Abstract
Purpose:
To describe the clinical profile of a large cohort of patients with Fuchs uveitis in a tertiary eyecare institute in India.
Methods:
Retrospective analysis of all patients with Fuchs uveitis visiting the uveitis clinic of a tertiary eye institute between January 2013 and January 2023.
Result:
The study included a total of 346 eyes of 339 patients with Fuchs uveitis (mean age 34.0 ± 11.7 years). Among the presenting complaints, 332 patients (97.9%) reported diminished vision, and 52 (15.3%) reported floaters. Misdiagnosis occurred in 25% patients. Clinical findings included a loss of fine iris pattern (100%), stellate keratic precipitates (69.3%), cataract (84.3%), vitreous membranes (44.2%), heterochromia (6.4%), iris nodules (9.2%), and iris atrophy (21.7%). Post-cataract surgery complications were rare, with comparable outcomes between preoperative steroid and nonsteroid groups. Visual acuity improved from 0.77 at the time of presentation to 0.11 logMAR at the time of final follow-up.
Conclusion:
Fuchs uveitis remains frequently underdiagnosed and overtreated in the Indian setting, often due to diagnostic challenges such as bilaterality, reliance on iris heterochromia in dark irides, and vitreous involvement. Our study suggests that systemic corticosteroids do not influence visual outcomes after cataract surgery in these patients.
Keywords: Anterior uveitis, cataract, Fuchs uveitis, intermediate uveitis, vitritis
The earliest description of Fuchs uveitis was by Ernst Fuchs, who described uveitis with heterochromia in 1906.[1] It is characterized by chronic, typically unilateral, usually asymptomatic mild inflammation predominantly affecting the anterior uvea and vitreous.[1,2] Fuchs uveitis is characterized by clinical signs such as minimal circumciliary congestion, stellate keratic precipitates (KPs), mild anterior chamber inflammation, and iris atrophy, often with heterochromia, occasional vitritis, and an absence of synechiae.[1,2] Over half of patients develop elevated intraocular pressure (IOP), and more than 80% of affected eyes develop posterior subcapsular cataracts.[1]
Retrospective data from tertiary eye care centers in India report that Fuchs uveitis accounts for 1–8% of all uveitis cases.[3,4] There is a paucity of literature on the clinical features and demography of patients with Fuchs uveitis from India.[5,6] The index study was conducted in a tertiary care eye institute in India to analyze the clinical profile of a large cohort of patients with Fuchs uveitis.
Methods
This was a hospital-based retrospective case series that reviewed the files of all consecutive patients with Fuchs uveitis at a single tertiary eyecare centre between January 2013 and January 2023. The study was approved by the institutional ethics committee, and it adhered to the tenets of the Declaration of Helsinki. The diagnosis of Fuchs uveitis was made based on the presence of the following characteristic clinical features, including: a lack of acute symptoms such as pain, photophobia, or ciliary injection; small- to medium-sized KPs covering the entire endothelial surface; a chronic, low-grade anterior chamber reaction; diffuse iris stromal atrophy with or without heterochromia; absence of posterior synechiae; and no signs of retinal vasculitis, snowbanks, or chorioretinal infiltrates, despite the presence of vitreous cells and debris. Other causes of uveitis were excluded when there was diagnostic uncertainty.
Patient records were accessed from the electronic medical records database of our institution. Data collected included demographic details (age, sex, laterality), clinical presentation, disease course, ocular involvement, etiology, associated systemic findings, and treatment outcomes. For each patient, we documented presenting complaints, comorbidities, and any history of previous misdiagnosis. A comprehensive ophthalmic examination was conducted for each patient, which included best-corrected visual acuity (BCVA), slit-lamp biomicroscopy, and dilated fundus examination. For patients undergoing surgery, details such as perioperative steroid use were noted. Post-surgical assessments recorded BCVA, inflammation status, and the presence of cystoid macular edema (CME). Any complications post-implantation, including posterior capsule opacification (PCO), and elevated IOP (>21 mm Hg), were carefully documented.
In cases of atypical clinical findings, patients underwent further ophthalmologic imaging, including fluorescein angiography and optical coherence tomography. Laboratory investigations—complete blood count, C-reactive protein, QuantiFERON TB Gold, chest X-ray, chest CT, Human immunodeficiency viruses (HIV) screening, Toxoplasma testing, Venereal Disease Research Laboratory test, and Treponema pallidum hemagglutination assay—were performed wherever clinically indicated, as the diagnosis of Fuchs uveitis remains primarily clinical but ultimately one of exclusion. For patients with suspected pulmonary tuberculosis, sputum samples were taken for acid-fast bacilli staining and mycobacterial culture. When clinically indicated and feasible for the patient, aqueous humor samples were collected for polymerase chain reaction (PCR) analysis.
