Cryoglobulinemic vasculitis

Cryoglobulinemia is idefined as the presence of serum of cryoglobulins, which are immunoglobulins that precipitate when the temperature is below 37°C and resolve when rewarmed.
A small vessel, vasculitis, characterized by the deposition of immune complexes, containing cryoglobulins in the capillaries, venules and arterioles.
Manifestations include purpura and leg ulcers, arthrialgia, and arthritis, peripheral nerve involvement, mononeuritis multiplex, distal sensorimotor polyneuropathy.
Most common cause is hepatitis C virus.

Occurs in 10 to 15% of patients with hepatitis C and has a spectrum of disease ranging from mild to life-threatening.

The large majority of patients with cryoglobulinemic vasculitis are HCV positive.

Alpha interferon yields a clinical, biochemical and virologic response in 40 to 60% of cases , however relapse within six months after completion of therapy is usual.

The addition of low to intermediate dose steroids mitigates vasculitic flares and provides symptomatic relief, however long term use of glucocorticoids should be avoided because of questionable effectiveness and long-term side effects.

High, pulsed doses of glucocorticoids may help manage severe vasculitic findings, including renal and neurologic involvement, and may prevent permanent organ damage.

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