title: Treatment of HCV-Related Mixed Cryoglobulinemia.
abstract: Mixed cryoglobulinemia syndrome (MCs) is a systemic vasculitis, involving skin, joints, peripheral nerves, and several internal organs. Hepatitis C virus (HCV) is recognized as the etiologic agent for the majority of MCs patients, as well as of number of autoimmune, lymphoproliferative, and neoplastic disorders. In this context, HCV-related MCs represents an important model autoimmune/ neoplastic disease triggered by a virus in humans. With regard the therapeutic strategies of MCs, we can treat these patients at different steps by means of etiological (antivirals), pathogenetic, symptomatic drugs (mainly immunosuppressors, corticosteroids, plasmapheresis). In the majority of individuals, MCs shows a mild, slow-progressive clinical course needing only symptomatic treatments, generally low doses of corticosteroids. Considering the etiopathogenesis of MCs, the eradication of HCV should be considered the gold standard in the treatment of MCs. The use of combined peg-interferon- α/ribavirin and/or novel antiviral drugs may lead to HCV eradication in a significant percentage of cases with possible remission of MCs. On the other hand, the presence of rapidly progressive, diffuse vasculitis with multiple organ involvement may be successfully treated with aggressive immunosuppressive and anti-inflammatory therapies, mainly based on cyclophosphamide or rituximab, high dose corticosteroids, and plasma aphaeresis. Moreover, sequential/combined antiviral or immunosuppressive treatments could represent an useful therapeutic strategy particularly in MCs patients with major clinical manifestations. The treatment of MCs should be decided for every patient according to the severity of clinical picture. Thus, a careful follow-up of the disease is necessary, with particular attention to the possibility of cancers onset, such as B-cell lymphoma. The present review focuses on the different therapeutic strategies in patients with MCs, including the treatment of cryoglobulinemic skin ulcers, which represents one of the most discouraging complications of the disease.
authors: Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Azienda Ospedaliero- Universitaria, Policlinico di Modena, Modena, Italy.
authors: Dilia
authors: D
authors: Giuggioli
authors: Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Azienda Ospedaliero- Universitaria, Policlinico di Modena, Modena, Italy.
authors: Marco
authors: M
authors: Sebastiani
authors: Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Azienda Ospedaliero- Universitaria, Policlinico di Modena, Modena, Italy.
authors: Michele
authors: M
authors: Colaci
authors: Department of Internal Medicine, School of Medicine, University of Pisa, Pisa, Italy.
authors: Poupak
authors: P
authors: Fallahi
authors: Center for Systemic Manifestations of Hepatitis Viruses (MASVE), Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
authors: Laura
authors: L
authors: Gragnani
authors: Center for Systemic Manifestations of Hepatitis Viruses (MASVE), Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
authors: Anna Linda
authors: AL
authors: Zignego
authors: Department of Internal Medicine, School of Medicine, University of Pisa, Pisa, Italy.
authors: Alessandro
authors: A
authors: Antonelli
authors: Rheumatology Unit, Department of Internal Medicine, University of Modena and Reggio E., Policlinico di Modena, via del Pozzo, 71, 41100 Modena, Italy.
authors: Clodoveo
authors: C
authors: Ferri
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use; D003449:Cryoglobulinemia / Q000188:drug therapy* / Q000821:virology; D004359:Drug Therapy, Combination / Q000379:methods*; D006526:Hepatitis C / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D057285:Precision Medicine
chemical_list: D000893:Anti-Inflammatory Agents; D000998:Antiviral Agents; D007166:Immunosuppressive Agents
keywords: Hepatitis C virus; cryoglobulinemic vasculitis; lymphoma; mixed cryoglobulinemia; rituximab; skin ulcers