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E.A. Khan, N. Sami, C. Foster, A. Ahmed; Intravenous Immunoglobulin Therapy in the Treatment of Refractory Ocular Cicatricial Pemphigoid: A Long-term Follow-up . Invest. Ophthalmol. Vis. Sci. 2003;44(13):2419.
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Purpose: The purpose is to report a long-term follow-up of the effect of intravenous immunogloublin in 10 patients with progressive ocular cicatricial pemphigoid who did not respond to conventional immunomodulatory regimens. Methods: This is a prospective study of a case series of 10 patients who were refractory to local and systemic immunosuppressive agents and had responded to IVIg therapy. All 10 patients were treated according to a protocol recently described. This study is a follow-up of the 10 patients after initial control of the disease was established and the IVIg therapy was tapered and discontinued. The patients were followed for a minimum period of 12 months after IVIg therapy was discontined. The clinical outcome measures which were studied were stage of the disease (according to Foster’s criteria for OCP) and visual acuity. Results: : All 10 patients initally responded to IVIg therapy and all systemic agents were discontinued. Once control was established the intervals between IVIg therapy were prolonged and IVIg therapy was discontinued in 9 patients. In one patient, IVIg therapy was abruptly discontinued. In a mean follow-up of 18 months after the discontinuation of IVIg therapy, visual acuity improved or remained the same in 8 of the 10 patients. In 6 patients, the stage of the disease remained the same. Two patients had progressive disease and progressed to stage IV. In one patient, the progression was due to abrupt interruption of IVIg therapy and in one patient, the disease was aggravated by surgery Conclusions: In patients with severe OCP who are treated with a recently described protocol of IVIg therapy, IVIg induces a sustained clinical remission. IVIg therapy is an effective alternative in the treatment of OCP
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