This book is the proceedings of the Falk Symposium No. 127 on
Autoimmune Diseases in Paediatric Gastroenterology' (IV
International Falk Symposium on Paediatric Gastroenterology), held
in Basel, Switzerland, on November 8-9, 2001. The symposium focused
on the role of the immune system, both the acquired and the innate
systems, in inflammatory bowel disease (IBD) in children and
adolescents. The innate system has an important fundamental role in
host defence by initiating immune responses against potentially
deleterious matter. However, a mutation within the innate system
may elicit an immune response against the host: hence, an
autoimmune response.
Chronic autoimmune hepatitis occurs predominantly in young
people, and especially in women. Immunological changes are
conspicuous. Tissue antibodies are found in a large number of
patients. This is a disease of disordered immunoregulation marked
by a deficit in suppressor T cells causing the production of
autoantibodies against specific hepatocyte surface antigen. Liver
membrane protein is found in the sera of patients with autoimmune
chronic acute hepatitis and with primary biliary cirrhosis (PBC).
The latter condition of progressive granulomatous destruction of
intrahepatic bile ducts is, in many respects, analogous to the
graft-versus-host syndrome where the immune system has become
sensitized to foreign HLA-molecules.
Primary sclerosing cholangitis (PSC) is another condition of
unknown origin. All parts of the biliary tree can be involved in a
chronic, fibrosing, inflammatory process that results in
obliteration of the biliary tree and ultimately in biliary
cirrhosis. About half of the patients also suffer from ulcerative
colitis and rarely from Crohn's disease. Circulating antibodies to
some antigens are found in obstructed portal tracts, as well as
increased concentrations of biliary immune complexes in patients
with PBC.
In all three previous Falk symposia on paediatric gastroenterology,
attention was focused on the role of the innate immune system in
the aetiology of IBD. It has become increasingly clear in recent
years that the innate system has a much more important and
fundamental role in host defence. The decision to initiate an
immune response is one of the major roles of the innate system.
Mutations within this system could transform it into becoming
constitutively active, resulting in an inflammatory reaction and
thus eliciting an autoimmune response.
Following an introduction to the basic phenomena of
autoimmunity, the proceedings discuss clinical aspects of
autoimmune diseases. In particular, current knowledge and the state
of the art about the diagnosis and treatment of the autoimmune
diseases of the gastrointestinal tract are described by
world-renowned experts. The book also contains the short
presentations on selected topics, as well as abstracts of the
mini-posters read by title, which were included in the
symposium.
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