title: High dose intravenous immunoglobulin (IVIG) in dermatomyositis: clinical responses and effect on sIL-2R levels.
abstract: An open study was conducted to identify and investigate dermatomyositis patients who benefit from IVIG treatment, based on dermatological criteria, myositis-related symptoms and immune/inflammatory parameters. 19 patients (16 females and three males, ages 31-84) suffered from dermatomyositis, and 4/19 patients had paraneoplastic dermatomyositis. We monitored the disease activity by documenting the clinical symptoms, recording muscle-related parameters (electromyography, serum creatine kinase, histopathology), and by determining circulating autoantibodies and serum levels of IL-6, sIL-2R, sTNF-a-R, sICAM-1, and sCD8. 7/19 patients responded to IVIG. They had severe skin but only moderate muscle involvement, no autoantibodies, and no malignancy. IVIG-nonresponders had severe skin and muscle disease, concomitant with autoantibodies and/or malignancy. sIL-2R levels were initially elevated in all patients but reverted to normal in IVIG-responders only. Creatine kinase-levels and other parameters did not correlate with disease activity and/or treatment response. IVIG is effective in selected dermatomyositis patients. sIL-2R serum levels appear to be useful predictors of IVIG-induced treatment response and disease activity.
authors.affiliation: Division of Immunology, Allergy and Infectious Diseases, Department of Dermatology, University of Vienna, Austria. beatrice.volc@akh-wien.ac.at
authors.forename: I
authors.initials: I
authors.lastname: Göttfried
authors.forename: A
authors.initials: A
authors.lastname: Seeber
authors.forename: B
authors.initials: B
authors.lastname: Anegg
authors.forename: A
authors.initials: A
authors.lastname: Rieger
authors.forename: G
authors.initials: G
authors.lastname: Stingl
authors.forename: B
authors.initials: B
authors.lastname: Volc-Platzer
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood; D016827:CD8 Antigens / Q000097:blood; D003402:Creatine Kinase / Q000097:blood; D003882:Dermatomyositis / Q000097:blood / Q000188:drug therapy* / Q000503:physiopathology; D004576:Electromyography; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D018799:Intercellular Adhesion Molecule-1 / Q000097:blood; D015850:Interleukin-6 / Q000097:blood; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000097:blood / Q000188:drug therapy* / Q000503:physiopathology; D015375:Receptors, Interleukin-2 / Q000097:blood*; D018124:Receptors, Tumor Necrosis Factor / Q000097:blood
chemical_list: D001323:Autoantibodies; D016827:CD8 Antigens; D016756:Immunoglobulins, Intravenous; D015850:Interleukin-6; D015375:Receptors, Interleukin-2; D018124:Receptors, Tumor Necrosis Factor; D018799:Intercellular Adhesion Molecule-1; D003402:Creatine Kinase