Cryoglobulins are immunoglobulins that precipitate at temperatures less than 37°C. They occur secondary to infectious, autoimmune, and malignant processes. In the Brouet classification, type I cryoglobulinemia is caused by hyperviscosity, whereas type II and III manifestations are caused by vasculitis in target organs (primarily skin, peripheral nerves, and kidney). New classification criteria were recently proposed that may help with study and treatment of cryoglobulinemic vasculitis (CryoVas). Hepatitis C virus is the most common cause of CryoVas and treatment with antivirals can be curative in mild cases, whereas rituximab is highly effective in treating active vasculitis in more severe cases.
Key points
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Cryoglobulins can be grouped by the Brouet classification criteria into 3 categories based on their immunoglobulin clonality. These categories have differing clinical presentations and treatment responses.
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Skin, peripheral nerves, and kidneys are the most commonly affected organs in mixed cryoglobulinemic vasculitis.
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Treatment of cryoglobulinemic vasculitis must take into account the cause of the cryoglobulins, the mechanism of damage, and the severity of symptoms.
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Most cases of cryoglobulinemic vasculitis are caused by hepatitis C virus (HCV) and advances in the identification and treatment of HCV have led to major progress in the treatment of cryoglobulinemic vasculitis.
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Mixed cryoglobulinemic vasculitis is associated with B-cell proliferation and rituximab is highly effective in the treatment of severe cases.
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