CRYOGLOBULINEMIC VASCULITIS: LITERATURE REVIEW AND CLINICAL CASE

Abstract

The purpose of this work was to analyze literature sources on the diagnosis and treatment of cryoglobulinemic vasculitis with the demonstration of our own data. Cryoglobulinemic vasculitis (CV) is a difficult and rare diagnosis not only for a physician, but also for a rheumatologist. The disease has polymorphic clinical manifestations and can debut with lesions of various organs and systems. Additional difficulties in the diagnosis of the disease are created by ambiguous laboratory research results. CV is often associated with lymphoproliferative diseases such as diffuse large B-cell lymphoma or non-Hodgkin’s lymphoma, autoimmune and infectious diseases. Viral hepatitis C is the most common underlying cause of CV and makes up approximately 80% of all cases. In case when the etiological factor is not established, the term «idiopathic» or «essential» CV is used. For the diagnosis of CV, classification criteria are proposed, including the results of the questionnaire, clinical manifestations and laboratory research data. The principles of treating patients with CV are determined by the etiopathogenetic mechanisms and the severity of the clinical manifestations of the disease. When the etiological factor of CV development is established, etiotropic therapy is administered. Patients with severe or life-threatening manifestations require urgent intervention to suppress the formation of immune complexes. This is achieved by means of immunosuppressive therapy including glucocorticosteroids, cytostatics, rituximab, and plasmapheresis.