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Anne Ruebsam, Richard Stefaniak, Margitta Worm, Uwe Pleyer; Rituximab for treatment refractory ocular cicatricial pemphigoid (OCP). Invest. Ophthalmol. Vis. Sci. 2014;55(13):1494.
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OCP is a chronic, progressive cicatrizing inflammatory disease of presumed autoimmune etiology affecting mucous membranes as well as the skin. It often progresses as a potentially blinding disorder. Systemic immunomodulatory therapy in a step latter approach remains current treatment of choice. Rituximab, a chimeric recombinant monoclonal antibody targeting CD20+ B cells, has been suggested to be effective in the treatment of various autoimmune disorders. Therefore we analyzed our first experience with Rituximab in OCP patients that previously failed to respond despite aggressive conventional treatment.
We reviewed medical records of six OCP patients treated with Rituximab between 2008 and 2012. Rituximab was administered i.v. at an average dosage of 1000 mg on days 0 and 14 per cycle according to the rheumatoid arthritis protocol. Three patients received a second cycle. Concomitant immunosuppressants were given in three patients. Primary outcome measures were the treatment response as indicated by stabilization of Foster Staging (modified Foster Staging) at four and 12 weeks and the sustainability of the best-corrected visual acuity. Secondary outcome measure was the occurrence of drug toxicity based reactions.
The median follow-up period was 18.8 months. Prior to initiation of Rituximab treatment median visual acuity was 0.3 log MAR. Four weeks after the first infusion it improved in four out of five patients and remained stable in one patient (one patient was excluded because of dementia). Stabilization of Foster staging could be achieved in all patients. Four patients still progressed after two months, but remained stable either after a second cycle or after initiation of immunosuppressive treatment. Overall complete response could be achieved in two patients, partial response in another two patients (overall response rate 66%). Two patients were stable by increase of immunosuppression. Rituximab was well tolerated, but an anaphylactic reaction occurred in our youngest individual at the first application.
Our preliminary experience indicates that Rituximab is a promising therapeutic option for patients with otherwise treatment-resistant OCP. Treatment overall was well tolerated, but high costs and limited experience of long-term effects limit its use to selected patients. The rheumatoid arthritis protocol seems to be sufficient to reach a remission, but repeated cycles are required.
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