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

Abstract

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

Pregnant patient with dermatomyositis successfully treated with intravenous immunoglobulin therapy

“…It also is used in the treatment of other pregnancy complications, such as idiopathic thrombocytopenic purpura and recurrent miscarriages, and appears to be safe and well tolerated by pregnant patients (11,12). In our patient, treatment with IVIG was aimed at maintaining disease remission while decreasing the corticosteroid dosage.…”

“…IVIG therapy has been shown to be very effective in the treatment of IIMs, in particular DM, and is widely used for various autoimmune conditions during pregnancy (11). It also is used in the treatment of other pregnancy complications, such as idiopathic thrombocytopenic purpura and recurrent miscarriages, and appears to be safe and well tolerated by pregnant patients (11,12).…”

A Case of Refractory Dysphagia in the Context of Polymyositis Treated with Subcutaneous Immunoglobulin and Review of the Literature

“…Eleven subjects presented esophageal disorders (including dysphagia, regurgitation and gastroesophageal reflux) evaluated with clinical assessment and manometry. In 8/11 (73%) patients they observed the complete disappearance of esophageal disorder [12].…”

Long-term efficacy of intravenously administered immunoglobulin in a case of polymyositis with limited application of steroid therapy

“…Furthermore, long-term effects on CK levels after IVIG were reported, with 50% of patients achieving normal CK levels for more than 300 days. With regard to the longterm effects of IVIG in PM, Cherin et al [7] previously reported 35 chronic refractory cases of PM. In this study, 12 out of 25 responders to IVIG maintained full remission for 51.4 ± 13.1 months after the initial IVIG.…”