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

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          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.

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          Intravenous immune globulin in autoimmune and inflammatory diseases.

          Erwin Gelfand (2012)
          Article 0 comments Cited 132 times – based on 0 reviews     Review now
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            A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis.

            Joshua Stein, Dennis Otero, A Soueidan … (1993)
            Dermatomyositis is a clinically distinct myopathy characterized by rash and a complement-mediated microangiopathy that results in the destruction of muscle fibers. In some patients the condition becomes resistant to therapy and causes severe physical disabilities. We conducted a double-blind, placebo-controlled study of 15 patients (age, 18 to 55 years) with biopsy-proved, treatment-resistant dermatomyositis. The patients continued to receive prednisone (mean daily dose, 25 mg) and were randomly assigned to receive one infusion of immune globulin (2 g per kilogram of body weight) or placebo per month for three months, with the option of crossing over to the alternative therapy for three more months. Clinical response was gauged by assessing muscle strength, neuromuscular symptoms, and changes in the rash. Changes in immune-mediated muscle abnormalities were determined by repeated muscle biopsies. The eight patients assigned to immune globulin had a significant improvement in sores of muscle strength (P < 0.018) and neuromuscular symptoms (P < 0.035), whereas the seven patients assigned to placebo did not. With crossovers a total of 12 patients received immune globulin. Of these, nine with severe disabilities had a major improvement to nearly normal function. Their mean muscle-strength scores increased from 74.5 to 84.7, and their neuromuscular symptoms improved. Two of the other three patients had mild improvement, and one had no change in his condition. Of 11 placebo-treated patients, none had a major improvement, 3 had mild improvement, 3 had no change in their condition, and 5 had worsening of their condition. Repeated biopsies in five patients of muscles whose strength improved to almost normal showed an increase in muscle-fiber diameter (P < 0.04), an increase in the number and a decrease in the diameter of capillaries (P < 0.01), resolution of complement deposits on capillaries, and a reduction in the expression of intercellular adhesion molecule 1 and major-histocompatibility-complex class I antigens. High-dose intravenous immune globulin is a safe and effective treatment for refractory dermatomyositis.
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              Dermatomyositis.

              D Callen (2000)
              Dermatomyositis is one of the idiopathic inflammatory myopathies with characteristic cutaneous manifestations including the heliotrope rash, Gottron's papules, cuticular changes including periungual telangiectasia, a photodistributed erythema or poikiloderma, and a scaly alopecia. Dermatomyositis has been linked to cancer, particularly ovarian cancer. Cancer-associated disease is more commonly found in older patients, and when present, is associated with a poor prognosis. A childhood form of the disease exists and is frequently complicated by the development of calcinosis. Dermatomyositis is a systemic disorder and whereas the skin and muscles are the most commonly affected organs, patients may have arthralgias, arthritis, oesophageal disease, or cardiopulmonary dysfunction. Recently described serological abnormalities, known as myositis-specific antibodies, add credence to the notion that this disorder is distinct from all other collagen-vascular diseases, and may lead to important discoveries about the pathogenesis of the inflammatory myopathies, which are not currently of practical use in the clinic or office. Management of the patient with myositis usually includes systemic corticosteroids with or without an immunosuppressive agent. Cutaneous disease is more difficult to manage, but antimalarials, methotrexate, and intravenous immunoglobulin are effective in small, often open-label, studies.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Open Rheumatol J
                Journal ID (iso-abbrev): Open Rheumatol J
                Journal ID (publisher-id): TORJ
                Title: The Open Rheumatology Journal
                Publisher: Bentham Open
                ISSN (Electronic): 1874-3129
                Publication date (Electronic): 4 November 2015
                Publication date Collection: 2015
                Volume: 9
                Pages: 77-81
                Affiliations
                [1 ]The Christ Hospital and The University of Cincinnati Medical Center, USA
                [2 ]Department of Dermatology, The University of Alabama at Birmingham, USA
                Author notes
                [* ]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@ 123456uabmc.edu
                Article
                Publisher ID: TORJ-9-77
                DOI: 10.2174/1874312901409010077
                PMC ID: 4676053
                SO-VID: 2741d4d2-29b1-4310-8a8b-fb43093a3b25
                Copyright © © Cafardi and Sami ; Licensee Bentham Open.
                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.

                History
                Date received : 23 April 2015
                Date revision received : 23 September 2015
                Date accepted : 23 September 2015
                Categories
                Subject: Article

                ScienceOpen disciplines: Rheumatology
                Keywords: intravenous immunoglobulin,treatment,amyopathic dermatomyositis,direct immunofluorescence.
                Data availability:
                ScienceOpen disciplines: Rheumatology
                Keywords: intravenous immunoglobulin, treatment, amyopathic dermatomyositis, direct immunofluorescence.

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