We report 2 patients with scleromyxedema, both associated with IgG-lambda paraproteinemia, who were treated with high-dose intravenous immunoglobulin (hdIVIg) 2 g/kg per month. The response to treatment was assessed u...
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We report 2 patients with scleromyxedema, both associated with IgG-lambda paraproteinemia, who were treated with high-dose intravenous immunoglobulin (hdIVIg) 2 g/kg per month. The response to treatment was assessed using an objective skin scoring system initially established for patients with scleroderma. This system grades the overall severity of the induration and the reduction in mobility of the skin. Both patients initially had a dramatic response to treatment which was sustained in one patient. The first patient, a 30-year-old black man, showed a reduction in skin scores from 36/60 to 11/60 over a 3-month period, during which time he had 3 infusions of hdIVIg. After an unplanned 2-month break from treatment, severe neuromuscular complications developed. These improved initially with more frequent infusions of hdIVIg but oral corticosteroids were required to treat worsening myopathy. Unfortunately the initial response to hdIVIg has not been sustained and his skin scores at 1 year returned to baseline. The second patient, a 60-year-old white man, showed a similarly dramatic reduction in skin scores from 36/60 to 15/60 over a 3-month period after having received only 2 infusions of hdIVIg. There has been sustained improvement after 10 months of therapy and the interval between hdIVIg infusions has been increased to 10 weeks without deterioration. HdIVIg may be an effective treatment for some patients with scleromyxedema, a rare condition with few effective treatments and a poor prognosis.
Chronic dysimmune neuropathies are less common than Guillain-Barre syndrome (GBS). They mainly comprise chronic idiopathic demyelinating polyneuropathy (CIDP), multifocal motor neuropathy with persistent conduction bl...
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Chronic dysimmune neuropathies are less common than Guillain-Barre syndrome (GBS). They mainly comprise chronic idiopathic demyelinating polyneuropathy (CIDP), multifocal motor neuropathy with persistent conduction blocks (MMNCB) and polyneuropathy associated with monoclonal gammopathy. However, as GBS, they are considered to be immune-mediated disorders and they may respond to various immunosuppressive treatments, such as corticosteroids, plasma exchanges (PE) or intravenous immunoglobulins (IVIg). The first step is to characterize these neuropathies on clinical, electrophysiological and sometimes immunochemical criteria, because the response to treatment may be different according to the type of neuropathy. For example. polyneuropathy associated with IgM monoclonal gammopathy does not respond to steroids and MMNCB may worsen under PE, while CIDP may respond either to steroids, PE or IVIg. The second step is to choose the type and the regimen of the treatment. Some neurological conditions may require only short-term therapy, for example in relapsing CIDP, whereas other conditions may require long-term therapy. (C) 1999 Elsevier Science Ltd. All rights reserved.
Immunochemical and serologic studies of cold agglutinins in patients with chronic cold agglutinin disease (CCAD) showed the almost exclusive occurrence of Ig[immunoglobulin]M .kappa. antibodies with specificity for th...
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Immunochemical and serologic studies of cold agglutinins in patients with chronic cold agglutinin disease (CCAD) showed the almost exclusive occurrence of Ig[immunoglobulin]M .kappa. antibodies with specificity for the I antigen of red cells. An unusual subgroup of patients was delineated in which the cryoprotein is IgM .lambda., frequently lacks I specificity and often cryoprecipitates. Studies of such a protein from a patient with an unusual array of immunoproliferative disorders including Grave''s disease with exophthalmos and Waldenstrom''s macroglobulinemia indicate that the cryoprecipitating and cold agglutinating properties probably derive from the same protein. The occurrence of this type of antibody should suggest the presence of a more aggressive lymphoproliferative disorder than simple CCAD.
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