Cryoglobulinemia (Review)

Abstract

Abstract.Cryoglobulins are immunoglobulins that precipitate at low temperatures and redissolve upon rewarming. Cryoglobulinemia refers to the presence of circulating cryoglobulins in serum, and generally leads to a systemic inflammatory syndrome characterized by fatigue, arthralgia, purpura, neuropathy and glomerulonephritis. The disease mainly involves small to medium-sized blood vessels and causes vasculitis due to cryoglobulin-containing immune complexes. Cryoglobulinemia is classified into three types (I, II and III) on the basis of immunoglobulin composition. Predisposing conditions include lymphoproliferative disease, collagen disease and hepatitis C virus (HCV) infection. The diagnosis of cryoglobulinemic syndrome is predominantly based on the laboratory demonstration of serum cryoglobulins. Treatment is often directed towards the underlying disease state. For patients with chronic HCV infection, anti-viral therapy is indicated. Intense immunosuppressive or immunomodulatory therapy, including steroids, plasmapheresis and cytotoxic agents, is reserved for organ-threatening or recalcitrant disease. In this review, we discuss the clinical characteristics of the three types of cryoglobulinemia.

Extensive choroidal infarction in a case of mixed essential cryoglobulinaemia in a postpartum female

“…Cryoglobulinaemia can occur secondary to various other systemic disorders and its idiopathic occurrence is referred to as essential cryoglobulinaemia 3. Diagnoses of essential cryoglobulinaemia principally rest on the demonstration of cryoglobulins or the cryocrit 3.…”

“…Diagnoses of essential cryoglobulinaemia principally rest on the demonstration of cryoglobulins or the cryocrit 3. However, due to the stringent requirement of the laboratory temperature conditions for the same, there are often false-negative results 4.…”

Vasculite leucocitoclástica e infecção crónica por Hepatite B

“…As crioglobulinémias podem ser do tipo I, II ou III 4 . A crioglobulinémia mista (tipo II ou III), representa 80% dos casos, estando relacionada com estados inflamatórios crónicos, nomeadamente doenças autoimunes e infecções víricas, sendo a situação mais provável neste caso.…”

Cryoglobulinemic vasculitis with renal involvement: A historic aspect of the problem

“…Связь криоглобулинемического МКГН с HСV-инфекцией в дальнейшем подтверждена в исследованиях, проведенных в Ев-ропе (Италия, Испания, Франция), США и Японии, России [4,6,[31][32][33]. В основе криоглобулинемического МКГН, развивающе-гося в рамках смешанной КГЕ, в том числе ассоциированной с HCV, лежит повышенная тропность моноклонального компо-нента IgMκ РФ (вследствие наличия в его антигенсвязывающей части кросс-идиотипа WA) к мезангиальному матриксу почки, в частности к фибронектину.…”