Successful treatment of dermatomyositis with IgG-apheresis

Objective: We report on a 9-year-old girl suffering from dermatomyositis (DM). DM is difficult to treat as the therapeutic options are limited to largely empirical and partial toxic therapy strategies. Since DM is associated with the production of autoantibodies, IgG-apheresis should be considered to be an effective therapy concept.

Methods: First-line treatment with prednisolone, followed by intravenous immunoglobuline therapy had inadequate clinical reponse. Thus, IgG-apheresis therapy was administered to remove the pathogenic antibodies. In total 20 sessions of immunoapheresis (Ig-Therasorb) were performed, each processing the 2.5 fold plasma volume (on average 3.700ml) within 2–2.5 hours.

Results: IgG-apheresis therapy resulted in a decrease of IgG levels below the detection limit (<195mg/dl), thereby enabling the effective reduction of disease related autoantibodies, e.g. ASMA titre from 1:320 to 0. After only 4 procedures of IgG-apheresis the serum creatine kinase level was within the normal range. After 18 sessions (within a period of 18 weeks) clinical remission was achieved without additional steroid therapy. Mild clinical deterioration occured 11 months later, therefore, another 2 apheresis sessions were performed. In the follow-up period of meanwhile 35 months the patient remained free of symptoms.

Conclusions: By this case report we refer IgG-apheresis as a new effective and safe therapeutical approach to treat patients suffering from dermatomyositis when conventional therapies failed.

Keywords: dermatomyositis, IgG-apheresis, immunoapheresis