Autoimmune phenomena in ocular cicatricial pemphigoid

Abstract

Twelve patients with various stages of ocular cicatricial pemphigoid were studied. Tear volumes measured by Schirmer tests and visual acuities were generally reduced in proportion to disease severity. Mannitol-positive staphylococcus was recovered from 67% (16/24) of the eyelids cultured. Immunoglobulins were bound to the basement membrane of clinically involved conjunctivae in 67% of the patients (8/12). Eighty percent (8/10) of the clinically normal conjunctivae had no immunoglobulins bound to the basement membrane or epithelium, further reinforcing the importance of tissue-fixed immunoglobulins in the pathogenesis of this condition. Four of eight patients with immunoglobulins bound to basement membrane also had C3 complement bound to basement membrane. Acute disease may be associated with the presence of complement in the conjunctiva. In addition, three of the eight patients with basement membrane staining, and three without, showed marked epithelial intercellular and intracellular staining for immunoglobulins. Indirect immunofluorescence of patients' sera showed no circulating antibodies to basement membrane, but three patients had circulating antibodies directed to the conjunctival epithelium in an intercellular or intracellular location. No significant association of ocular pemphigoid with other circulating autoantibodies. HLA aantigens, or abnormal serum immunoglobulin levels was realized.