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N. Sami, A.R. Ahmed; Results of Intravenous Immunoglobulin Therapy in Ocular involvement in Epidermolysis Bullosa Acquisita . Invest. Ophthalmol. Vis. Sci. 2003;44(13):1388.
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Purpose: The purpose of this study is to report treatment outcomes with intravenous immunoglobulin (IVIg) therapy in three patients with EBA with severe ocular involvement. The patients failed to respond to a combination of a prolonged usage of optimal doses multiple conventional agents. Methods: All three patients received an IVIg dose of 1-2 mg/kg per cycle. The parameters used to assess the response to IVIg therapy included time observed for effective control of disease, duration of IVIg maintenance therapy, total duration of IVIg, number of IVIg cycles, systemic drug therapy, and frequency of recurrences and relapses. The differences in the following variables were statistically analyzed using the SAS UNIVARIATE software running the two sided Wilocoxon signed rank test pre- and post-IVIg therapy: number of side effects, frequency of recurrences and relapses, duration and total dosage of prednisone therapy, and the quality of life. Results: All patients had an effective clinical response to IVIg therapy and were able to discontinue all previous systemic therapies. The difference between Pre- and Post-IVIg therapy were statistically significant for the number of side effects, frequency of recurrences and relapses, duration and total dosage of prednisone, and the quality of life. One patient achieved a sustained and prolonged clinical remission and is off all systemic therapies. The two other patients continue to receive IVIg as monotherapy at increased intervals while maintaining an effective clinical control of their disease. IVIg improved the quality of life in all patients. and demonstrated a steroid sparing effect. No serious side effects were observed Conclusions: IVIg therapy appears to be a safe biologic alternative in patients with EBA unresponsive to conventional agents. IVIg is effective as monotherapy and may be needed for a period of several months to achieve a long-term clinical remission.
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