Home Print this page Email this page Users Online: 594
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
BRIEF COMMUNICATIONS
Year : 2022  |  Volume : 10  |  Issue : 1  |  Page : 37-40

Sympathetic ophthalmia in a phthisical eye with B-cell proliferation


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 Submission02-Oct-2020
Date of Decision06-Apr-2021
Date of Acceptance26-Apr-2021
Date of Web Publication3-Feb-2022

Correspondence Address:
Dipankar Das
Department of Ocular Pathology, Uveitis and Neuro-Ophthalmology Services, Sri Sankaradeva Nethralaya, Guwahati, Assam
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcor.jcor_216_20

Rights and Permissions
  Abstract 


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 2022 May 24];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 Top


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

Click here to view


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

Click here to view
Figure 3: Light microscopy showed eosinophilic exudation with cholesterol clefts (marked with arrows) in hematoxylin and eosin stained slide, ×40

Click here to view
Figure 4: Immunostaining revealed CD20 (B cell marker, CELL MARQUE, USA): +++, ×20

Click here to view
Figure 5: Immunostaining revealed CD3 (T cell Marker, CELL MARQUE, USA): +, ×10

Click here to view



  Discussion Top


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.



 
  References Top

1.
Rao NA, Robin J, Hartmann D, Sweeney JA, Marak GE Jr. The role of the penetrating wound in the development of sympathetic ophthalmia experimental observations. Arch Ophthalmol 1983;101:102-4.  Back to cited text no. 1
    
2.
Chu XK, Chan CC. Sympathetic ophthalmia: To the twenty-first century and beyond. J Ophthalmic Inflamm Infect 2013;3:49.  Back to cited text no. 2
    
3.
Biswas J, Fogla R. Sympathetic ophthalmia following cyclocryotherapy - Report of a case with histopathological correlation. Ophthalmic Surg Lasers 1996;27:1035-308.  Back to cited text no. 3
    
4.
Bawankar P, Das D, Kuri GC, Medhi J, Barman M, Soibam R, et al. Cytophotocoagulation-induced sympathetic ophthalmia in a Coat's disease patient supported by histopathology and immunohistochemistry. Indian J Ophthalmol 2017;65:744-6.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Jakobiec FA, Marboe CC, Knowles DM 2nd, Iwamoto T, Harrison W, Chang S, et al. Human sympathetic ophthalmia. An analysis of the inflammatory infiltrate by hybridoma-monoclonal antibodies, immunochemistry, and correlative electron microscopy. Ophthalmology 1983;90:76-95.  Back to cited text no. 5
    
6.
Rao NA. Mechanisms of inflammatory response in sympathetic ophthalmia and VKH syndrome. Eye (Lond) 1997;11 (Pt 2):213-6.  Back to cited text no. 6
    
7.
Bawankar P, Das D, Barman M, Soibam R. Sympathetic Ophthalmia associated with uncomplicated retinal detachment surgery in young male: An uncommon entity. J Clin Ophthalmol Res 2018;6:24-6.  Back to cited text no. 7
  [Full text]  
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.  Back to cited text no. 8
    
9.
Gupta V, Gupta A, Dogra MR. Posterior sympathetic ophthalmia: A single centre long-term study of 40 patients from North India. Eye (Lond) 2008;22:1459-64.  Back to cited text no. 9
    
10.
Sen HN, Nussenblatt RB. Sympathetic ophthalmia: What have we learned? Am J Ophthalmol 2009;148:632-3.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Case Report
Discussion
References
Article Figures

 Article Access Statistics
    Viewed605    
    Printed6    
    Emailed0    
    PDF Downloaded38    
    Comments [Add]    

Recommend this journal