Objective To report the clinical outcome and long-term follow-up of 10 patients with progressive ocular-cicatricial pemphigoid (OCP), nonresponsive to conventional therapy and treated with IV immunoglobulin (IVIg) therapy, reported earlier as a preliminary study. Design Noncomparative, prospective, interventional case series according to a defined protocol for IVIg therapy. Participants Ten patients, with a diagnosis of OCP present bilaterally confirmed by both biopsy and immunofluorescence studies and who had failed conventional therapy and had objectively demonstrated a positive response to IVIg therapy in a preliminary study, published in 1999. Main outcome measures Comparison of objective clinical outcome parameters before and after IVIg therapy, including visual acuity (VA) and prevention of progression of subepithelial conjunctival fibrosis and blindness. Results All 10 patients initially demonstrated signs of clinical improvement with IVIg therapy. 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). Eight patients who completed the protocol had an improvement in their VA and did not have further progression of subepithelial conjunctival fibrosis. These 8 patients have been maintained in a sustained remission for a total follow-up period ranging from 24 to 48 months (mean, 35) after the discontinuation of IVIg therapy. Two patients did not complete the protocol. Both had initially demonstrated a positive clinical response. One patient had worsening of the OCP and, after IVIg therapy, was abruptly and involuntarily withdrawn. In the second patient, deterioration occurred after ocular surgery. Intravenous immunoglobulin therapy was not provided postoperatively. These 2 patients who did not complete the protocol lost vision. Conclusions Intravenous immunoglobulin therapy is an effective treatment in OCP in patients nonresponsive to conventional therapy. In 8 patients who completed the protocol, progression of the disease was not observed. 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.
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