High dose intravenous immunoglobulin (IVIG) in dermatomyositis: clinical responses and effect on sIL-2R levels

An open study was conducted to identify and investigate dermatomyositis pat ients who benefit from IVIG treatment, based on dermatological criteria, my ositis-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 ac tivity by documenting the clinical symptoms, recording muscle-related param eters (electromyography, serum creatine kinase, histopathology), and by det ermining circulating autoantibodies and serum levels of TL-6, sIL-2R, sTNF- alpha-R, sICAM-1, and sCD8. 7/19 patients responded to IVIG. They had severe skin but only moderate mus cle involvement, no autoantibodies, and no malignancy. IVIG-nonresponders h ad severe skin and muscle disease, concomitant with autoantibodies and/or m alignancy. sIL-2R levels were initially elevated in all patients but revert ed to normal in IVIG-responders only. Creatine kinase-levels and other para meters 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 dise ase activity.