Remission and recurrence after withdrawal of therapy for ocular cicatricial pemphigoid

Ocular cicatricial pemphigoid (OCP) is a chronic, progressive, autoimmune disease that scars mucus membranes and may lead to blindness. The authors studied the long-term effects of OCP in 104 consecutive patients (average follow-up, 4 years) to determine whether complete remission could be achieved after a course of treatment with immunosuppressive drugs. Prolonged periods of remission while not undergoing therapy were maintained in approximately one third of patients with OCP. Follow-up must be continued for life, as relapse occurred in 22% of those who were in remission and not undergoing therapy. Those who relapsed regained disease control readily upon reinstitution of therapy and did not deteriorate to more advanced cicatrization. Sex, age, initial degree of inflammation, and the incidence of extraocular involvement did not have a prognostic significance on outcome. Mechanisms that underlie the differing responses to therapy are not yet known.