All the data were entered in Microsoft Excel, and data analysis was performed. Mean ± standard deviation was used to represent continuous variables. Categorical variables were presented as proportions. A paired t-test was used to determine statistical significance.
Results
The study included a total of 346 eyes of 339 patients with Fuchs uveitis [Table 1]. There were 149 females and 190 males. Of these, 332 patients had unilateral presentation, while seven had bilateral presentation (2%) [Fig. 1]. The mean age of the patients was 34.0 ± 11.7 years (range, 10–79 years). Among the presenting complaints, 332 patients (97.9%) reported diminished vision, and 52 (15.3%) reported floaters. Ocular pain was noted in 24 patients (7%). Three patients presented for a routine check-up and were diagnosed with Fuchs uveitis during the examination. A history of trivial trauma was present in 27 (7.9%) patients. Systemic comorbidities included diabetes mellitus in 32 patients (9.4%), hypertension in 32 patients (9.4%), and hypothyroidism in 15 patients (4.4%).
Table 1.
Clinical profile of patients with Fuchs uveitis
| Total number of patients | 339 |
|---|---|
| Total number of eyes | 346 |
| Laterality: | |
| Unilateral Bilateral |
332 (97.9%) 7 (2.1%) |
| Gender: | |
| Male Female |
190 (56%) 149 (44%) |
| Presenting complaints: | |
| Diminished vision Floaters Ocular pain |
332 (97.9%) 52 (15.3%) 24 (7%) |
| Systemic diseases: | |
| Diabetes Mellitus Hypertension Hypothyroidism |
32 (9.4%) 32 (9.4%) 15 (4.4%) |
| Misdiagnosis of Fuchs uveitis: | |
| Anterior Uveitis (other than Fuchs Uveitis) Intermediate Uveitis/Pars planitis |
27 (7.8%) 61 (17.6%) |
| Ocular features (eyes): Loss of fine iris pattern Stellate keratic precipitates Heterochromia Iris nodules Iris atrophy Cataract Vitreous membranes/debris |
346 (100%) 240 (69.3%) 22 (6.4%) 32 (9.2%) 75 (21.7%) 292 (84.3%) 153 (44.2%) |
Figure 1.
(a and b): Slit-lamp photographs of both eyes of a case of bilateral Fuchs uveitis
All patients exhibited a loss of fine iris patterns, and stellate KPs were noted in 240 eyes (69.3%). Heterochromia was observed in 22 eyes (6.4%). Iris nodules were seen in 32 eyes (9.2%), and iris atrophy was noted in 75 eyes (21.7%). Cataract was observed in 292 eyes (84.3%), while vitreous membranes or debris were present in 153 eyes (44.2%). The fundus appeared normal in all patients, although glaucomatous cupping was identified in 19 eyes (5.4%).
One fourth of these patients were initially diagnosed with entities other than Fuchs uveitis. A diagnosis of intermediate uveitis was made in 61 patients (17.6%), and anterior uveitis other than Fuchs uveitis was erroneously made in 27 patients (7.8%).
At presentation, 229 patients (67.6%) were treatment-naïve. Among those previously treated, 54 patients (15.9%) had received topical steroids, 40 (11.8%) had been treated with oral steroids, 25 (7.4%) had undergone posterior sub-Tenon’s steroid (PST) injections, and four (1.2%) had received intravitreal steroids. Additionally, three patients had been managed with immunomodulatory therapy, one with antituberculosis therapy, and one with antiviral medication. Glaucoma was diagnosed in 25 eyes (7.3%) at presentation. Of these, 22 patients were already using antiglaucoma medications, and two had undergone filtration surgery prior to presentation. Additionally, 21 eyes developed glaucoma during follow-up, bringing the total to 46 eyes (13.3%) with Fuchs uveitis that developed glaucoma. Five of the newly diagnosed glaucoma cases required surgical intervention, and the rest were effectively managed with antiglaucoma medications alone.
PCR from aqueous aspirate was performed in eight eyes (2.3%). Among these, five eyes tested negative for all herpes viruses, two were positive for Mycobacterium tuberculosis (MTB), and one was positive for cytomegalovirus (CMV). Both patients with MTB-positive PCR results underwent further evaluation with a Mantoux test, interferon-gamma release assay, and high-resolution chest computed tomography, and a pulmonologist subsequently ruled out latent tuberculosis in both cases. The patient positive for the CMV genome from aqueous aspirate did not receive any antiCMV treatment.
