Title:Treatment of HCV-Related Mixed Cryoglobulinemia
Volume: 18
Issue: 7
Author(s): Dilia Giuggioli, Marco Sebastiani, Michele Colaci, Poupak Fallahi, Laura Gragnani, Anna Linda Zignego, Alessandro Antonelli and Clodoveo Ferri*
Affiliation:
- Rheumatology Unit, Department of Internal Medicine, University of Modena and Reggio E., Policlinico di Modena, via del Pozzo, 71, 41100 Modena,Italy
Keywords:
Hepatitis C virus, mixed cryoglobulinemia, cryoglobulinemic vasculitis, lymphoma, skin ulcers, rituximab.
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.
