Crioglobulinemias

Cryoglobulinemia are a special form of systemic vasculitis secondary to the presence of circulating immunoglobulins that precipitate at temperatures below 37 °C and are solubilised again with increasing temperature. This precipitation leads to medium and small-vessel systemic vasculitis as a result of the presence of circulating immune complexes and clonal expansion of B lymphocytes. This group of entities should be suspected in patients with Raynaud phenomenon, clinical signs of ischemia in the absence of peripheral vascular disease, coldinduced cyanosis of ears, nose and fingers, cutaneous vasculitis, membranoproliferative glomerulonephritis, and monoclonal gammopathy. The presence of circulating cryoglobulins, decreased complement levels, usually early components, such as C1q and C4, and orthostatic purpura, are characteristic of the disease. Treatment of cryoglobulinemia depends of severity of the symptoms, underlying disease, the presence of hepatitis C virus and the type of cryoglobulins. The main causes of death in these individuals are: renal, hepatic, gastrointestinal involvements, infections, male and cryocrit more than 5%.