Abstract
Purpose:
To report the efficacy of rituximab in patients with severe ocular cicatricial pemphigoid (OCP).
Methods:
In this non controlled retrospective study, 18 patients with severe OCP (8 males, 10 females ; median age, 73 years ; 31 eyes) were treated with rituximab, because of toxicity or resistance to cyclophosphamide (in 10 and 4 cases, respectively), or of rapid evolutivity (4 cases). The treatment consisted of 4 weekly intravenous administrations (375 mg/m2) or 2 monthly intravenous administrations (1 gram). This treatment was repeated in case of persistent conjunctival inflammation. Complete remission was defined as the absence of conjunctival inflammation.
Results:
Complete remission was obtained in 16 patients (89%) within a median period of 2.5 months (range, 1 to 6 months) after 1 or 2 treatments (in 12 and 4 patients, respectively). The disease recurred in 5 of the 16 patients after 6 to 27 months. Complete remission was obtained after an additional rituximab treatment. Median follow up without any recurrence was 21 months (range, 12 to 54 months). No serious adverse effect was noted.
Conclusions:
Rituximab is a potent treatment of severe OCP. It is particularly interesting in case of resistance or contraindication to cyclophosphamide, or in rapidly evolutive forms.
Keywords: 432 autoimmune disease •
474 conjunctiva •
555 immunomodulation/immunoregulation