Abstract:Vogt-Koyanagi-Harada (VKH) disease is a systemic disorder causing bilateral panuveitis. Histopathological documentation along with molecular diagnostic evidence in VKH eye is a rarity. We present a 46-year-old woman with VKH with several ocular complications and subsequently enucleation of the right eye was done because of painful blind eye. Patient had clinical complications of VKH and some of the complications were observed in histopathology. Pathology of the case showed nongranulomatous uveitis, indicating … Show more
“…Several studies have indicated that VKH disease is associated with histopathological changes in the choroid 12,13 . Infiltration of plasma cells and lymphocytes and the presence of CD3-positive cells in the choroid www.nature.com/scientificreports/ have been observed in HE and immunohistochemistry sections from human patients.…”
Anti-Cytotoxic T-Lymphocyte Associated protein 4 agents, such as ipilimumab, are widely applied to various cancers. However, they cause immune-related adverse effects throughout the body, including the eye. This study examined whether ipilimumab induces retinal and choroidal abnormalities in rodents, and investigated potential underlying mechanisms. Female wild-type mice were injected with ipilimumab three times/week for 5 weeks. The mice underwent optical coherence tomography (OCT) on the first day of the 6th week. Retinal function and morphology were evaluated by light microscopy, immunohistochemistry and electroretinography (ERG). On OCT, the lines indicating the ellipsoid and interdigitation were obscure in treated mice, suggesting outer retina destruction. Haematoxylin–eosin staining revealed destruction, shortening, and outer segment vacuolization. Treated mice exhibited weaker, fragmented rhodamine peanut agglutinin staining in outer photoreceptor structures. The choroid of treated mice showed severe infiltration of CD45-positive cells. In addition, CD8-positive cells invaded into the outer retina. On ERG, rod, maximum responses of combined rods and cones, and cone response wave amplitudes were significantly reduced in treated mice. Ipilimumab may induce impairments in outer photoreceptor architecture accompanied with CD8- positive infiltration in the retina and CD45-positive cell infiltration in the choroid, which may contribute to retinal function deterioration.
“…Several studies have indicated that VKH disease is associated with histopathological changes in the choroid 12,13 . Infiltration of plasma cells and lymphocytes and the presence of CD3-positive cells in the choroid www.nature.com/scientificreports/ have been observed in HE and immunohistochemistry sections from human patients.…”
Anti-Cytotoxic T-Lymphocyte Associated protein 4 agents, such as ipilimumab, are widely applied to various cancers. However, they cause immune-related adverse effects throughout the body, including the eye. This study examined whether ipilimumab induces retinal and choroidal abnormalities in rodents, and investigated potential underlying mechanisms. Female wild-type mice were injected with ipilimumab three times/week for 5 weeks. The mice underwent optical coherence tomography (OCT) on the first day of the 6th week. Retinal function and morphology were evaluated by light microscopy, immunohistochemistry and electroretinography (ERG). On OCT, the lines indicating the ellipsoid and interdigitation were obscure in treated mice, suggesting outer retina destruction. Haematoxylin–eosin staining revealed destruction, shortening, and outer segment vacuolization. Treated mice exhibited weaker, fragmented rhodamine peanut agglutinin staining in outer photoreceptor structures. The choroid of treated mice showed severe infiltration of CD45-positive cells. In addition, CD8-positive cells invaded into the outer retina. On ERG, rod, maximum responses of combined rods and cones, and cone response wave amplitudes were significantly reduced in treated mice. Ipilimumab may induce impairments in outer photoreceptor architecture accompanied with CD8- positive infiltration in the retina and CD45-positive cell infiltration in the choroid, which may contribute to retinal function deterioration.
“…Our study showed cytotoxic T-cells in the eye ball specimen of Eales disease. T cell mediated immunologic reaction has been found in Behcet’s disease [ 4 ], Vogt Koyanagi Harada disease [ 5 ] and sympathetic ophthalmia [ 6 ]. Our study indicates in active stage of Eales disease, immunomodulatory therapy with T cell inhibitors may be effective.…”
Section: Discussionmentioning
confidence: 99%
“…4 Department of Uvea, Sri SankaradevaNethralaya, Guwahati, Assam, India. 5 Sankara Nethralaya Referral Laboratory, Tamil Nadu, Chennai, India.…”
Eales’ disease is a retinal vasculitis characterized by retinal inflammation, ischemia, and neovascularization. Exact pathogenesis of this disease is yet to be found out. We present a 29-year-old male, diagnosed with Eales’ disease in both eyes with persistent intraocular inflammation. Enucleation of the pthisical right eye was subjected for histopathological examination immunohistochemistry and molecular biologic study for mycobacterial tuberculosis DNA. Our study showed that Eales disease is probably a T cell mediated disease which is triggered by mycobacterial TB DNA. Further studies are needed to confirm our findings.
“…Hyperpigmented areas in atrophic patch are due to the proliferation of RPE, rarely associated with subretinal neovascularization [33]. Immunological markers like CD3 and CD20 with increased T cell involvement have been found in cases of VKH syndrome [34].…”
Vogt-Koyanagi-Harada (VKH) disease is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin, and hair. This disease is mainly a T1 lymphocyte-mediated aggression to melanocytes. The availability of new investigational methods has improved our knowledge of the pathogenesis, clinical approach, diagnosis, and management of VKH disease. The disease has an acute onset of bilateral blurred vision with hyperemia in the absence of prior ocular trauma or any previous intraocular surgery. The chronic phase comprises of ocular and integumentary system pigmentary changes. Ocular findings may be accompanied by meningismus, hearing impairment, and skin lesions in a variable proportion of patients. Prompt diagnosis with early, aggressive, and long-term treatment of high-dose corticosteroids ensures good visual outcomes. The aim of this chapter is to present the clinicopathology, classification, recent imaging, investigations, and management of VKH disease.
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