Cryoglobulinemia Vasculitis
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Edité par CCSD ; Elsevier [Commercial Publisher] -
International audience. Cryoglobulinemic vasculitis (CryoVas) is a small vessel vasculitis involving mainly the skin, the joints, the peripheral nerve system and the kidneys. Type I CryoVas are single monoclonal immunoglobulins related to an underlying B-cell lymphoproliferative disorder. Type II and III cryoglobulins, often referred to as mixed cryoglobulinemia, consist of polyclonal IgG with or without monoclonal IgM with rheumatoid factor activity. Hepatitis C virus (HCV) infection represents the main cause of mixed CryoVas. The 10-year survival rates are 63%, 65% and 87% in HCV-positive mixed CryoVas, HCV-negative mixed CryoVas and type I CryoVas patients, respectively. In HCV-positive patients, baseline poor prognostic factors include the presence of severe liver fibrosis, and central nervous system, kidney, and heart involvement. Treatment with antivirals is associated with a good prognosis whereas use of immunosuppressant (including corticosteroids) is associated with a poor outcome. In HCV-negative patients, pulmonary and gastrointestinal involvement, renal insufficiency and age>65 years are independently associated with death. Increased risk of lymphoma should also be underlined. Treatment of type I CryoVas is that of the hemopathy; specific treatment also include plasma exchange, corticosteroids, rituximab and ilomedine. In HCV-CryoVas with mild to moderate disease, an optimal antiviral treatment should be given. For HCV-CryoVas with severe vasculitis (i.e. worsening of renal function, mononeuritis multiplex, extensive skin disease, intestinal ischemia…) control of disease with rituximab, with or without plasmapheresis, is required before initiation of antiviral therapy. Other immunosuppressants should be given only in case of refractory forms of CryoVas, frequently associated with underlying B-cell lymphoma.
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