six months prior to her initial visit in our clinic, the patient developed a recurrence of her heliotrope rash and gottron’s papules of her hands and knees. She also experienced worsening proximal muscle weakness, with difficulty brushing her hair, walking up and down stairs and standing up from a seated position. Magnetic resonance imaging mri of her thighs revealed diffuse t2 signal abnormalities bilaterally, consistent with myositis. Tests with abnormal results included a creatine kinase of 383 u/l 60–230 u/l , aldolase of 13.2 u/l 1.2–11.8 u/l , lactate dehydrogenase of 1,042 u/l 340–700 u/l , neopterin of 71.4 nmol/l 2–10 nmol/l , and immunoglobulin g igg of 1,810 mg/dl 524–1,403 mg/dl. In view of these findings, the patient was started on a new treatment regimen that consisted of subcutaneous methotrexate at a higher dose, oral corticosteroids, and azathioprine. Nevertheless, the patient continued to have a rash and progressive muscle weakness. A decision was therefore made to start treatment with intravenous pulse corticosteroids and monthly high dose intravenous immunoglobulin ivig infusions.

Immediately following the start of this treatment, the patient noticed marked improvement in both her rash and strength. Her muscle enzymes normalized after three months of ivig therapy, and she was able to ride a bicycle for the first time in two years. Her cutaneous findings subjectively improved after four months of ivig therapy, and her neopterin level normalized after 12 months.

She maintained remission on monthly high dose ivig infusions and stable methotrexate dosing with continued tapering of the oral corticosteroids. Her ivig therapy was discontinued after 14 total infusions, and her jdms has remained quiescent on subcutaneous methotrexate and hydroxychloroquine. Ivig is a highly purified globulin preparation obtained from the pooled plasma of thousands of healthy donors. Although initially given as replacement therapy for patients with primary and secondary immunodeficiency states, ivig has proven to be effective in the treatment of various autoimmune and inflammatory disorders. This success has led to a dramatic increase in the use of ivig, with its use as an antiinflammatory agent now vastly surpassing its use in the treatment of immunodeficiencies. In fact, approximately 70% of ivig administered today is for immunomodulation in patients with autoimmune disease. While multiple modes of action have been proposed, there may not be a single effect and the different proposed actions may not be mutually exclusive.

This article will review some of these mechanisms, preceded by a brief discussion of clinically relevant aspects of ivig therapy.

posted by sara fazio bull november 23rd, 2012

the latest article in our mechanisms of disease series comes from dr. Erwin gelfand at national jewish health in denver, who reviews the many proposed mechanisms by which intravenous immune globulin may exert its antiinflammatory and autoimmunity inhibiting clinical effects. No single mechanism can explain its activity in diseases with diverse pathophysiological pathways. In an era in which new biologics are being introduced to target inflammation and autoimmunity, some older treatments persist.

Immune globulin replacement therapy has been a lifesaving treatment for patients with antibody deficiency. how is intravenous immune globulin prepared? intravenous immune globulin is prepared from plasma pooled from thousands of healthy donors. This pooling provides a diversity of antibody repertoires and antibody specificities. More than a dozen preparations suitable for intravenous administration have been approved by the food and drug administration fda for the treatment of primary immunodeficiency diseases. what are the fda approved indications for the use of intravenous immune globulin? currently, immune globulin is used in the treatment of a wide variety of diseases, with more than 75% of the intravenous immune globulin in the united states administered to patients with autoimmune or inflammatory conditions. At present, the fda approved indications for immune globulin therapy include primary immunodeficiency disease, chronic lymphocytic leukemia, pediatric hiv infection, kawasakis disease, allogeneic bone marrow transplantation, and chronic inflammatory demyelinating polyneuropathy. The use of intravenous immune globulin is also now accepted for patients undergoing kidney transplantation when the recipient has a high antibody titer or when the donors blood is abo incompatible.

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Additional approved indications with limitations include a variety of neuromuscular e.g. q: what is the potential role for the use of ivig in alzheimers syndrome? a: one area of growing interest is the potential use of intravenous immune globulin in patients with alzheimers syndrome. Passive immunotherapy with the use of anti beta amyloid a beta antibodies was attempted e.g. Monoclonal antibodies such as bapineuzumab , but this approach had limited success.

Ivig Review Article Text

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posted by sara fazio bull november 23rd, 2012

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