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| Year : 2022 | Volume
: 10
| Issue : 1 | Page : 37-40 |
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Sympathetic ophthalmia in a phthisical eye with B-cell proliferation
Dipankar Das1, Saurabh Desmukh2, Bhavya Gokani2, K Vanitha3, Apurba Deka1, Jyotirmay Biswas3
1 Department of Ocular Pathology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
2 Department of Ophthalmology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
3 Department of Larsen and Toubro Ocular Pathology, Sankara Nethralaya, Chennai, Tamil Nadu, India
| Date of Submission | 02-Oct-2020 |
| Date of Decision | 06-Apr-2021 |
| Date of Acceptance | 26-Apr-2021 |
| Date of Web Publication | 3-Feb-2022 |
Correspondence Address:
Dipankar Das
Department of Ocular Pathology, Uveitis and Neuro-Ophthalmology Services, Sri Sankaradeva Nethralaya, Guwahati, Assam
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jcor.jcor_216_20
Sympathetic ophthalmia is a dreaded ocular condition resulting in bilateral panuveitis following penetrating injury in one eye or associated with surgeries and laser procedures. We describe a case of a 13-year-old girl who presented with blurring of vision in the right eye for the past 2 years following penetrating trauma in the left eye 9 years back. Histopathology of left enucleated phthisical eye showed diffuse stromal choroiditis with B-cell proliferation on immunohistochemistry, thus confirming chronicity of the disease.
Keywords: Corticosteroids, penetrating, phthisical, sympathetic ophthalmia
How to cite this article:
Das D, Desmukh S, Gokani B, Vanitha K, Deka A, Biswas J. Sympathetic ophthalmia in a phthisical eye with B-cell proliferation. J Clin Ophthalmol Res 2022;10:37-40 |
How to cite this URL:
Das D, Desmukh S, Gokani B, Vanitha K, Deka A, Biswas J. Sympathetic ophthalmia in a phthisical eye with B-cell proliferation. J Clin Ophthalmol Res [serial online] 2022 [cited 2023 Mar 25];10:37-40. Available from: https://www.jcor.in/text.asp?2022/10/1/37/337189 |
Sympathetic ophthalmia (SO) is a bilateral granulomatous diffuse uveitis that occurs following ocular trauma and surgical procedure to one eye, which incites inflammation both in the traumatic eye and the fellow sympathizing eye.[1],[2],[3],[4],[5],[6] In 80% of the cases, SO occurs within 3 months of the initial insult, and 90% of the cases occur within 1 year, but it can occur anytime between 1 week and 66 years after injury.[3],[4],[5],[6] The first line of treatment of this disease is high dose of corticosteroids (1–2 mg/kg/day) with slow tapering.[3],[7] Corticosteroid sparing immunosuppressive agents are typically required as most patients need long-term treatment.[3],[4],[5],[6],[7],[8] We present a case of a young girl with a history of penetrating injury in the left eye (L/E) 9 years back followed corneal tear repair and subsequently developed retinal detachment. Histopathological examination and molecular pathologic investigations were carried out subsequently.
| Case Report | |  |
A 13-year-old girl presented to a tertiary care center with blurring of vision in only seeing right eye (R/E) for the past 2 years. She suffered an open globe injury in the L/E 9 years back followed by corneal repair. L/E later developed retinal detachment and became phthisical and had no light perception. R/E had best-corrected visual acuity of 20/25, N6. Anterior segment showed large pigmented mutton-fat keratic precipitates, posterior synechia [Figure 1] with 1+ posterior vitreous cells and 2+ vitreous haze. Retina and disc were within normal limits. Swept-source optical coherence tomography showed subfoveal choroidal thickness of 535 microns in the L/E. Fundus fluorescein angiography and indocyanine green angiography confirmed the absence of any posterior segment involvement in the R/E. Oral steroids were started at 1 mg per Kg body weight along with immunomodulatory therapy (tablet azathioprine 50 mg thrice daily). Vision was maintained at 20/25 in the R/E. Choroidal thickness was remained unchanged in that eye. Enucleation of the L/E was done as the patient was interested in cosmesis for her phthisical eye. The patient was continued on immunomodulators, and a customized prosthesis for L/E was made. | Figure 1: Slit-lamp picture of right eye showing granulomatous keratic precipitates with posterior synechia. Please note: Vitreous cells was + and vitreous haze 2 + in that eye
