Abstract
Purpose :
Ocular cicatricial pemphigoid (OCP) is one of the most common variants of mucous membrane pemphigoid (MMP) and is a vision threatening autoimmune disease if left untreated. We performed a retrospective, observational clinical study to learn about the efficacy of intravenous immunoglobulin (IVIg) monotherapy in ocular cicatricial pemphigoid (OCP).
Methods :
A chart review of all the patients seen at the Massachusetts Eye Research and Surgery Institution between 2005 and 2015 with the diagnosis of OCP were treated with IVIg monotherapy was completed. Stage at presentation and before starting IVIg was graded by using the Foster grading system. Therapy and clinical course were recorded. The dosage of IVIg (Gammunex) infusion is 2g/kg/month and it should be administered in 3 consecutive days.
Results :
In a total of 512 OCP patients (1024 eyes), 14 patients (28 eyes) treated with IVIg monotherapy were identified. Age at diagnose was from 44 to 83 year-old and 11/14 patients were between 60 to 75 years old with an average of 67 years. Seven were female and 7 were male. The follow up time after diagnosis ranged from 4 months to 120 months. Twenty-two eyes of 11 patients achieved remission with IVIg infusion alone, and 6 eyes (2 stage 2, 2 stage 3, 2 stage 4) from 3 patients failed to achieve remission with IVIg monotherapy. Nine remission eyes from 5 patients had cataract surgeries during IVIg monotherapy, 2 eyes (1 stage 2, I stage 3) from 2 patients were reactive after remission (2/9 =22.2% recurrence rate). Other 13 remission eyes without ocular surgeries were stable (0/13=0% recurrence rate).
Conclusions :
Infused IVIg monotherapy at dose of 2 g/kg/month shows high efficacy in stage 1 OCP. Ocular surgery can increase recurrence rate over non-operative cases in OCP (22.2% to 0%), even when they have achieved remission under IVIg monotherapy.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.