Intravenous Immune Globulin in Amyopathic Dermatomyositis - Report of Two Cases and Review of the Literature

Amyopathic dermatomyositis (ADM) is a rare subtype of dermatomyositis which is often recalcitrant to immune suppressing treatments. Intravenous immunoglobulin (IVIG) has been used in the treatment of refractory dermatomyositis. We present two patients with severe ADM, who were treated with IVIG at 2 g/kg every four weeks. Both patients had a successful response and were able to taper the dosage of prednisone. We present both cases in describing IVIG as a rescue and maintenance steroid-sparing agent in the treatment of severe refractory ADM. We also review the treatment of refractory ADM with IVIg in the English literature.