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.