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Microscopic findings of enucleated L/E showed normal corneal epithelium, Bowman's layer, stromal corneal scarring with normal Descemet's membrane, and loss of endothelial cells. Anterior chamber was deep with portion of iris seen in the periphery and touching the posterior aspect of the cornea and limbus. Iris neovascularization was evident with loss of internal architecture. Portion of the choroid near the ciliary body showed that inflammatory cell infiltrates mostly with mononuclear cells including lymphocytes and plasma cells [Figure 2]. Choriocapillaris was spared in one of the areas of stromal choroidal infiltration. Few epitheloid cells were also noted. Choroid showed increased thickness with exudative retinal detachment. Cholesterol clefts were seen within the eosinophilic exudation [Figure 3]. Retina showed hyperplastic change. Sclera was thickened posteriorly. Diagnosis of SO with phthisical change was confirmed pathologically. Interestingly, immunohistochemistry for CD20: +++ [Figure 4] and CD3: + [Figure 5] (CELL MARQUE, USA) was documented. Slides were compared with positive and negative controls. | Figure 2: Light microscopy showed that inflammatory cells infiltrate of choroid by lymphocytes, plasma, and few epitheloid cells in hematoxylin and eosin stained slide, ×10. Arrows showed the choriocapillaris, spared by inflammation
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 | Figure 3: Light microscopy showed eosinophilic exudation with cholesterol clefts (marked with arrows) in hematoxylin and eosin stained slide, ×40
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 | Figure 4: Immunostaining revealed CD20 (B cell marker, CELL MARQUE, USA): +++, ×20
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 | Figure 5: Immunostaining revealed CD3 (T cell Marker, CELL MARQUE, USA): +, ×10
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| Discussion | | |
T-cells play a major role in autoimmunity and immunological cell mechanism of SO.[1],[2],[3],[4],[5],[6],[7],[8] T-cell-mediated immune response is not only caused by humoral route but also through lymphocytes and lymphokines that control the recruitment of other cells in the immune process.[3],[4],[5],[6],[7],[8] The mechanism of autoimmune disease can vary depending the structures of eye involvement, chronicity of the disease, and merging toward the end-stage disease.[4],[5],[8],[9],[10] The prototype of inflammatory cascade of panuveitis is SO where T-cells and macrophages are the targeted cells.[8] This is usually seen in early stage of SO when bilateral granulomatous panuveitis sets-in but in some occasions when the disease is prolonged and chronic, in spite of having maximum immunosuppressive medications, immunological behavior may be altered and B-cell proliferations may be evident.[8] In the absence of infectious causes, auto-inflammatory (autoimmune) responses work against our own tissues including uveal tissue sites.[1],[8] Irrespective of which triggering event breaks the regulation, antigen-specific CD4+ T cells can initiate the disease only after peripheral clonal expression and probably on further presentation of antigen either in regional drainage lymph nodes or at the targeted site.[5],[6],[8],[9] Initiation of auto-inflammation may be subclinical.[10] Once if it is overt symptomatically, it is likely that majority of responses we see clinically in eye are in chronic stage of involvement.[8],[10] There may be numerous additional local cellular and molecular immune regulatory mechanisms acting to reduce the bystander collateral damage on the reduced side of inflammation but at constant antigen-specific response.[8],[10] Another simpler thought process is that when immunosuppressive agents acting on T-cells are used for prolonged duration, there clonal expression for those T-cells may be reduced whereas the B-cells affection may be unaltered. Our patient was diagnosed to have SO in the R/E following trauma and surgeries in the L/E. She received best possible treatment and subsequently L/E turned blind and phthisical. Histopathology of enucleated eyeball showed stromal choroidal involvement by lymphocytes, plasma cells, and few epitheloid cells. There was sparing of choriocapillaris which was evident in that late stage of SO also. Our case showed B-cell proliferations (+++) more than T-cell proliferations (+), and these alterations in immune-markers suggest that the case was long standing and chronic. The time duration of penetrating injury to enucleation was 9 years. Cholesterol clefts in the exudation of exudative retinal detachment with phthisical change were evident for chronic stage of the disease.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient's parent consent forms. In the form, the patient's father had given his consent for his daughter images and other clinical information to be reported in the journal. The patient's father understands that their daughter name and initial will not be published and due efforts will be made to conceal their identity.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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| 8. | Nussenblatt RB, Palestine AB, Whitcup SM, Craven L. Sympathetic Ophthalmia. Uveitis: Fundamentals and Clinical Practice. St Louis, MO: CV Mosby; 1995. p. 299-311. |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
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