Rheumatology Advance Access originally published online on June 12, 2007
Rheumatology 2007 46(8):1234-1242; doi:10.1093/rheumatology/kem132
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Hepatitis C-associated mixed cryoglobulinaemia: a crossroad between autoimmunity and lymphoproliferation
1Université Pierre et Marie Curie-Paris 6, CNRS, UMR 7087 2AP-HP, Hôpital Pitié-Salpêtrière, Service de Médecine Interne, Paris, F-75013 France 3Department of Rheumatic and Immunologic Diseases, Cleveland Clinic Lerner, Cleveland, OH, USA.
Correspondence to: Prof. Patrice Cacoub, MD, PhD, AP-HP, Hôpital Pitié-Salpêtrière, Service de Médecine Interne, Paris, F-75013 France. E-mail: patrice.cacoub{at}psl.aphp.fr
| Abstract |
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Hepatitis C virus (HCV) infection is the second most cocmmon chronic viral infection in the world with a global prevalence of about 2%. Chronic HCV infection is commonly associated with a number of extrahepatic complications. Circulating mixed cryoglobulins (MCs) are detected in 40–60% of HCV-infected patients whereas overt cryoglobulinaemia vasculitis develops in only 5–10% of the cases. MC reflects the expansion of B cells producing a pathogenic IgM with rheumatoid factor (RF) activity. Because cryoglobulin-producing B cells in HCV are mostly monoclonal, HCV-associated MC can be viewed as a benign B cell lymphoproliferative condition. The disease expression of MC vasculitis is variable, ranging from mild clinical symptoms (purpura, arthralgia) to fulminant life-threatening complications (glomerulonephritis, widespread vasculitis). The overall risk of non-Hodgkin's lymphoma in patients with HCV-MC is estimated to be 35 times higher than that in the general population. This review will focus on recent advances in our understanding of the clinical course, complications, pathophysiology and treatment of those immune-mediated disorders.
KEY WORDS: HCV infection, Mixed cryoglobulinaemia, Vasculitis, Non-Hodgkin's lymphoma
Submitted 7 January 2007;
revised version accepted 11 April 2007.
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