Cholangitis 283
Annika Bergquist and Ulrika Broome
This article describes clinical features in primary sclerosing cho¬
langitis, including clinical presentation and complications. The
natural history of the disease is discussed, as well as the strong
association to inflammatory bowel disease.
Autoimmune Cholangitis 303
E. Jenny Heathcote
Autoimmune cholangitis is the term that has been used to de¬
scribe patients who have the clinical, biochemical, and histologic
Viii CONTENTS
characteristics of primary biliary cirrhosis (PBC), but who are
antinuclear antibody positive rather than anti mitochondrial anti¬
body (AMA) positive in their sera. The course of their disease is
similar to AMA positive cases, and the associated nonhepatic
autoimmune diseases are the same in both AMA positive and
AMA negative PBC. Serial testing for AMA using highly sensitive
and specific techniques over time suggests that in subjects with
autoimmune cholangitis, their AMA negative status remains neg¬
ative. The beneficial response to treatment with ursodeoxycholic
acid is the same as for AMA positive PBC. It may be preferable
to use the term autoimmune cholangitis, further stratified by
AMA status, instead of the somewhat innapropriate term primary
biliary cirrhosis.
Predictive Models of Natural History in Primary
Biliary Cirrhosis 313
W. Ray Kim and E. Rolland Dickson
Primary biliary cirrhosis is a slow, progressive disease. Although
many years may elapse before asymptomatic primary biliary
cirrhosis patients begin experiencing symptoms of liver disease,
their overall survival is significantly lower than the normal popu¬
lation. The Mayo natural history model has been developed to
depict patient survival in the absence of effective therapeutic
intervention. Although there are a number of caveats in applying
this model, it has been validated using external data sets and
established as an accepted tool for clinical or research purposes.
Furthermore, recent data suggest that the Mayo natural history
model continues to provide useful, predictive information in the
presence of ursodeoxycholic acid therapy, which has been shown
to lower the serum bilirubin to the natural history model for
patient survival. In addition to the natural history model for
patient survival, mathematical models have been developed to
describe histologic progression and development of esophageal
varices.
Management of Primary Biliary Cirrhosis and
Autoimmune Cholangitis 333
Paul Angulo and Keith D. Lindor
Several drugs have been evaluated in the treatment of primary
biliary cirrhosis over a number of years. These drugs have immu
nosuppressive, antiinflammatory, cupruretic, antifibrotic and bile
acid properties. Ursodeoxycholic acid has been shown to improve
survival free of transplantation in a conclusive fashion. This drug
is the single agent that can be recommended for the treatment of
primary biliary cirrhosis. Corticosteroid therapy and ursodeoxy¬
cholic acid have been evaluated in a few patients with autoim¬
mune cholangitis. This article reviews a large number of studies
that have been published assessing different drugs in the treat¬
ment of these two entities, particularly in the treatment of pri¬
mary biliary cirrhosis.
CONTENTS ix
The Management of Primary Sclerosing Cholangitis 353
Stephen A. Mitchell and Roger W. G. Chapman
Primary sclerosing cholangitis is a chronic cholestatic liver disease
characterized by a progressive, obliterating fibrosis of the intrahe
patic and extrahepatic bile ducts. The pathogenesis of PSC is
unknown, but it is thought to be an immune mediated disease.
Although the role of cupruretics, immunosuppressants (cortico
steroids, azathioprine, tacrolimus, methotrexate), antifibrogenic
agents, and ursodeoxycholic acid in the treatment of primary
sclerosing cholangitis is reviewed, none of these agents has been
shown to retard or reverse the rate of disease progression. Of
these therapies, ursodeoxycholic acid at high doses looks the
most promising, but large trials are needed to establish whether
treatment with high dose ursodeoxycholic acid influences the
morbidity and mortality associated with primary sclerosing cho¬
langitis.
