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.


METHODS
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.
Wang
Robert C
RC
Doheny Eye Institute and the Department of Ophthalmology, Keck School of Medicine of the University of Southern California, Los Angeles 90033, USA.
Zamir
Ehud
E
Dugel
Pravin U
PU
Sipperley
Jack O
JO
Thirkill
Charles E
CE
Shabatian
Babak
B
Rao
Narsing A
NA
D000368:Aged; D001323:Autoantibodies / Q000097:blood; D001766:Blindness / Q000209:etiology* / Q000473:pathology; D002825:Chorioretinitis / Q000150:complications* / Q000175:diagnosis; D003094:Collagen / Q000378:metabolism; D004279:DNA, Viral / Q000032:analysis; D018450:Disease Progression; D005355:Fibrosis; D005451:Fluorescein Angiography; D019084:Fluorescent Antibody Technique, Indirect; D006099:Granuloma / Q000150:complications* / Q000175:diagnosis; D018259:Herpesvirus 1, Human / Q000235:genetics; D006801:Humans; D008297:Male; D009879:Ophthalmia, Sympathetic / Q000175:diagnosis / Q000209:etiology*; D020419:Photoreceptor Cells, Vertebrate / Q000276:immunology; D010857:Pigment Epithelium of Eye / Q000276:immunology; D016133:Polymerase Chain Reaction; D011183:Postoperative Complications; D012160:Retina / Q000378:metabolism / Q000473:pathology*
D001323:Autoantibodies; D004279:DNA, Viral; D003094:Collagen