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



John M. Cafardi1, Naveed Sami*, 2
1 The Christ Hospital and The University of Cincinnati Medical Center, USA
2 Department of Dermatology, The University of Alabama at Birmingham, USA


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© Cafardi and Sami ; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the University of Alabama at Birmingham, 1520 3rd Ave South, EFH 414, Birmingham, AL 35294-0009, USA; Tel: 205-934-5188; Fax: 205-996-7546; E-mail: nsami@uabmc.edu


Abstract

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.

Keywords: Intravenous immunoglobulin, treatment, amyopathic dermatomyositis, direct immunofluorescence.