A total of 253 eyes underwent cataract surgery for complicated cataracts, with 195 eyes operated under perioperative steroid cover and 58 eyes without steroid cover. Postoperatively, early PCO requiring YAG capsulotomy occurred in two eyes (0.79%), CME developed in two eyes (0.8%), and persistent inflammation treated with PST occurred in another two eyes (0.8%). Among these six eyes with post-surgical complications, four (66.7%) had been operated under steroid cover. In comparing cataract surgery outcomes for Fuchs uveitis patients, there was no significant difference in postoperative visual acuity between the two groups. Mean visual acuity in the steroid-covered group was 0.0492 ± 0.15 logMAR, while in the nonsteroid group it was 0.101 ± 0.48 logMAR (P = 0.401). Three eyes underwent optical vitrectomy for removal of vitreous debris, as the patients complained of significant visual disturbances due to floaters following cataract surgery.
The median duration of follow-up was 380 days. At presentation, the mean visual acuity was 0.77 logMAR, which improved to 0.11 logMAR at the final visit. However, vision remained poor in 25 eyes at the final follow-up. Causes included cataract in 15 eyes, full-thickness macular hole in one eye, advanced glaucomatous cupping with disc pallor in two eyes, epiretinal membrane in one eye, rhegmatogenous retinal detachment in two eyes, and isolated disc pallor in one eye. Additionally, three eyes developed corneal decompensation following surgery: one case after Ahmed glaucoma valve implantation and two cases post-phacoemulsification. One eye had both Fuchs endothelial dystrophy and Fuchs uveitis.
Discussion
This study analyzed the demographic and clinical profiles of 339 patients with Fuchs uveitis, with a particular focus on cataract surgery outcomes in this cohort. To the best of our knowledge, this represents the largest reported series on Fuchs uveitis from India.
Demographic and clinical characteristics of the patients reported in the index study were in accordance with most of the studies described in the literature. The mean age of patients in our series was 34, consistent with other studies in the literature but higher than the mean age reported in other Indian studies.[7,8,9,10] In the current series, 2% of patients had bilateral presentations, which is lower compared to the bilateral cases observed in other series. Bilateral cases have been reported in up to 21% of patients with Fuchs uveitis.[11,12] Fuchs uveitis, often considered a unilateral entity, may present bilaterally—an atypical feature that can contribute to misdiagnosis in clinical practice. Chief presenting complaints in patients with Fuchs uveitis remains blurring or diminished vision, outnumbering other symptoms.[10,11] In our series, 98% of patients presented with diminished vision, suggesting a probable delay in presentation, which may partly explain the relatively higher age of presentation in our patients.
Fuchs uveitis remains an often underdiagnosed and overtreated condition in the spectrum of uveitis. In our series, one-fourth of patients were initially misdiagnosed, most commonly as having intermediate uveitis, due to overlapping features. Patients were also treated with immunosuppressives and antituberculosis therapy based on misdiagnosis. A similar observation was reported by another study from Northern India, where half of the patients in the series were erroneously diagnosed and received medication for the misdiagnosed condition.[3] Intermediate uveitis or pars planitis remains the most common misdiagnosis of Fuchs uveitis in all case series.[3,4,5,6] Thus, anterior uveitis with vitreous involvement can be mistaken for intermediate uveitis [Fig. 2]. CME in Fuchs uveitis is extremely uncommon and is more commonly seen as a complication of intermediate uveitis or pars planitis. In our series, only 0.8% of eyes developed CME, and this occurred as a complication of cataract surgery. Such misdiagnosis in Fuchs uveitis is more likely to occur when the diagnosis of the clinical phenotype relies solely on iris heterochromia. In darker irides, as commonly seen in Indian patients, iris heterochromia is subtle and unreliable for diagnosing Fuchs uveitis, with only 6.4% of our patients presenting this feature. Loss of iris pattern, especially near the collarette, was a more consistent finding, noted in all patients in our series; however, this sign may be missed if not examined in an undilated pupil [Fig. 3]. Additionally, stellate KPs were observed in only 69.3% of eyes, indicating they are not a definitive sign of Fuchs uveitis. Iris nodules were seen in 9.2% of eyes in our patients. One patient in our series with Fuchs uveitis was suspected to have granulomatous uveitis and was subsequently started on antituberculosis treatment based solely on positive Mantoux test. Such diagnostic confusion can occur in patients with anterior uveitis and iris nodule in tuberculosis-endemic countries [Fig. 4]. Iris nodules without formation of synechiae, and loss of fine iris pattern in unilateral anterior uveitis, should raise suspicion of Fuchs uveitis.[7] Additionally, bilateral presentation of Fuchs uveitis can also add to diagnostic confusion. In a study of 466 eyes, Kianersi et al.[4] identified four factors responsible for mistaken diagnoses of Fuchs uveitis: presence of iris changes, bilateral involvement, cataract, and glaucoma.
