Diagnostic and Treatment of Cryoglobulinemiс Vasculitis: What is Important for Real Clinical Practice?

Abstract

Cryoglobulinemia is a condition in which circulating cryoprecipitate immune complexes are detected in serum. The cryoglobulin concentration above 50 mg/l is considered diagnostically significant for the statement of cryoglobulinemia. The production of cryoglobulins, as a rule, is a consequence of the underlying disease, which requires etiological evaluation. The diagnosis of cryoglobulinemic vasculitis (CV) is based on laboratory detection of serum cryoglobulinemia in combination with characteristic clinical signs and symptoms. The main clinical manifestations include common symptoms (severe fatigue, unexplained fever with or without weight loss), skin lesions (orthostatic palpable purpura, necrotic ulcers), joints (arthritis, arthralgia), peripheral nervous system (mononeuritis, polyneuritis) and kidney (membranoproliferative glomerulonephritis). Given the many signs and symptoms, a patient with CV rarely turns primarily to a rheumatologist. First contact doctors are often family doctors or dermatologists, along with the fact that the awareness of doctors of other specialties about this pathology is insufficient. The article presents modern data on the etiological factors, CV types and variants of the clinical course and treatment. CV classification criteria are highly informative and available to practitioners. Treatment of CV remains a challenge due to serious specific target organ damage and sometimes life-threatening manifestations. In secondary cryoglobulinemia, treatment of the underlying disease is crucial. In case of CV on the background of mixed cryoglobulinemia, the treatment strategy is based on antiviral, anti-inflammatory and immunosuppressive therapy. The therapy goals for CV include reducing the immunoglobulin level and removing the antigen. The first goal can be achieved with immunosuppressants, while the second goal depends on whether the antigen is known or not. With CV associated with HCV, antiviral therapy reduces the number of antigens. However, in autoimmune diseases, the potential antigen is usually not recognized, and only non-specific immunosuppressants are used.