ABSTRACT
OBJECTIVE: The efficiency of B-cell depletion therapy for severe ocular cicatricial pemphigoid (OCP) highlights the key role of B lymphocytes in the immunopathogenesis of OCP. B-cell activating factor (BAFF) is a potent B-cell growth factor and costimulator of immunoglobulin production. Elevated serum BAFF is associated with systemic autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis and bullous pemphigoid. We hypothesize that serum BAFF levels are also increased in patients with OCP. METHODS: Sera were collected from 30 patients with new-onset active OCP, 9 with disease in remission, 10 with OCP relapse, and 15 healthy control individuals. An enzyme-linked immunosorbent assay was performed to measure the concentration of serum BAFF. RESULTS: BAFF was significantly higher in patients with new-onset active OCP (700.8 ± 181.8 pg/mL) than in healthy control individuals (564.1 ± 133.2 pg/mL; pâ¯=â¯0.014). No significant difference was found between patients with OCP in remission (585.4 ± 216.2 pg/mL) and healthy control individuals. Patients with disease relapse treated with rituximab had an extremely high concentration of BAFF (1721.9 ± 790.8 pg/mL). Longitudinal analysis of serum BAFF from 6 patients showed that BAFF decreased as the disease went from new onset (895.0 ± 240.8 pg/mL) to remission (625.4 ± 199.8 pg/mL; pâ¯=â¯0.003). CONCLUSIONS: BAFF is involved in the active inflammation of OCP. Targeting BAFF with an antagonist may be therapeutically beneficial for patients with refractory OCP, especially those resistant to rituximab.
ABSTRACT
Purpose: To assess the long-term efficacy and safety of IVIg monotherapy in patients with recalcitrant ocular cicatricial pemphigoid (OCP). Methods: A chart review of all OCP patients seen at the Massachusetts Eye Research and Surgery Institution (MERSI) between 2005 and 2015 was completed. Stage was graded by using the Foster grading system. IVIg infusion was 2g/kg/cycle administered in 3 consecutive days monthly. Results: Of 512 OCP patients, 17 patients (34 eyes) treated with IVIg monotherapy were identified. Seven were female and ten were male. The average age at diagnosis was 60.7-year-old. The follow up time ranged from 12 to 140 months. Twenty-six eyes (76.5%) achieved remission. Nine remission eyes received cataract surgeries, and 2 of them had relapse (22.2%). The other 17 eyes did not undergo ocular surgery and remained in remission. IVIg monotherapy showed high efficacy in stage 1 OCP (7/7, 100%). Ocular surgery can be associated with OCP relapse (Table 2). Conclusions: IVIg monotherapy is an effective and safe therapy in patients with recalcitrant OCP. Ocular surgery can be associated with OCP relapse.