Cases reported "Ophthalmia, Sympathetic"

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1/5. Progressive subretinal fibrosis and blindness associated with multifocal granulomatous chorioretinitis: A variant of sympathetic ophthalmia.

    OBJECTIVE: To report a case of bilateral progressive subretinal fibrosis and blindness with multifocal granulomatous chorioretinitis occurring after intraocular surgery. We propose that this is a variant of sympathetic ophthalmia. DESIGN: Clinicopathologic case report. methods: The left enucleated globe was examined by histopathologic methods. The patient's sera were subjected to immunohistochemical studies against retinal antigens, and collagen 2 types in areas of fibrosis were identified. polymerase chain reaction was used to test for herpes virus dna in microdissected, formalin-fixed, paraffin-embedded tissue. RESULTS: The enucleated globe demonstrated histopathologic features similar to an entity previously described as progressive subretinal fibrosis with multifocal granulomatous chorioretinitis. The patient's sera demonstrated antibodies directed against retinal photoreceptors and pigment epithelium. Polymerase chain reaction for herpes virus was negative. Immunohistochemical studies demonstrated types III, IV, V, and VI collagen in areas of fibrosis. CONCLUSIONS: The clinical history along with the histopathologic and immunohistochemical findings suggest that progressive subretinal fibrosis with multifocal granulomatous chorioretinitis may represent a variant of sympathetic ophthalmia and that retinal autoimmunity may play a role in its pathogenesis.
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2/5. Twenty cases of sympathetic ophthalmia.

    We reviewed the charts of 20 patients with sympathetic ophthalmia who were seen in the uveitis clinic at the eye and ear Infirmary within an 11-year period. Of these 20 patients 14 maintained 20/50 or better visual acuity in at least one eye. We found early enucleation to be associated with a better visual prognosis, possibly due to earlier diagnosis and faster, more aggressive therapy rather than a reduction in antigenic load. The clinical appearance of Dalen-Fuchs nodules appears to indicate a more severe stage of disease. chlorambucil was useful in patients with severe disease. To be effective and to lessen its side effects chlorambucil was given in daily dosages that were increased weekly over a short period to achieve bone marrow suppression. After a course of chlorambucil therapy intraocular inflammation could be controlled with topical steroids alone.
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3/5. immunohistochemistry and electron microscopy of choroidal infiltrates and Dalen-Fuchs nodules in sympathetic ophthalmia.

    An immunohistological study using monoclonal antibodies directed at specific membrane antigens of various inflammatory cells was carried out in order to evaluate the identity and topographic localization of the immuno-competent cells in an enucleated eye from a 6-year-old black patient with a three-month history of sympathetic ophthalmia. Correlative light and transmission electron microscopic examination of serial sections was also performed. The data demonstrated that the predominant cells within the choroidal infiltrate were t-lymphocytes (Leu 1 ). T-cell subset analysis disclosed that most of these cells harbored specific antigenic determinants of the helper phenotype (Leu 3a ). A smaller proportion of the T cells demonstrated the specific determinants of the suppressor subtype (Leu 2a ). The helper/suppressor ratio varied slightly and ranged in most areas of the choroid between 3:1 and 4:1. Additionally, approximately 15% of the infiltrating lymphocytes harbored the Leu 14 determinant specific for B cells. The latter were located in the outer choroid adjacent to the sclera. Very few natural killer (NK) cells (Leu 7 ) were identified throughout the choroid. The granulomatous foci in the choroid were composed mainly of epithelioid cells and histiocytes expressing the OKM1 and M221 antigenic determinants on their membranes and demonstrating a high cytoplasmic nonspecific esterase activity (ANAE ). Within the Dalen-Fuchs nodules, similar to the choroidal nodules, there was a predominance of histiocytes and epithelioid cells (OKM1 , M221 , ANAE ), a few T-helper cells (Leu 1 , Leu3a ) and some OKM1-, M221- cells whose origin could not be determined. These findings were corroborated by electron microscopic observations of serial sections. Careful light and electron microscopic studies disclosed breaks in Bruch's membrane underlying some of Dalen-Fuchs nodules. In our opinion, these observations may be interpreted as the demonstration that Dalen-Fuchs' nodules and the choroidal granulomatous foci could be formed by identical cells of similar function and origin.
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4/5. Early stage of human sympathetic ophthalmia. Histologic and immunopathologic findings.

    Sympathetic uveitis developed in a 64-year-old woman 11 weeks after posttraumatic vitrectomy accompanied by vitreous hemorrhage. After removal of the exciting eye three weeks later, the sympathetic inflammation in the second eye subsided. Histologic examination revealed typical signs of an early stage of sympathetic ophthalmia characterized by a focally pronounced infiltration of lymphoid and T cells exclusively in the choroid. These T cells consisted of Leu 2a suppressor-cytotoxic T cells and a few Leu 3a inducer T cells situated near HLA-DR cells of the uvea. The latter contained electron-lucent cytoplasm without pigment granules and may represent the accessory cells for the initiation of the T-cell reaction. The findings suggest that sympathetic ophthalmia represents a T-cell-mediated cytotoxic reaction (similar to allograft rejection and graft-v-host disease) toward antigens on or near the pigment epithelium or the uveal melanocytes.
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5/5. Human sympathetic ophthalmia. An analysis of the inflammatory infiltrate by hybridoma-monoclonal antibodies, immunochemistry, and correlative electron microscopy.

    A case of human sympathetic ophthalmia, enucleated after surgical trauma, was studied by means of hybridoma-derived monoclonal antibodies, histochemistry, and transmission electron microscopy. The choroidal infiltrate was composed predominantly of t-lymphocytes of the suppressor/cytotoxic subset (OKT8 ); only 5% of the cells were immunoglobulin-producing b-lymphocytes (kappa or lambda light chain positive), thereby explaining the well-known paucity of plasma cells in the infiltrate. The epithelioid cells and phagocytic histiocytes in the choroid were la and OKM1 , antigenic determinants specific for bone marrow-derived monocytes, and their cytoplasms exhibited histochemical reactivity for alpha-1-antichymotrypsin and lysozyme. Ultrastructurally, the choroidal epithelioid cells contained single melanin granules in the cytoplasm, but these were membrane-bound and frequently associated with lysosomal material, features militating against these cells being transformed choroidal melanocytes. By means of immunologic and ultrastructural analysis, the Dalen-Fuchs nodules were found to be composed of a mixture of histiocytes (la and OKM1 ) and depigmented retinal pigment epithelial cells (la- and OKM1-); the latter cells focally formed desmosomes and displayed inclusions of lipofuscin. Scattered within the Dalen-Fuchs nodules were small numbers of t-lymphocytes of the suppressor/cytotoxic subset. We have concluded that the uveitis and retinal pigment epithelial changes are mediated by a T-cell, delayed hypersensitivity pathogenetic mechanism (cell-mediated immunity), possibly directed at surface membrane antigens that may be shared by photoreceptors, retinal pigment epithelial cells, and choroidal melanocytes.
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