Rituximab and intravenous immunoglobulin in the treatment of cicatrizing conjunctivitis associated with pemphigoid

Purpose

To characterize the use of rituximab (RTX) and intravenous immunoglobulin (IVIG) in the treatment of cicatrizing conjunctivitis associated with pemphigoid.

Design

Retrospective consecutive case series of patients with ocular cicatricial pemphigoid (OCP) and mucous membrane pemphigoid with ocular involvement (ocMMP) treated with RTX/IVIG.

Methods

A retrospective chart review was performed on patients at the University of Texas Southwestern Medical Center between 2012-2024 with ocMMP or OCP undergoing treatment with RTX/IVIG. Data collected included patient demographics, ocular history, response to RTX/IVIG, and adverse events. Primary outcomes were ocular response to the treatment. The secondary outcome was the change or decrease in concomitant steroid dependence.

Results

A total of 31 patients (20, 64.5% female) met inclusion criteria. There were 20 (64.5%) patients with ocMMP, and 11 (35.5%) patients with OCP. The mean age at initiation of RTX was 64 years. In our cohort, 2 (6.5%) patients had no response to treatment, 10 (32.3%) achieved a partial response, 6 (19.4%) achieved clinical remission with steroids, 11 (35.5%) achieved steroid-free remission, and 2 (6.5%) achieved durable remission. The mean time to remission was 9.0 months. Mean follow up time was 54 months and the mean time to relapse was 32 months. There were five adverse events in our cohort: 1 case of neutropenia, 1 cerebrovascular accident, and 3 malignancies.

Conclusion

High dose RTX/IVIG is an effective treatment for cicatrizing conjunctivitis associated with pemphigoid, particularly in patients with severe recalcitrant disease. Judicious use due to risk of serious adverse events may limit widespread use.