Allogenic fetal retinal pigment epithelial cell transplant in a patient with geographic atrophy.

PURPOSE: To test the hypothesis that healthy fetal retinal pigment epithelium (RPE) can rescue the remaining viable RPE and choriocapillaries and thereby the photoreceptors in non-neovascular age-related macular degeneration (ARMD) (geographic atrophy [GA]). METHODS: A 65-year-old legally blind woman with non-neovascular ARMD underwent fetal RPE transplantation. Best-corrected visual acuity testing, detailed fundus examination, fundus photography, fluorescein angiography, scanning laser ophthalmoscope macular perimetry, and humoral and cellular immune response testing were performed. A suspension of RPE was infused into the subretinal space through a retinotomy along the superotemporal arcade at the edge of the area of GA. The patient did not take systemic immunosuppressants. RESULTS: The patient's vision remained unchanged for 5 months after the surgery. Fluorescein angiography after transplantation showed leakage and staining at the level of the outer retina. There was progressive subretinal fibrosis in the area of the transplant. Immune response studies showed a weakly positive mixed lymphocyte response against phosducin and rhodopsin. CONCLUSION: Although it is surgically feasible to transplant fetal RPE to the subretinal space of patients with GA, such an allogenic RPE transplant without immunosuppression leads to leakage on fluorescein angiography and eventual fibrosis. A very weak immune response against proteins associated with photoreceptors is also of concern.

Intraocular inflammatory reactions without focal necrotizing retinochoroiditis in patients with acquired systemic toxoplasmosis.

PURPOSE: To describe the occurrence of intraocular inflammatory reactions as the sole ophthalmic manifestation of acquired systemic toxoplasmosis. METHODS: Review of medical records for 10 patients with uveitis and evidence of recent Toxoplasma gondii infection. RESULTS: Patient ages ranged from 3 to 51 years. Ocular symptoms were present in each of eight adult patients. Inflammation was unilateral in nine patients; it manifested as vitreous humor cells and haze (10 patients), anterior chamber cells (seven patients), and retinal vasculitis (seven patients). No patient had necrotizing retinochoroiditis upon initial examination. Inflammation resolved in each of nine patients who had follow-up examinations. Foci of retinitis or inactive retinochoroidal scars were seen in four of these nine patients during follow-up examinations, at intervals of 2.0 weeks to 2.5 years after initial examination. CONCLUSIONS: Retinal vasculitis and associated inflammatory reactions may be the only ophthalmic disorder during the early stages of a newly acquired T. gondii infection. Later development of retinitis or scars consistent with toxoplasmic retinochoroiditis in the same eyes suggests that the initial, isolated inflammation may be caused by the presence of parasites in retinal tissue. These cases may have implications for understanding the original source of retinal infection in patients who have recurrent toxoplasmic retinochoroiditis and for treatment of newly acquired T gondii infection.

Ketorolac treatment of pseudophakic cystoid macular edema identified more than 24 months after cataract extraction.

OBJECTIVE: To evaluate whether ketorolac ophthalmic drops prescribed four times a day can be associated with improved visual acuity and prompt resolution of edema for patients with pseudophakic cystoid macular edema identified more than 24 months after cataract surgery. DESIGN: Prospective, nonrandomized, comparative (subject self-controlled) trial. PARTICIPANTS: The records of nine patients who had pseudophakic cystoid macular edema more than 24 months after cataract surgery at the time treatment commenced were identified at the Wilmer Retinal Vascular Center from September 1, 1996, through March 1, 1997. MAIN OUTCOME MEASURES: Best-corrected visual acuities measured on a retroilluminated Bailey-Lovie chart approximately every 3 months, contact lens biomicroscopy, and fluorescein angiography following ketorolac. INTERVENTION: Commercially available ketorolac ophthalmic drops 0.5% were prescribed for the affected eye four times a day for at least 3 months and continued until edema resolved. RESULTS: Ten eyes of nine patients were identified more than 24 months after cataract extraction (median, 59 months). Seven eyes (70%) improved (mean, +3.2 lines; range, +1 to +13 lines), including six by 2 or more lines 3 months after treatment initiation. Two eyes (20%) were unchanged, and one eye (10%) was 1 line worse. All seven eyes that improved 1 line or more had some or complete angiographic resolution of fluorescein dye leakage. In these seven eyes, ketorolac was discontinued when dye leakage completely resolved or failed to continue to improve on periodic 3-month follow-up examinations. In all seven eyes, recurrence of edema was noted within 3 months after ketorolac was stopped. CONCLUSIONS: Chronic pseudophakic cystoid macular edema identified more than 24 months after cataract surgery can improve with topical ketorolac but probably requires persistent use.

pANCA antibodies in patients with anterior uveitis: identification of a marker antibody usually associated with ulcerative colitis.

