Publication: Hepatitis C virus-associated mixed cryoglobulinemia. Clinical manifestations, histopathological changes, mechanisms of
cryoprecipitation and options of treatment
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Date
2001
Authors
Schott, P. ; Hartmann, H. ; Ramadori, G.
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Chronic hepatitis C virus (HCV) infection is
frequently associated with a variety of autoimmune
phenomenons. Mixed cryoglobulinemia (MC) appears in
up t o 50% o f chronic HCV-infected patients.
Cryoglobulins consist of immunoglobulin complexes
precipitating in vitro when cooled below body
temperature. In most cases IgM with rheumatoid factor
activity is found in cryoprecipitates which could lead to
vasculitis induced by the deposition of immnuocomplexes
in small vessels. This vasculitis is thought to
cause clinical symptoms called Meltzer's triad. This
triad is represented by purpura, arthralgia and weakness.
One third of patients suffering from HCV-associated
mixed cryoglobulinemia are developing typical
symptoms during their course of disease.
The striking association between HCV infection and
MC has conduced to the hypothesis that HCV is of
major importance in the production of MC with followed
vasculitis.
Both hepatrophism and lymphotrophism have been
reported for the hepatitis C virus. Infection of B-cells by
HCV could probably lead to a bcl-2 translocation and
immunoglobulin gene rearrangement which results in
clonal lymphoproliferation and in synthesis of
monoclonal IgM with rheumatoid factor activity. These
IgM form immunocomplexes with IgG in the cold which are finally responsible for the described vasculitis.
Histopathological changes of the liver are dominated
by chronic HCV infection. The majority of times mild
activity of hepatitis or mild fibrosis could be found.
Nevertheless, cirrhosis is more often found in HCVinfected
patients suffering from MC compared to
patients without MC.
Conventional treatment of MC is aimed to reduce
circulating immune complexes by immunosupression
and plasmapheresis. With the emerging concept of a
viral pathogenesis the therapeutic approach has changed
during the last decade. Interferon treatment of MC,
particularly of HCV-associated MC is well established
nowadays.
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