Effectiveness of Intravenous Immunoglobulin Therapy in Patients with Ocular Cicatricial Pemphigoid– long–term follow–up study

Abstract

Abstract: : Purpose: Ten biopsy–proven cases of ocular cicatricial pemphigoid (OCP), who had failed to respond by conventional therapy, were treated with intravenous immunoglobulin (IVIg), according to a defined therapeutic protocol. We will present long–term (mean: 35 months) clinical outcome of these patients. Methods: Clinical outcome of the patients with OCP in pre– and post–IVIg therapy, including: visual acuity, inflammation and progression of subepithelial conjunctival fibrosis were objectively compared. Results: All 10 patients initially demonstrated signs of clinical improvement with IVIg therapy, i.e. abolition of clinically obvious inflammation. The total number of IVIg cycles ranged from 20 to 42 (mean 32), and the total duration of IVIg therapy ranged from 25 to 43 months (mean: 35 months). Eight patients who completed the protocol had improvement in visual acuity and did not have further progression of subepithelial conjunctival fibrosis. These eight patients have been maintained in sustained remission for a total follow–up period ranging from 24 to 48 months (mean: 35 months) after the discontinuation of IVIg therapy. Two patients did not complete the protocol. Both had initially demonstrated positive clinical response. One patient had worsening of the clinical features, after IVIg therapy was abruptly and involuntarily discontinued. In the second patient, deterioration occurred after the patient had undergone ocular surgery. IVIg therapy was not provided post–operatively. Both the patients who did not complete the protocol lost vision. Conclusions: IVIg therapy is an effective treatment option for patients who are non–responsive to conventional therapy. In our study, all the 8 patients of OCP who completed the protocol, showed significant clinical improvement. A gradual withdrawal of IVIg therapy, as described in the protocol, may be beneficial in maintaining a sustained clinical remission. Abrupt cessation or discontinuation can result in a severe recurrence that may possibly progress to blindness.

Keywords: autoimmune disease • immunomodulation/immunoregulation • inflammation