A Controlled Trial of High-Dose Intravenous Immune Globulin Infusions as Treatment for Dermatomyositis

Dalakas, Marinos C.; Illa, Isabel; Dambrosia, James M.; Soueidan, Shawke A.; Stein, Daniel P.; Otero, Carlos; Dinsmore, Steven; McCrosky, Susan

doi:10.1056/nejm199312303292704

Cited by 989 publications

(560 citation statements)

References 26 publications

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“…The MDS score in 3 of the 10 remaining patients partially improved, but their muscle power did not significantly improve. The benefit of IVIG in PM is similar to that observed in DM (23).…”

Section: Discussionsupporting

confidence: 74%

“…The majority of these published reports were case reports or small, open studies about adult and/or juvenile DM (10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22), and only 1 was a controlled crossover study in adult DM (23).…”

Section: Discussionmentioning

confidence: 99%

“…In DM, IVIG acts by preventing activated complement from damaging cutaneous and muscular lesions, inhibiting C3 uptake in the sera, reducing serum levels of SC5b-9 complex, depleting muscle from C3b NEO and membrane attack complex deposits from the endomysial capillaries, and restoring the capillary network (23)(24)(25). IVIG treatment led to a decrease in class I major histocompatibility complex and intercellular adhesion molecule 1 staining, which suggests that IVIG binds to Fc receptors on macrophages and leads to a decreased production of pathologically important cytokines (25).…”

Section: Discussionmentioning

confidence: 99%

“…High-dose intravenous immunoglobulin (IVIG) has been shown to be beneficial in a few open, prospective studies on adult and/or juvenile DM with short followup periods (8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22), and in 1 controlled crossover study (23). In DM, IVIG prevents activated complement from damaging cutaneous and muscular lesions, inhibits C3 uptake in the sera, reduces serum levels of the SC5b-9 complex, depletes C3b NEO (a neoantigen that is expressed on the surface of activated C3 component upon incorporation into immune complexes) in muscles, prevents membrane attack complex deposits from entering the endomysial capillaries, and restores the capillary network (23)(24)(25).…”

mentioning

confidence: 99%

“…In DM, IVIG prevents activated complement from damaging cutaneous and muscular lesions, inhibits C3 uptake in the sera, reduces serum levels of the SC5b-9 complex, depletes C3b NEO (a neoantigen that is expressed on the surface of activated C3 component upon incorporation into immune complexes) in muscles, prevents membrane attack complex deposits from entering the endomysial capillaries, and restores the capillary network (23)(24)(25). However, in PM, no controlled studies or significant cohort studies have been published.…”

mentioning

confidence: 99%

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Results and long‐term followup of intravenous immunoglobulin infusions in chronic, refractory polymyositis: An open study with thirty‐five adult patients

