OBJECTIVE To assess the pathogenesis, symptomatology, and severity of rheumatoid arthritis (RA) in patient's with concomitant ocular cicatricial pemphigoid (OCP). METHODS We retrospectively analyzed the charts of eight patients seen at a single institution between the years 1972 and 1997 with concomitant RA and OCP. Patients with OCP secondary to medical therapy, radiation, or chemical burns, or Stevens-Johnson syndrome were excluded. RESULTS The female-to-male ratio was 7:1. All patients had positive serum rheumatoid factors and immunohistochemical confirmation of OCP. The mean number of years of RA prior to OCP diagnosis was 19. The number of patients with stage II OCP was three of eight (37.5%). The number of patients with stage III OCP was five of eight (62.5%). All patients had radiologic evidence of degenerative joint disease and synovial thickening. All eight patients had keratoconjunctivitis sicca, five of eight patients (62.5%) had Sjögren's syndrome, and five of eight patients (62.5%) developed rheumatoid cornea necrosis leading to corneal perforation. CONCLUSIONS The implication exists that RA and OCP may be linked via an immunologically mediated mechanism and that patients with severe extraarticular symptoms associated with RA may be more likely to develop OCP. Prompt recognition of overlying symptoms may facilitate proper therapy for control of both diseases.