Lipids and Lipid Activated Vitamins in Chronic
Cholestatic Diseases 373
Kris V. Kowdley
Primary biliary cirrhosis, primary sclerosing cholangitis and auto¬
immune cholangiopathy are cholestatic liver diseases of unknown
cause. Destruction of small to medium bile ducts (in primary
biliary cirrhosis and autoimmune cholangiopathy) and large bile
ducts (in primary sclerosing cholangitis) leads to progressive cho
lestasis, liver failure and end stage liver disease. A variety of
abnormalities in lipid metabolism have been described in primary
biliary cirrhosis, and range from alterations in serum lipid levels
and lipoprotein subsets to deranged metabolism of cholesterol.
Progressive cholestasis and, consequently, decreased small intesti¬
nal bile acid concentrations in these cholestatic liver disease can
also lead to impaired absorption of fats and fat soluble vitamins,
resulting in steatorrhea and deficiencies in vitamins A, D, E, and
K. This article focuses on abnormalities in lipid metabolism in
primary biliary cirrhosis and primary sclerosing cholangitis, and
on lipid activated vitamin deficiencies in these disorders.
The Pruritus of Cholestasis: Evolving Pathogenic Concepts
Suggest New Therapeutic Options 391
Nora V. Bergasa and E. Anthony Jones
Pruritis is a distressing symptom experienced by a large propor¬
tion of patients with cholestasis. The cause of this form of pruritus
is unknown, and therapy tends to be empirical and unsatisfactory.
This article discusses the emerging role of the brain and neuro
transmitter systems in the pathogenesis of the pruritus of chole¬
stasis and emphasizes the importance of the application of quanti¬
tative methodology in clinical trials of therapies for the pruritus
of cholestasis.
X CONTENTS
Osteoporosis 407
J. Eileen Hay
Osteopenia, in the form of osteoporosis, is a common complica¬
tion of chronic cholestatic liver diseases and, although its cause
is poorly understood, it appears to be intimately related to the
cholestasis itself. With more patients surviving longer with suc¬
cessful liver transplantation, the clinical significance of such os¬
teopenia has increased, and a traumatic fracturing has become a
major cause of morbidity in this patient population. Noninvasive
diagnosis is easy, and serial measurements allow assessment of
disease progression. Although no effective therapy can treat or
prevent this complication, supportive measures can improve skel¬
etal well being, especially in high risk individuals who are candi¬
dates for liver transplantation.
Recurrence of Primary Biliary Cirrhosis, Autoimmune
Cholangitis and Primary Sclerosing Cholangitis after
Liver Transplantation 421
Kenneth P. Batts and Xuedong Wang
Given the usually prolonged natural history of primary biliary
cirrhosis, autoimmune cholangitis, and primary sclerosing cholan¬
gitis, and the relatively recent introduction of orthotopic liver
transplantation, our understanding of recurrence of these autoim¬
mune diseases after orthotopic liver transplantation has been
slow to evolve. Present data suggest that after orthotopic liver
transplantation, patients with primary biliary cirrhosis will have
persistence of serum antimitochondrial antibodies, develop histo
logic lesions suggestive of recurrent primary biliary cirrhosis with
a frequency in the 8% to 16% range at 2 to 5 years after orthotopic
liver transplantation, but will demonstrate little if any symptom¬
atic disease as a consequence. Although data are extremely
limited, autoimmune cholangitis patients will have a similar post
transplant course (without antimitochondrial antibodies). Recur¬
rence of primary sclerosing cholangitis remains the most contro¬
versial, however, these patients probably develop nonanastomotic
intrahepatic and extrahepatic strictures more frequently than pa¬
tients without primary sclerosing cholangitis, with a frequency in
the 20% to 25% range at 3 to 5 years. With longer patient follow
up and additional studies, it is hoped that our understanding of
recurrent autoimmune biliary diseases will grow considerably in
the future.
