Controlled studies with high-dose intravenous immunoglobulin in the treatment of dermatomyositis, inclusion body myositis, and polymyositis. 1998

M C Dalakas
Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health Bethesda, MD 20892-1382, USA.

There are three major subsets of the inflammatory myopathies: polymyositis (PM), dermatomyositis (DM), and inclusion-body myositis (IBM). High-dose intravenous immunoglobulin (IVIg) has been tried in controlled clinical trials in patients with DM and IBM but not with PM. In patients with DM that is resistant or partially responsive to conventional therapies, IVIg was very effective. The treated patients experienced dramatic improvement not only in muscle strength but also of their skin rash. Repeated muscle biopsies with quantitative histologic studies showed the IVIg-treated patients had a statistically significant improvement of the muscle cytoarchitecture, with resolution of the aberrant immunopathologic parameters. In two controlled clinical trials conducted in IBM patients, IVIg showed marginal improvements in muscle strength which were nonsignificant. However, a few IBM patients had a definite clinical improvement with increased activities of daily living, but when analyzed within the entire IVIg-treated group, their total gains in muscle strength did not reach statistical significance compared to the placebo-treated group. Of interest is that certain muscle groups in the IVIg-treated patients, such as the muscles of swallowing, showed significant improvement compared to those of the placebo-treated patients, implying mild regional effects. In PM, uncontrolled trials have shown improvements in muscle strength, but the controlled clinical trial is still ongoing.

UI MeSH Term Description Entries
D003882 Dermatomyositis A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. (From Adams et al., Principles of Neurology, 6th ed, pp1405-6) Polymyositis-Dermatomyositis,Dermatomyositis, Adult Type,Dermatomyositis, Childhood Type,Dermatopolymyositis,Juvenile Dermatomyositis,Juvenile Myositis,Adult Type Dermatomyositis,Childhood Type Dermatomyositis,Dermatomyositis, Juvenile,Myositis, Juvenile,Polymyositis Dermatomyositis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D016756 Immunoglobulins, Intravenous Immunoglobulin preparations used in intravenous infusion, containing primarily IMMUNOGLOBULIN G. They are used to treat a variety of diseases associated with decreased or abnormal immunoglobulin levels including pediatric AIDS; primary HYPERGAMMAGLOBULINEMIA; SCID; CYTOMEGALOVIRUS infections in transplant recipients, LYMPHOCYTIC LEUKEMIA, CHRONIC; Kawasaki syndrome, infection in neonates, and IDIOPATHIC THROMBOCYTOPENIC PURPURA. Antibodies, Intravenous,Human Intravenous Immunoglobulin,IV Immunoglobulin,IVIG,Intravenous Antibodies,Intravenous Immunoglobulin,Intravenous Immunoglobulins,Alphaglobin,Endobulin,Flebogamma DIF,Gamimmune,Gamimmune N,Gamimune,Gamimune N,Gammagard,Gammonativ,Gamunex,Globulin-N,IV Immunoglobulins,Immune Globulin Intravenous (Human),Immune Globulin, Intravenous,Immunoglobulins, Intravenous, Human,Intraglobin,Intraglobin F,Intravenous IG,Intravenous Immunoglobulins, Human,Iveegam,Modified Immune Globulin (Anti-Echovirus Antibody),Privigen,Sandoglobulin,Venimmune,Venoglobulin,Venoglobulin-I,Globulin N,Human Intravenous Immunoglobulins,Immunoglobulin, Human Intravenous,Immunoglobulin, IV,Immunoglobulin, Intravenous,Immunoglobulins, Human Intravenous,Immunoglobulins, IV,Intravenous Immune Globulin,Intravenous Immunoglobulin, Human,Venoglobulin I
D017285 Polymyositis Diseases characterized by inflammation involving multiple muscles. This may occur as an acute or chronic condition associated with medication toxicity (DRUG TOXICITY); CONNECTIVE TISSUE DISEASES; infections; malignant NEOPLASMS; and other disorders. The term polymyositis is frequently used to refer to a specific clinical entity characterized by subacute or slowly progressing symmetrical weakness primarily affecting the proximal limb and trunk muscles. The illness may occur at any age, but is most frequent in the fourth to sixth decade of life. Weakness of pharyngeal and laryngeal muscles, interstitial lung disease, and inflammation of the myocardium may also occur. Muscle biopsy reveals widespread destruction of segments of muscle fibers and an inflammatory cellular response. (Adams et al., Principles of Neurology, 6th ed, pp1404-9) Myositis, Multiple,Polymyositis Ossificans,Polymyositis, Idiopathic,Idiopathic Polymyositides,Idiopathic Polymyositis,Multiple Myositis,Myositides, Multiple,Ossificans, Polymyositis,Polymyositides,Polymyositides, Idiopathic
D018979 Myositis, Inclusion Body Progressive myopathies characterized by the presence of inclusion bodies on muscle biopsy. Sporadic and hereditary forms have been described. The sporadic form is an acquired, adult-onset inflammatory vacuolar myopathy affecting proximal and distal muscles. Familial forms usually begin in childhood and lack inflammatory changes. Both forms feature intracytoplasmic and intranuclear inclusions in muscle tissue. (Adams et al., Principles of Neurology, 6th ed, pp1409-10) Inclusion Body Myositis,Myopathy, Inclusion Body, Sporadic,Inclusion Body Myopathy, Sporadic,Inclusion Body Myositis, Sporadic,Myositis, Inclusion Body, Sporadic,Sporadic Inclusion Body Myositis,Inclusion Body Myositides,Myositides, Inclusion Body
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