Article information
Resumen
Las crioglobulinemias son una forma especial de vasculitis sistémica secundaria a la presencia de inmunoglobulinas circulantes que se precipitan a temperaturas menores de 37°C y se solubilizan nuevamente con el aumento de la temperatura. Esta precipitación lleva a una vasculitis sistémica de medianos y pequeños vasos, como consecuencia de la presencia de complejos inmunes circulantes y expansión clonal de linfocitos B. Este grupo de entidades debe sospecharse en pacientes con fenómeno de Raynaud, signos clínicos de isquemia periférica en ausencia de enfermedad vascular, cianosis de orejas, nariz y dedos inducida por el frío, vasculitis cutánea, glomerulonefritis membranoproliferativa y gammopatías monoclonales, principalmente. La presencia de crioglobulinas circulantes, la disminución en los niveles de complemento, usualmente de los componentes tempranos como el C4 y el C1q, y la púrpura ortostática son característicos de la enfermedad. El tratamiento de las crioglobulinemias depende de la severidad de los síntomas, la enfermedad de base, la presencia del virus de la hepatitis C y el tipo de crioglobulinas. Las principales causas de muerte en estos individuos son: los compromisos renal, hepático y gastrointestinal, las infecciones, sexo masculino y criocrito mayor a cinco por ciento.
Palabras clave:
crioglobulinemia
crioglobulinas
hepacivirus
Summary
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%.
Key words:
cryoglobulinemia
cryoglobulins
hepacivirus
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* El trabajo no fue soportado por alguna beca o por fondos de farmacéutica.
Copyright © 2012. Asociación Colombiana de Reumatología