Hepatobiliary Malignancy 437
Blake A. Jones and Gregory J. Gores
Chronic cholestatic liver disease may be complicated by hepato¬
biliary malignancy. The early detection of hepatocellular carci¬
noma and cholangiocarcinoma is of paramount importance in the
evaluation of candidates for liver transplantation, which remains
the only effective treatment modality for advanced primary bili
CONTENTS xi
ary cirrhosis and primary sclerosing cholangitis. This article re¬
views the identification of patients at high risk, current techniques
for diagnosis, and makes recommendations for screening high
risk patients. This article also reviews preliminary data from the
Mayo Clinic regarding liver transplantation for cholangiocarci
noma following radiation therapy.
Index 451
Subscription Information Inside Back Cover
Xii CONTENTS
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spelling | Primary biliary cirrhosis, primary sclerosing cholangitis, and adult cholangiopathies Keith D. Lindor, ... guest ed. PBC, PSC, and adult cholangiopathies Philadelphia [u.a.] Saunders 1998 XII S., S. 217 - 455 txt rdacontent n rdamedia nc rdacarrier Clinics in liver disease 2,2 NST: PBC, PSC, and adult cholangiopathies Cholangite primitive sclérosante Cirrhose biliaire Foie, maladies Voies biliaires, maladies Liver Diseases Primäre biliäre Zirrhose (DE-588)4205037-6 gnd rswk-swf Gallenwegskrankheit (DE-588)4124902-1 gnd rswk-swf (DE-588)4143413-4 Aufsatzsammlung gnd-content Gallenwegskrankheit (DE-588)4124902-1 s DE-604 Primäre biliäre Zirrhose (DE-588)4205037-6 s Lindor, Keith D. Sonstige oth Clinics in liver disease 2,2 (DE-604)BV011603500 2,2 HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=008134581&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
spellingShingle | Primary biliary cirrhosis, primary sclerosing cholangitis, and adult cholangiopathies Clinics in liver disease Cholangite primitive sclérosante Cirrhose biliaire Foie, maladies Voies biliaires, maladies Liver Diseases Primäre biliäre Zirrhose (DE-588)4205037-6 gnd Gallenwegskrankheit (DE-588)4124902-1 gnd |
subject_GND | (DE-588)4205037-6 (DE-588)4124902-1 (DE-588)4143413-4 |
title | Primary biliary cirrhosis, primary sclerosing cholangitis, and adult cholangiopathies |
title_alt | PBC, PSC, and adult cholangiopathies |
title_auth | Primary biliary cirrhosis, primary sclerosing cholangitis, and adult cholangiopathies |
title_exact_search | Primary biliary cirrhosis, primary sclerosing cholangitis, and adult cholangiopathies |
title_full | Primary biliary cirrhosis, primary sclerosing cholangitis, and adult cholangiopathies Keith D. Lindor, ... guest ed. |
title_fullStr | Primary biliary cirrhosis, primary sclerosing cholangitis, and adult cholangiopathies Keith D. Lindor, ... guest ed. |
title_full_unstemmed | Primary biliary cirrhosis, primary sclerosing cholangitis, and adult cholangiopathies Keith D. Lindor, ... guest ed. |
title_short | Primary biliary cirrhosis, primary sclerosing cholangitis, and adult cholangiopathies |
title_sort | primary biliary cirrhosis primary sclerosing cholangitis and adult cholangiopathies |
topic | Cholangite primitive sclérosante Cirrhose biliaire Foie, maladies Voies biliaires, maladies Liver Diseases Primäre biliäre Zirrhose (DE-588)4205037-6 gnd Gallenwegskrankheit (DE-588)4124902-1 gnd |
topic_facet | Cholangite primitive sclérosante Cirrhose biliaire Foie, maladies Voies biliaires, maladies Liver Diseases Primäre biliäre Zirrhose Gallenwegskrankheit Aufsatzsammlung |
url | http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=008134581&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |
volume_link | (DE-604)BV011603500 |
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