A Controlled Trial of High-Dose Intravenous Immune Globulin Infusions as Treatment for Dermatomyositis

• Published in: The New England journal of medicine Vol. 329; no. 27; pp. 1993 - 2000
• Main Authors: Dalakas, Marinos C, Illa, Isabel, Dambrosia, James M, Soueidan, Shawke A, Stein, Daniel P, Otero, Carlos, Dinsmore, Steven T, McCrosky, Susan
• Format: Journal Article
• Language: English
• Published: Boston, MA Massachusetts Medical Society 30.12.1993
• Subjects: Activities of daily living; Adolescent; Adult; Antigens; Biological and medical sciences; Biopsy; Body weight; Capillaries; Cardiovascular disease; Cell adhesion; Clinical trials; Complement system; Dermatomyositis; Dermatomyositis - drug therapy; Dermatomyositis - pathology; Dermatomyositis - therapy; Dietary fiber; Double-Blind Method; Exanthema; Female; Follow-Up Studies; Globulins; Humans; Immunoglobulins; Immunoglobulins, Intravenous - therapeutic use; Immunomodulators; Immunotherapy; Intercellular adhesion molecule 1; Intravenous administration; Male; Medical disorders; Medical research; Medical sciences; Middle Aged; Muscle strength; Muscles; Muscles - pathology; Myopathy; Neuromuscular diseases; Patients; Pharmacology. Drug treatments; Prednisone; Prednisone - therapeutic use; Skin; Human; Skin disease; Chemotherapy; Immunoglobulins; Treatment; Intravenous administration; Immunotherapy; Systemic disease; Dermatomyositis; Autoimmune disease
• ISSN: 0028-4793; 1533-4406
• DOI: 10.1056/NEJM199312303292704

Polymyositis, dermatomyositis, and inclusion-body myositis are distinct groups of inflammatory myopathies, each with characteristic immune-mediated mechanisms 1 – 6 . In polymyositis and inclusion-body myositis, sensitized CD8+ cytotoxic T cells recognize heretofore unidentified muscle antigens, leading to phagocytosis and fiber necrosis 1 , 7 – 9 . Dermatomyositis, a clinically distinct entity because of the rash it causes, is characterized by an intramuscular microangiopathy mediated by the complement C5b-9 membranolytic attack complex, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy 10 – 14 . In spite of its distinct characteristics, however, dermatomyositis has not been treated separately but as part of the inflammatory-myopathy . . .