Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 30  |  Issue : 3  |  Page : 128-130

Unusual Exaggerated Inflammatory Response in Fuchs Uveitis: A Case Report


Department of Ophthalmology, All India Institute of Medical Sciences, Bhopal, Madhya Pradesh, India

Date of Submission02-May-2021
Date of Decision11-Jan-2022
Date of Acceptance18-Jan-2022
Date of Web Publication07-Nov-2022

Correspondence Address:
Dr. Samendra Karkhur
Department of Ophthalmology, All India Institute of Medical Sciences, Bhopal 462020, Madhya Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njo.njo_3_21

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  Abstract 


Objective: To report a case of Fuchs uveitis with an unusual exaggerated inflammatory response in the form of multifocal fibrin deposition. Case Report: Fuchs uveitis syndrome (FUS) has a characteristic clinical appearance but is frequently overlooked when heterochromia is subtle or absent. Specific atrophic changes in the iris, keratic precipitates, minimal cells and flare, the lack of posterior synechia, and the frequent presence of cataract and secondary glaucoma represent the clinical spectrum observed during the usual natural course of FUS. The occurrence of multifocal fibrin deposition on the entire iris surface in a young Indian male patient who presented with the clinical picture of Fuchs uveitis is reported. The finding of multifocal fibrin was unusual and thought to be an exaggerated inflammatory response in FUS. The patient was managed with topical corticosteroid therapy which resulted in complete resolution of fibrin. Regular follow-up was advised in view of the chronic nature of the disease and predisposition to complicated cataract and secondary glaucoma.

Keywords: Anterior uveitis, complicated cataract, Fuchs uveitis, iris atrophy, secondary glaucoma


How to cite this article:
Karkhur S, Soni D, Patel S, Sharma R, Nyodu R, Sharma B. Unusual Exaggerated Inflammatory Response in Fuchs Uveitis: A Case Report. Niger J Ophthalmol 2022;30:128-30

How to cite this URL:
Karkhur S, Soni D, Patel S, Sharma R, Nyodu R, Sharma B. Unusual Exaggerated Inflammatory Response in Fuchs Uveitis: A Case Report. Niger J Ophthalmol [serial online] 2022 [cited 2023 Feb 6];30:128-30. Available from: http://www.nigerianjournalofophthalmology.com/text.asp?2022/30/3/128/360559




  Introduction Top


Fuchs uveitis syndrome (FUS) is an uncommon uveitic entity that accounts for 1% to 10% of anterior uveitis cases.[1],[2] The prevalence of FUS varies between 0.4% and 13.2% in uveitis patients,[3],[4] the true prevalence might probably be higher because the heterochromia may be subtle or absent, especially in those with darker iris pigmentation. Definitive etiology remains unknown, however, correlation with rubella, varicella-zoster, herpes simplex, and ocular toxoplasmosis have been implicated in its pathogenesis.[5],[6] Diagnosis of FUS is usually clinically characterized by unilateral, chronic, low-grade anterior uveitis with variable clinical presentation. The characteristic clinical features include diffuse and stellate keratic precipitates on the corneal endothelium, diffuse iris atrophy with or without obvious heterochromia, absence of posterior synechiae, development of complications like posterior subcapsular cataract and secondary glaucoma, and absence of significant acute exacerbations.[7] FUS usually carries a favorable prognosis because of low-grade inflammation and absence of posterior synechiae. Though secondary glaucoma once established is the most challenging complication, thus mandating the need for regular and frequent follow-ups in patients diagnosed with FUS. We report findings of multifocal fibrin deposition on the entire iris surface in a young male patient presented with a clinical picture of Fuchs uveitis.


  Case Report Top


A 28-years-old male presented with recurrent complaints of blurred vision and photophobia in the left eye. Visual acuity was 20/20 and 20/25 and intraocular pressure was 12 mm Hg and 18 mm Hg in right (OD) and left (OS) eyes, respectively. Examination of OD was unremarkable. Examination of OS revealed the presence of prominent corneal nerves, diffuse stellate keratic precipitates (KPs) [Figure 1]a, cells 1+, and flare 1+. There was diffuse, multifocal fibrin deposition over the entire iris surface, in a background of classic “moth-eaten” appearance [Figure 1]b. The lesion did not appear to correspond with the characteristics of Busacca’s nodules; since they did not arise from the substance of iris, but rather had a stuck-on appearance—akin to surface deposits. However, characteristic Koeppe’s nodules were present on the pupillary margin [Figure 1]b. Gonioscopy (OS) revealed fine, lacy vessels and open angles in all quadrants. Corneal sensations were preserved, and dilated fundus examination was normal. Routine investigations including complete blood count (CBC), erythrocyte sedimentation rate (ESR), chest X-ray, Mantoux, VDRL, and HIV were performed and were found unremarkable. This constellation of signs pointed toward the diagnosis of FUS, which remains predominantly clinical. The patient was started on topical corticosteroid (prednisolone acetate 1% every 3 h) and topical cycloplegic (homatropine 2% twice a day) and followed up for 4 weeks with gradual tapering of topical steroids. However, anterior chamber tap for viral PCR test was withheld during this visit in view of good response to treatment.
Figure 1 Left eye slit-lamp photographs. (a) High magnification view showing prominent corneal nerve (yellow arrow) and multiple stellate keratic precipitates (red arrow). (b) Multifocal fibrin deposition on the entire iris surface (black arrowheads), moth-eaten appearance with diffuse atrophy of iris surface, and Koeppe’s nodules (white arrowhead).

