Cryoglobulinemic Vasculitis
摘要
Cryoglobulinemic syndrome is a systemic inflammatory situation characterized by the presence of cryoglobulinemia with the coexistence of ischemic lesions causing tissue damage due to cryoglobulinemic vasculitis (CV) or hyperviscosity syndrome secondary to the presence of monoclonal immunoglobulins with cryoglobulin activity. Infectious diseases, mainly chronic hepatitis C virus infection, hematologic malignancies, and autoimmune diseases, are the main associated or causative factors for cryoglobulin production. Clinical vignettes highlight heterogeneous presentations, including asthenia, arthralgia, and cutaneous, neurological, and renal involvement. Diagnostic algorithms incorporating serological and histopathological assessments have been delineated. The evolving landscape of targeted therapies and immunomodulatory interventions has been also successfully explored, emphasizing the need for treating the underlying disease in all cases in which this can be identified. In this sense, the recent HCV eradication has almost eliminated the main cause of CV to date.