Figure 2.
(a and b): Vitreous involvement in patients with Fuchs uveitis
Figure 3.
(a and b) – Slit-lamp photographs of the left eye in a case of Fuchs uveitis, highlighting the importance of undilated examination. (a). With a dilated pupil, the diagnosis of Fuchs uveitis can be missed, as the iris pattern details are obscured. (b). Undilated slit-lamp photograph showing loss of the fine iris pattern near the collarette — an important clinical clue, especially in patients with dark irises
Figure 4.
Slit-lamp photographs of Fuchs uveitis: (a and b) iris nodules, (c) moth-eaten iris atrophy, and (d) stellate keratic precipitates
PCR was conducted on aqueous aspirates from only eight eyes in the study. However, an important observation can be drawn from the two cases that tested positive for the MTB genome, highlighting how PCR in Fuchs uveitis can erroneously suggest such associations. Clinicians must be cautious when interpreting these results, paying careful attention to the clinical phenotype of the uveitic entity.[8] In a prospective, randomized analysis from India, PCR analysis of the aqueous humor of all 25 patients with Fuchs uveitis was negative for viruses such as rubella, Herpes simplex virus (HSV), Cytomegalovirus (CMV), and Varicella zoster virus (VZV).[6] The authors concluded that the diagnosis of Fuchs uveitis remains primarily clinical, emphasizing the limited utility of PCR in diagnosing the condition.[6] We also felt that PCR may not be a valuable option in Indian patients with Fuchs uveitis.
Cataract remains the most common complication in Fuchs uveitis, with reported incidence rates ranging from 15% to 75%.[2] In a study by Javadi et al.,[9] 41 eyes with Fuchs uveitis underwent cataract surgery without steroid cover. While postoperative visual acuity remained good, complications such as fibrinous reactions, IOL deposits, IOL decentration, and posterior capsular opacification were commonly observed. Milazzo et al.[10] reviewed 93 eyes operated on without steroid cover and found that 65.5% of these eyes developed IOL precipitates post-surgery. Tejwani et al.,[11] in contrast, analyzed 103 eyes operated on without steroid cover and reported excellent postoperative visual outcomes, supporting recent findings that cataract surgery can yield favorable results without the need for steroids. Consistent with these observations, our study found no significant difference in postoperative visual acuity between the steroid-covered and nonsteroid groups. Overall, the majority of patients in our series achieved good visual outcomes. However, three eyes developed corneal decompensation; among these, one had previously undergone Ahmed glaucoma valve surgery, and another had Fuchs endothelial dystrophy. Although corneal decompensation following cataract surgery is an extremely rare complication of Fuchs uveitis, it has been documented in the literature.[12]
As mentioned earlier, the diagnosis of Fuchs uveitis remains primarily clinical; however, differentiating it from other uveitic entities is crucial. The Standardization of Uveitis Nomenclature Working Group has proposed classical criteria for the diagnosis of Fuchs uveitis syndrome.[13] These include unilateral anterior uveitis without posterior segment involvement such as retinitis, the presence of heterochromia or diffuse iris atrophy, and stellate KPs without evidence of endotheliitis or nodular, coin-shaped endothelial lesions. However, one should remember that these criteria were originally intended for research purposes and emphasized the need to exclude conditions such as syphilis (using serology), sarcoidosis (via chest imaging or biopsy), and viral anterior uveitis (through PCR).[13] It is important to note that these conditions usually have distinct clinical phenotypes and can often be differentiated through meticulous clinical examination. For instance, unlike Fuchs uveitis, which shows diffuse iris atrophy, HSV and VZV anterior uveitis typically present with sectoral iris atrophy. Additionally, corneal involvement in the form of endothelitis or scarring is extremely uncommon in Fuchs uveitis. Therefore, PCR for viral or tuberculous etiologies is generally unnecessary in a straightforward case of Fuchs uveitis.
As a retrospective case series, our study has several limitations. However, we believe the index study provides a clearer understanding of the demographic and clinical profile of patients with Fuchs uveitis in India. Fuchs uveitis remains an underdiagnosed uveitic entity that is frequently misdiagnosed as another condition and, as a result, often overtreated inappropriately.
