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Medical Author:

William C. Shiel Jr., MD, FACP, FACR

William C. Shiel Jr., MD, FACP, FACR

Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

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In this Article

• What is cryoglobulinemia?
• What conditions are associated with cryoglobulinemia?
• What is essential mixed cryoglobulinemia?
• What is the prognosis (outlook) for patients with essential mixed cryoglobulinemia?
• How is essential mixed cryoglobulinemia treated?
• Patient Comments: Cryoglobulinemia - Experience
• Find a local Internist in your town
• Essential Mixed Cryoglobulinemia Index

When the cryoglobulin proteins are a mixture of various antibody types, and forming for unknown reasons (essential), the conditions is referred to as essential mixed cryoglobulinemia.

Essential mixed cryoglobulinemia is characterized by joint pains and swelling (arthritis), enlargement of the spleen, skin vasculitis with purplish patches, and nerve and kidney disease. This can lead to recurrent pain in the abdomen, heart attack, and bleeding in the lungs. Weight loss can occur as well as poor appetite.

Essential mixed cryoglobulinemia is sometimes associated with hepatitis C virus infection.

The prognosis and natural history of the illness is not predictable. Kidney damage can be serious and recent reports state that permanent failure of the kidney occurs in approximately 10% of patients. Death can occur, usually from serious heart disease, infection, or brain hemorrhage.

Essential mixed cryoglobulinemia is treated with combinations of medications which reduce inflammation and suppress the immune system. Medications used include nonsteroid antiinflammatory drugs (ibuprofen, aspirin, and others), cortisone preparations (prednisone, prednisolone), cyclophosphamide (Cytoxan), chlorambucil (Leukeran), and azathioprine (Imuran). Plasmapheresis (hemapheresis), a procedure whereby the blood's serum is replaced with saline (salt water), is also performed for severe symptoms.

Recent studies have demonstrated some benefit of using interferon-alpha for those patients with evidence of hepatitis C virus, particularly those with mild disease or in those with remission of manifestations after immune suppression treatment.