Abstract
Purpose:
To analyze the clinical course of patients with recalcitrant Ocular Cicatricial Pemphigoid (OCP) treated with Rituximab (RTX) and intravenous immunoglobulin (IV-Ig)
Methods:
A non comparative retrospective case series of 12 immunohistopathologically proven OCP patients who failed conventional immunosuppressive therapy. All patients received a combination of RTX and IV-Ig according to the protocol and a minimum follow up of 6 months. The main outcome measures were blindness (best corrected visual acuity BCVA 20/200 or worse), control of inflammation and Foster OCP staging. RTX + IV-Ig Treatment Protocols: 1) RTX: Dose 375mg/m2 once weekly for 8 consecutive weeks, then monthly for the subsequent 4 months, total of 12 infusions during a period of 6 months. 2) IV-Ig: Dose 2g/kg/cycle. Total dose was divided into 3 equal parts on 3 consecutive days. The patients were given one cycle of IV-Ig prior to the initiation of RTX and then monthly until the B cell count returned to normal. Thereafter, IV-Ig was continued at 6, 8, 10, 12, 14 and 16 weeks. The last cycle was given at 16 week interval.
Results:
Twelve OCP patients (24 eyes), 7 female and 5 male, with a median age of 58 years (range 40-70 years) were included. The duration of the disease varied from 2-168 months (median 24). Total follow-up period was between 6-108 months (median 18 months. All but 5 patients received the above RTX+IV-Ig protocol (median 12.5 infusions). Five patients with aggressive disease (2 were monocular, and 1 relapsed after being in remission for a year) received additional RTX infusions. Of these 1 eventually failed therapy. The duration of RTX infusions was 4-19 months (median 12months). None of the eyes became blind at the end of follow up. Eight patients (66.6%) were in remission after 3-12 months (median 6months). Of these 8 patients, 6 achieved remission off therapy while 2 (16.6%) had resolving inflammation with continuation of therapy. Two patients failed (16.6%). Before and after therapy, OCP staging remained stable throughout the study period.
Conclusions:
Combination of RTX and IV-Ig is effective in preventing blindness and arresting disease progression in patients with recalcitrant OCP.
Keywords: 432 autoimmune disease •
475 conjunctivitis •
555 immunomodulation/immunoregulation