Conclusion
Fuchs uveitis continues to be an underdiagnosed and frequently misdiagnosed uveitic entity, particularly in the Indian context, where it is often confused with other forms of uveitis and consequently overtreated. Clinical factors such as bilaterality, over-reliance on iris heterochromia in darkly pigmented eyes, and prominent vitreous involvement contribute to diagnostic challenges. Our study found no significant difference in postoperative visual outcome between patients who received perioperative systemic corticosteroids and those who did not, indicating that systemic corticosteroid use does not influence visual outcomes following cataract surgery in Fuchs uveitis.
Informed consent
Written informed consent was obtained from the patients before the study.
Conflicts of interest:
There are no conflicts of interest.
Funding Statement
Nil.
References
- 1.Sun Y, Ji Y. A literature review on Fuchs uveitis syndrome: An update. Surv Ophthalmol. 2020;65:133–43. doi: 10.1016/j.survophthal.2019.10.003. [DOI] [PubMed] [Google Scholar]
- 2.Mohamed Q, Zamir E. Update on Fuchs’ uveitis syndrome. Curr Opin Ophthalmol. 2005;16:356–63. doi: 10.1097/01.icu.0000187056.29563.8d. [DOI] [PubMed] [Google Scholar]
- 3.Tandon M, Malhotra PP, Gupta V, Gupta A, Sharma A. Spectrum of Fuchs uveitic syndrome in a North Indian population. Ocul Immunol Inflamm. 2012;20:429–33. doi: 10.3109/09273948.2012.723113. [DOI] [PubMed] [Google Scholar]
- 4.Kianersi F, Kianersi H, Pourazizi M, Beni AN, Noorshargh P. Fuchs’ uveitis syndrome: A 20-year experience in 466 patients. Sci Rep. 2024;14:8621. doi: 10.1038/s41598-024-59393-w. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 5.Zarei M, Darabeigi S, Mehrpour M, Roohipoor R, Ghassemi H, Ebrahimiadib N. Fuchs’ Uveitis in Iranian patients: A review of 89 eyes. Ocul Immunol Inflamm. 2019;27:1077–85. doi: 10.1080/09273948.2018.1500612. [DOI] [PubMed] [Google Scholar]
- 6.Tugal-Tutkun I, Güney-Tefekli E, Kamaci-Duman F, Corum I. A cross-sectional and longitudinal study of Fuchs uveitis syndrome in Turkish patients. Am J Ophthalmol. 2009;148:510–515.e1. doi: 10.1016/j.ajo.2009.04.007. [DOI] [PubMed] [Google Scholar]
- 7.Rothova A, La Hey E, Baarsma GS, Breebaart AC. Iris nodules in Fuchs’ heterochromic uveitis. Am J Ophthalmol. 1994;118:338–42. doi: 10.1016/s0002-9394(14)72958-7. [DOI] [PubMed] [Google Scholar]
- 8.Chawla R, Singh MK, Singh L, Shah P, Kashyap S, Azad S, et al. Tubercular DNA PCR of ocular fluids and blood in cases of presumed ocular tuberculosis: A pilot study. Ther Adv Ophthalmol. 2022;14:25158414221123522. doi: 10.1177/25158414221123522. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 9.Javadi MA, Jafarinasab MR, Araghi AAS, Mohammadpour M, Yazdani S. Outcomes of phacoemulsification and in-the-bag intraocular lens implantation in Fuchs’ heterochromic iridocyclitis. J Cataract Refract Surg. 2005;31:997–1001. doi: 10.1016/j.jcrs.2004.08.050. [DOI] [PubMed] [Google Scholar]
- 10.Milazzo S, Turut P, Borhan M, Kheireddine A. Intraocular lens implantation in eyes with Fuchs’ heterochromic iridocyclitis. J Cataract Refract Surg. 1996;22(Suppl 1):800–5. doi: 10.1016/s0886-3350(96)80165-9. [DOI] [PubMed] [Google Scholar]
- 11.Tejwani S, Murthy S, Sangwan VS. Cataract extraction outcomes in patients with Fuchs’ heterochromic cyclitis. J Cataract Refract Surg. 2006;32:1678–82. doi: 10.1016/j.jcrs.2006.05.010. [DOI] [PubMed] [Google Scholar]
- 12.Huang Z, Wang XY, Liu L, Han W. Corneal decompensation in bilateral Fuchs heterochromic uveitis. Can J Ophthalmol. 2014;49:e11–4. doi: 10.1016/j.jcjo.2013.09.022. [DOI] [PubMed] [Google Scholar]
- 13.Standardization of Uveitis Nomenclature (SUN) Working Group Classification Criteria for Fuchs Uveitis Syndrome. Am J Ophthalmol. 2021;228:262–7. doi: 10.1016/j.ajo.2021.03.052. [DOI] [PMC free article] [PubMed] [Google Scholar]