Antibodies to Oral Mucosa in Patients with 'Ocular Behcet's Disease JOSEPH B. MICHELSON, MD, FRANCIS V. CHISARI, MD, TULAY KANSU, MD Abstract: A method is reported for the identification of cytoplasmic antibodies in patients with Behcet's disease and uveitis. The assay appears positive in at least 80% of patients in an American population with definite or probable Behcet's disease and 60% of patients from a Turkish population with definite Behcet's disease, with a false-positive rate of 6.5% among non-Behcet's ocular inflammatory disorders with vasculitis. When refined, this test may prove useful to the ophthalmologist in selecting out those patients with Behcet's disease from the larger group of patients with uveitis for whom no systemic etiology is identified. [Key words: antibodies, Behcet's disease, perivasculitis, retinal vasculitis.] Ophthalmology 92: 1277 -1281, 1985 Behcet's disease is an aftliction of unknown etiology with no diagnostic test and no known cure_ Its clinical picture is characterized by three primary components: iridocyclitis with hypopyon, aphthous ulceration in the mouth, and ulceration of the genitalia. Erythema no- dosum, arthropathy, thrombophlebitis and nervous sys- tem involvement often accompany these manifestations, but the ocular symptoms may be the most serious manifestations of the disease, I including a necrotizing vasculitis of the choroid and retina. I ,2 The burden of diagnostic recognition of Behcet's disease often lies with the ophthalmologist since he/she may be the first phy- sician to encounter the patient or put the patient's random systemic complaints into meaningful order. The histologic hallmark of Behcet's disease is an obliterative, necrotizing vasculitis affecting both arteries and veins, characterized microscopically by fibrinoid degeneration, endothelial cell swelling and proliferation From the Retina-Uveitis Service, Division of Ophthalmology and the Department of Basic and Clinical Research Scripps Clinic and Research Foundation, La Jolla, California. Presented at the Eighty-ninth Annual Meeting of the American Academy of Ophthalmology, Atlanta, Georgia, November 11-15, 1984. Publication number 3064-BCR from the Research Institute of Scripps Clinic. Supported in part by a grant from the Eye Bank, San Diego County Medical Society. Reprint requests to Joseph B. Michelson, MD, Division of Ophthalmology, Scripps Clinic and Research Foundation, 10666 North Torrey Pines Road, La Jolla, CA 92037. and a mononuclear cell infiltrate. 3 ,4 These findings are characteristic of several immunologically induced vas- culitides, especially those associated with circulating immune complexes. 5 Indeed, it has been reported that approximately 40% of patients with Behcet's disease have circulating immune complexes detectable by the Raji cell assay.5 Others have reported the presence of autoantibodies reactive with oral mucosal antigens in Behcet's disease, especially during relapse. 6 The antigenic specificity and diagnostic sensitivity of assays for these autoantibodies has not been determined. The importance of mucosal antigen stimulation in these patients is emphasized by the reported evidence of macrophage migration inhibitory factor production by mucosal an- tigen-stimulated T cells from these patients. 7 Addition- ally, peripheral blood lymphocytes from Behcet's patients have been reported to be cytotoxic for cultured oral epithelial cells in vitro. 8 The immunologic basis of Behcet's disease is strengthened by the favorable thera- peutic effect of corticosteroids 9 and isolated reports of improvement following transfer factor 9 ,10 or levamisole therapy. 10, II Clearly, ancillary diagnostic assistance is required in the identification of patients with Behcet's disease because of the absence of strictly pathognomonic clinical findings. Further, there is a spectrum of clinical presentation of the signs of the disease, underscoring the fact that this diagnosis is still a strictly clinical determination. Ac- cording to the criteria of the Behcet's disease Research Committee of Japan,2 the range of signs can be classified 1277