Chapter

Hepatitis C virus-associated mixed cryoglobulinaemia

Edgar D. Charles

in Rheumatology and the Kidney

Second edition

Published on behalf of Oxford University Press

Published in print April 2012 | ISBN: 9780199579655
Published online February 2013 | e-ISBN: 9780191763472 | DOI: http://dx.doi.org/10.1093/med/9780199579655.003.0121

Series: Oxford Clinical Nephrology Series

Hepatitis C virus-associated mixed cryoglobulinaemia

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1. HCV-related cryoglobulins contain RF, polyclonal IgG and HCV RNA that precipitate and deposit on vascular endothelium, causing an end-organ vasculitis predominantly in skin, kidneys and peripheral nerves. 2. Patients often have striking clonal expansions of RF-bearing memory B cells with restricted usage of RF-encoding Ig gene segments. Most of these activated B cells have low to moderate levels of somatic hypermutations that suggest an immunological response to antigenic stimulation. 3. A smaller subset of patients with MC develop a low-grade NHL comprising B cells that are immunophenotypically similar to the expanded B cells seen in MC. The antigenic dependence of these B cells is supported by evidence that HCV-related MC and NHL disappear after successful treatment of HCV infection. 4. Treatment of HCV-related MC should aim to eradicate HCV infection. MC symptoms almost always resolve within 6 months of successful virological clearance. However, patients who are unlikely to tolerate or benefit from anti-HCV therapy may be offered symptomatic therapy with immunosuppressives (e.g. rituximab, corticosteroids or cyclophosphamide) or plasmapheresis. 5. Continued patient-centred studies are necessary to elucidate the pathogenesis of HCV MC and to devise improved therapeutic strategies for affected patients.

Chapter.  6782 words. 

Subjects: Nephrology

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