Chérìn

Pelletier

Teixeira

et al. 2002

Arthritis & Rheumatism

225

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Objective. Polymyositis is a rare inflammatory muscular disease of unknown cause. Corticosteroids and immunosuppressive drugs are the first choice of therapy but are not always effective and may cause serious side effects. Many studies have shown that polyvalent intravenous immunoglobulin (IVIG) may be of interest for the treatment of dermatomyositis. We carried out an open, prospective study to evaluate the efficacy of IVIG in subjects with polymyositis that was refractory to traditional treatments, and we evaluated the benefits of this therapy over a long-term period of followup.Methods. Thirty-five adult white patients (20 female, 15 male, mean age 43.5 years [SD 16.8]) with chronic, refractory polymyositis were treated with high doses of IVIG, after the patients had received the following traditional treatments: prednisone (n ‫؍‬ 35), methotrexate (n ‫؍‬ 24), azathioprine (n ‫؍‬ 13), cyclophosphamide (n ‫؍‬ 4), cyclosporine (n ‫؍‬ 7), chlorambucil (n ‫؍‬ 1), plasmapheresis (n ‫؍‬ 8), lymphopheresis (n ‫؍‬ 1), and total body irradiation (n ‫؍‬ 1). There had been no changes in the patients' treatment in the 2 months before the initiation of IVIG therapy, and doses were not increased during IVIG treatment. We used preparations of polyvalent human IVIG with increased concentrations of intact IgG. The patients received 1 gm/kg/day for 2 consecutive days per month. The mean course of treatment was 4-6 months. The clinical assessment involved the evaluation of proximal muscle power, muscle disability scale score, and esophageal disorders. The biochemical evaluations carried out before each treatment period were compared by Student's t-test and nonparametric Wilcoxon test. Results were considered to be significant at P ‫؍‬ 0.05.Results. In the short-term, significant clinical improvement was noted in 25 of the 35 patients (71.4%). Mean muscle power was estimated before and after IVIG therapy and was found to be significantly improved (P < 0.01). All patients had a significant biochemical response. Mean creatine kinase levels during IVIG therapy decreased significantly before the fourth IVIG perfusion (P < 0.01). Side effects, usually minor, were noted in 6 patients. This benefit allowed the initial prednisone dose to be reduced by >50% in all patients. The mean (؎SD) followup time for the 25 patients who responded favorably to IVIG treatment was 51.4 ؎ 13.1 months. Twelve of these 25 patients remained in full remission following their initial course of IVIG, resulting in complete stoppage of medication in 5 patients or low doses of steroids in 7 patients. The condition of 6 patients remained improved and no other drugs were prescribed, but the patients remained dependent on IVIG infusions. Seven of the 25 patients who responded well to IVIG treatment relapsed at an average of 17.1 months (range 4-23 months) after the discontinuation of IVIG. Conclusion. IVIG is an interesting therapy for the treatment of polymyositis, with results showing that the

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“…The MDS score in 3 of the 10 remaining patients partially improved, but their muscle power did not significantly improve. The benefit of IVIG in PM is similar to that observed in DM (23).…”

Section: Discussionsupporting

confidence: 74%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Results and long‐term followup of intravenous immunoglobulin infusions in chronic, refractory polymyositis: An open study with thirty‐five adult patients

Chérìn

Pelletier

Teixeira

et al. 2002

Arthritis & Rheumatism

225

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Dermatological Uses of High-Dose Intravenous Immunoglobulin

Jolles¹,

Hughes²,

Whittaker³

1998

Arch Dermatol

141

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Mycophenolate Mofetil as an Effective Corticosteroid-Sparing Therapy for Recalcitrant Dermatomyositis

Edge¹,

Outland²,

Dempsey³

et al. 2006

Arch Dermatol

114

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Background: Dermatomyositis (DM) is a multisystem idiopathic inflammatory disorder that most commonly affects the muscles and skin. Systemic corticosteroids are the mainstay of therapy but are limited by their longterm adverse effects.Observations: We sought to evaluate the effectiveness of oral mycophenolate mofetil in patients with cutaneous lesions of DM recalcitrant to other therapies through an open-label retrospective medical chart review of patients in a university-affiliated private practice setting. Twelve patients with DM who had skin lesions recalcitrant to traditional therapies or who developed toxic effects from traditional therapies began mycophenolate mofetil treatment at doses ranging from 500 mg to 1 g twice a day. Response was based on improvement in skin disease as judged clinically, an increase in strength, and/or an ability to decrease or discontinue concomitant therapies. Improvement was seen in 10 of the 12 patients, most within 4 to 8 weeks. Most patients tolerated mycophenolate mofetil treatment without problem; however, 1 patient developed a B-cell lymphoma of the central nervous system, and another developed abnormal levels of hepatic enzymes along with urinary symptoms. Resolution of these toxic reactions occurred with cessation of mycophenolate mofetil treatment in each patient. Conclusion:Mycophenolate mofetil may be an effective corticosteroid-sparing therapy for the treatment of some patients with DM.

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A Controlled Trial of High-Dose Intravenous Immune Globulin Infusions as Treatment for Dermatomyositis

Abstract: High-dose intravenous immune globulin is a safe and effective treatment for refractory dermatomyositis.

Cited by 989 publications

References 26 publications

Results and long‐term followup of intravenous immunoglobulin infusions in chronic, refractory polymyositis: An open study with thirty‐five adult patients

Results and long‐term followup of intravenous immunoglobulin infusions in chronic, refractory polymyositis: An open study with thirty‐five adult patients

Dermatological Uses of High-Dose Intravenous Immunoglobulin

Mycophenolate Mofetil as an Effective Corticosteroid-Sparing Therapy for Recalcitrant Dermatomyositis

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