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  Discussion Top


FUS is a chronic, unilateral, low-grade iridocyclitis that is often underdiagnosed and overtreated.[8],[9] Reasons behind underdiagnosis are consideration of evident heterochromia as A hallmark feature for FUS diagnosis and to neglect vitreous opacities, which can be often the first symptom, as a part of the diagnosis.[9] Specific atrophic changes in the iris with or without heterochromia, diffuse keratic precipitates, minimal cells and flare in the anterior chamber with few cells and opacities in anterior vitreous phase, the lack of posterior synechia, the frequent presence of cataracts and secondary glaucoma, and absence of significant acute exacerbations represent the typical natural course of FUS.[7] Typical cases have minimal cells and flare in the anterior chamber as a result of the breakdown of blood–aqueous barrier rather than the active inflammation, and this is one of the major factors behind the poor response of Fuchs uveitis to corticosteroid therapy.[10] Moreover, unnecessary and long-term steroid therapy is associated with side effects like accelerated cataract formation and glaucoma that may present with greater complications than the disease.

The unusual finding of multifocal, diffuse fibrin deposit was thought to be an exaggerated inflammatory response in FUS, which has not been reported in the literature, as part of the natural course of the disease.[7],[11] Following this observation, the patient was treated with topical corticosteroids and cycloplegics. The fibrin over the iris surface fully cleared over 2 weeks with partial resolution of KPs and anterior chamber inflammation. The patient is on continued follow-up in view of the recurrent nature of this disease and maintains minimal aqueous cells and flare that do not interfere significantly with visual function. The patient has been counseled regarding the chronic nature of the disease, regular follow-up for possible development of secondary glaucoma, and the need for surgical intervention when the cataract becomes visually significant.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Kazokoglu H, Onal S, Tugal-Tutkun I et al. Demographic and clinical features of uveitis in tertiary centers in Turkey. Ophthalmic Epidemiol 2008;15:285-93.  Back to cited text no. 1
    
2.
Rathinam SR, Namperumalsamy P. Global variation and pattern changes in epidemiology of uveitis. Indian J Ophthalmol 2007;55:173-83.  Back to cited text no. 2
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3.
Oruc S, Kaplan AD, Galen M, Kaplan HJ. Uveitis referral pattern in a Midwest University Eye Center. Ocul Immunol Inflamm 2003;11:287-98.  Back to cited text no. 3
    
4.
Thean LH, Thompson J, Rosenthal AR. A uveitis register at the Leicester Royal Infirmary. Ophthalmic Epidemiol 1996;3:151-8.  Back to cited text no. 4
    
5.
Cimino L, Aldigeri R, Parmeggiani M et al. Searching for viral antibodies and genome in intraocular fluids of patients with Fuchs uveitis and non-infectious uveitis. Graefes Arch Clin Exp Ophthalmol 2013;251:1607-12.  Back to cited text no. 5
    
6.
Kreps EO, Derveaux T, De Keyser F, Kestelyn P. Fuchs’ uveitis syndrome: no longer a syndrome? Ocul Immunol Inflamm 2016;24:348-57.  Back to cited text no. 6
    
7.
Liesegang TJ. Clinical features and prognosis in Fuchs’ uveitis syndrome. Arch Ophthalmol 1982;100:1622–6.  Back to cited text no. 7
    
8.
La Hey E, de Jong PT, Kijlstra A. Fuchs’ heterochromic cyclitis: review of the literature on the pathogenetic mechanisms. Br J Ophthalmol 1994;78:307-12.  Back to cited text no. 8
    
9.
Norrsell K, Sjödell L. Fuchs’ heterochromic uveitis: a longitudinal clinical study. Acta Ophthalmol. 2008;86:58-64.  Back to cited text no. 9
    
10.
Meacock WR, Spalton DJ, Bender L et al. Steroid prophylaxis in eyes with uveitis undergoing phacoemulsification. Br J Ophthalmol 2004;88:1122-4.  Back to cited text no. 10
    
11.
Nalçacıoğlu P, Çakar Özdal P, Şimşek M. Clinical characteristics of Fuchs’ uveitis syndrome. Turk J Ophthalmol 2016;46:52-57.  Back to cited text no. 11
    


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