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S. Doan, C. Le Roux–Villet, C. Prost–Squarcioni, F. Caux, T. Hoang–Xuan; Rituximab in Severe Ocular Cicatricial Pemphigoid . Invest. Ophthalmol. Vis. Sci. 2006;47(13):594.
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Ocular cicatricial pemphigoid (CP) is a potentially blinding auto–immune bullous disease characterized by a chronic cicatrizing conjunctivitis. Involvement of the larynx can also be lethal. In severe cases, immunosuppressive systemic therapies like cyclophosphamide or steroids are needed. In case of failure, few alternate therapies are available. Rituximab (Mabthera®) is a monoclonal anti–CD20 antibody. Its efficacy has been shown in B cell lymphomas and more recently in severe forms of pemphigus, another auto–immune bullous disease. We report the efficacy of Rituximab in 4 cases of ocular CP.
Four patients (mean age, 73 years) with severe ocular and laryngeal CP were treated with rituximab. Rituximab was introduced because of a severe ocular involvement that did not respond to at least 4 bolus of intravenous cyclophosphamide in 2 cases, and because cyclophosphamide was contra–indicated in the 2 other cases. Four weekly intravenous perfusions of Rituximab (375 mg/m²) were administered. All other systemic immunosuppressive treatments were discontinued except for dapsone in 2 cases.
Clinical efficacy was detectable after 4 to 8 weeks of treatment in all cases. After a mean follow–up of 8 months, ocular fibrosis stopped progressing, and ocular and laryngeal inflammation was controlled in all cases. A second treatment with rituximab was needed in 2 cases. No complications were noted.
Rituximab seems to be effective in severe ocular cicatricial pemphigoid, but a longer follow–up is required.
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