A pANCA autoantibody (antineutrophil cytoplasmic antibody, perinuclear pattern) has been described in uveitis patients, but its correlation with systemic illnesses and the specific type of pANCA has not been defined. The goals of this study were to determine the (1) frequency of pANCA autoantibodies in uveitis, (2) systemic associations in the pANCA + uveitis patients, and (3) type of pANCA antigen recognized by the uveitis-associated autoantibody. Serum was obtained from 59 patients with anterior uveitis or panuveitis and from nonuveitis controls. A detailed medical and family history was obtained from each subject at the time of phlebotomy. Sera were screened by neutrophil ELISA to determine the frequency of ANCA positivity. Immunofluorescence assays were then used to differentiate cANCA from pANCA. The specificity of the pANCA + antibodies was further characterized by DNase 1 sensitivity and granule antigen ELISAs. ANCA antibodies were detected in 29% of all patients with panuveitis or anterior uveitis. In 41% of these ANCA + patients, serum antibody detected a perinuclear antigen that was sensitive in all cases to DNase 1. The majority of pANCA + uveitis patients were either HLA-B27 positive or had systemic evidence of immune-mediated diseases. Two pANCA + patients had no medical or family history of other immune-mediated diseases. This study identifies a subset of uveitis patients distinguished by expression of a specific pANCA marker antibody. The characteristics of this antibody are similar to the pANCA antibody present in most patients with ulcerative colitis. Expression of the pANCA autoantibody in uveitis patients is a susceptibility marker for other immune-mediated diseases.

Delusions of ocular parasitosis.

PURPOSE: To describe four cases of delusions of parasitosis in which self-inflicted ocular trauma occurred. Delusions of parasitosis is a somatic delusional disorder in which patients have the irrational belief that their bodies are infested by parasites or other infectious organisms. Self-inflicted trauma can result from attempts to eliminate the supposed infestation. METHODS: We reviewed the case histories of four patients (one male, three females, 35 to 45 years of age) who presented with complaints of ocular infestation but had no evidence of infectious ocular disease. The characteristics of these cases were compared with the features of delusions of parasitosis. RESULTS: All patients maintained their beliefs regarding infestation, despite extensive clinical and laboratory investigations that found no evidence of infectious diseases. Self-inflicted eye injury, associated with attempts to eliminate the infestation, occurred in each case. CONCLUSIONS: The cases presented in this report are consistent with a diagnosis of delusions of parasitosis. The eye can be a principal focus of attention in this disorder, which may lead to vision loss caused by self-inflicted injury.

Recurrent ocular toxoplasmosis in patients treated with systemic corticosteroids.

PURPOSE: The authors discuss a possible relationship between systematic corticosteroid use and reactivation of ocular toxoplasmosis. METHODS: Patients were identified who developed foci of recurrent toxoplasmic retinochoroiditis while being treated with systemic corticosteroids. Case histories were reviewed retrospectively. RESULTS: During a 10-year interval, three patients were identified at the University of California, Los Angeles, who had been receiving systemic corticosteroid therapy (dose range, 0.27-1.23 mg/kg/day) when they developed recurrent toxoplasmic retinochoroiditis. Disease occurred at intervals of 20 days to approximately 1 year after start of corticosteroid therapy. Lesions were typical in appearance, course, and manner in which they responded to antimicrobial therapy. CONCLUSION: Recurrent toxoplasmosis in patients receiving corticosteroid therapy probably is uncommon. These cases do not confirm a causal relationship between corticosteroid use and initiation of disease recurrence.

Association between Vogt-Koyanagi-Harada syndrome and HLA-DR1 and -DR4 in Hispanic patients living in southern California.

BACKGROUND: Vogt-Koyanagi-Harada (VKH) syndrome is associated with human leukocyte antigen (HLA)-B54, -DR4, -DR beta 1*0405, -DQ4, and -DR53 in Japanese patients. Disease-associated HLA specificities may differ among races. This study examined HLA associations with VKH syndrome in Hispanic patients living in southern California, a racial subgroup at increased risk for the disease. METHODS: Human leukocyte antigen specificities were determined on 25 Hispanic patients with VKH syndrome and compared with HLA specificities of 217 healthy Hispanic control subjects. Inclusion criteria for study patients were nontraumatic panuveitis with exudative retinal detachments, with or without extraocular manifestations. Tests were performed using standard cytotoxic assays. RESULTS: HLA-DR4 was present in 14 (56%) patients with VKH syndrome and in 62(29%) control subjects (relative risk = 1.96). HLA-DR1 was present in 9 (36%) patients with VKH syndrome and in 19 (9%) control subjects (relative risk = 4.11). HLA-DR1 and DR4 share a common epitope within the DR beta 1 gene. HLA-DR1 and/or DR4 were present in 21 (84%) patients with VKH syndrome and in 76 (35%) control subjects (relative risk = 2.40). CONCLUSIONS: HLA-DR1 and -DR4 were found in a significantly disproportionate number of Hispanic patients with VKH syndrome living in southern California. HLA-DR4, although not HLA-DR1, has been previously associated with VKH syndrome in other groups. These associations suggest a common immunogenic predisposition to VKH among different racial groups, and suggest that a common epitope shared by DR1 and DR4 may be involved in the pathogenesis of the disease.