Background and Aims

Cryoglobulins are antibodies that precipitate at low temperatures and dissolve after rewarming.

Cryoglobulinemia refers to the presence of circulating cryoglobulins and generally leads to a systemic inflammatory syndrome characterized by fatigue, arthralgia, purpura, ulcers, neuropathy and/or glomerulonephritis.

The disease mainly involves small to medium-sized blood vessels and causes vasculitis due to cryoglobulin-containing immune complexes.

Methods

A review of current literature on the approach to diagnosis, classification and treatment of cryoglobulinemic vasculitis.

Results

Cryoglobulinemia is classified into three types (I, II and III) on the basis of immunoglobulin composition.

Predisposing conditions include lymphoproliferative, autoimmune diseases and hepatitis C virus infection.

The diagnosis of cryoglobulinemic syndrome is predominantly based on the presence of clinical features and laboratorial demonstration of serum cryoglobulins.

The treatment strategy depends on the cause of cryoglobulinemia. For patients with chronic HCV infection, antiviral therapy is indicated. Immunosuppressive or immunomodulatory therapy, including steroids, plasmapheresis and cytotoxic agents, is reserved for organ-threatening manifestations.

Conclusions

In this review, we discuss the main clinical presentations, diagnostic approach and treatment options.