Association of Fuchs Heterochromic Iridocyclitis with Multiple Sclerosis.

PURPOSE:  To draw attention to the higher proportion of Fuchs heterochromic iridocyclitis (FHI) cases in patients with multiple sclerosis (MS). MATERIALS AND METHODS:  Retrospective study of data collected at the Center for the Diagnosis and Treatment of Uveitis. RESULTS:  An analysis of the medical records of 3016 patients with uveitis (in the years 2003-2020) was performed with a focus on MS. MS-associated uveitis was diagnosed in 90 patients (3%): anterior uveitis (n = 7), intermediate uveitis (n = 23), retinal vasculitis (n = 24), and panuveitis (n = 36). A clinical examination revealed signs of FHI in the anterior segment in 11 out of 90 cases (12%). Atypical manifestations of FHI included a higher incidence of bilateral involvement (45%), retinal vasculitis (27%), and vitreous snowballs (18%). The diagnosis of FHI preceded the diagnosis of MS in 4 cases. The median latency was 10.5 (range 8-15) years. In 4 patients, the diagnosis of demyelinating disease was established within one year of the diagnosis of FHI. We recommended a neurological examination for optic neuritis (n = 1), paresthesia (n = 3), relapse of motor deficit (n = 1), and screening of etiology in cases with involvement of the posterior segment (n = 3). In the other 3 cases, the diagnosis of MS preceded the diagnosis of FHI, with a median latency of 13 (range 8-19) years. CONCLUSION:  We detected clinical symptoms of FHI in 12% of uveitis cases associated with MS, more often in bilateral manifestations of intraocular inflammation. Based on our experience, we recommend an investigation of the medical history of patients with FHI for manifestations of sensitive, sensory and motor deficits, especially in bilateral cases.

Features of hemodynamic and immunological parameters in patients with recurrent uveitis complicated by hypertension, Fuchs heterochromic uveitis and Posner-Schlossman syndrome.

Introduction: Uveitis is a disease that manifests with increased vascular permeability and occlusion, with some ischemia and inflammatory mediators. It is characterized by a wide range of pathological processes, including inflammation, increased vascular permeability and occlusion, local ischemia and cell alteration by inflammatory mediators, and is characterized by the presence of complications. Aim: To study the state of ocular hemodynamics by rheoophthalmography, as well as the immune status in patients with idiopathic recurrent anterior uveitis complicated by intraocular hypertension, Fuchs heterochromic uveitis, Posner-Schlossman syndrome, during the relapse period. Materials and methods: 93 patients with idiopathic recurrent anterior uveitis were included in this study, 8 patients with Fuchs' uveitis, and 6 patients with Posner-Schlossman syndrome. According to clinical signs, relapse and remission were considered. The control group (healthy volunteers of the same age) consisted of 27 people. In this regard, 5 groups of subjects were formed. The mean age of the patients was (M ± SD) 39.2 ± 14.6 years. According to the Median (range), the duration of the disease in patients was 2033 (350-3285) days, intraocular hypertension being recorded at P0 > 20 mm Hg. Statistical analysis was carried out in spreadsheets using STATISTICA 8.0 (StatSoft.Inc) program. Quantitative indicators were evaluated according to the correspondence to the normal distribution and to the Kolmogorov-Smirnov criterion. With a normal distribution, arithmetic means (M) and standard deviations (SD), limits of the 95% confidence interval (95% CI) and Student's t-test were calculated. Results: The volumetric blood filling of the eye according to the rheoophthalmographic indicator RQ during the period of remission of uncomplicated and complicated by hypertension anterior uveitis was reduced by 32.4%-40.5%, respectively, compared with the norm. During the period of relapse, RQ was significantly higher by 28% (p<0.05) than in remission, in the group of uncomplicated uveitis, and in the group of uveitis with increased IOP, no significant differences between the periods of remission and relapse were observed, which reflected the ischemic process in the relapse period. Volumetric blood filling in Fuchs and Posner-Schlossman syndromes in the acute period did not differ from the norm. Cellular immunity in the groups of uncomplicated and complicated by intraocular hypertension idiopathic uveitis, as well as with Fuchs and Posner-Schlossman syndromes, had a higher level of CD4 helper lymphocytes and a lower level of CD8 suppressor lymphocytes, which reflected higher values of the immunoregulatory index. The increase in the immunoregulatory index is most pronounced in Fuchs and Posner-Schlossman syndromes. Discussion: In the presented study, the incidence of idiopathic recurrent anterior uveitis complicated by intraocular hypertension was 9,9% among all cases of idiopathic recurrent anterior uveitis in one-time period. According to literature, this complicated form of uveitis occurs in 11,5%-46,1% of cases. Most often (up to 92% of cases), the anterior chamber angle was open. Conclusions: Different activity of the mechanisms regulating the balance of cellular and humoral immunity, sensitivity of T-cells to eye antigens in idiopathic anterior uveitis, Fuchs and Posner-Schlossman syndromes was assumed. Peculiarities of eye hemodynamics in these forms of uveitis were also revealed. Abbreviations: IOP = intraocular pressure, IOHS = inflammatory ocular hypertension syndrome, HSV = herpes simplex virus, CMV = cytomegalovirus, OCT = optical coherence tomography, OD = right eye, OS = left eye.

Atypical herpes zoster ophthalmicus with madarosis of upper eyelid, recurrent iridocyclitis and atrophic multifocal chorioretinopathy.

INTRODUCTION: Ocular involvement due to varicella-zoster virus (VZV) infection includes conjunctivitis, scleritis, keratitis, uveitis, and necrotizing retinitis. Non-necrotizing chorioretinopathy as a late manifestation has been described. CASE REPORT: A 50-year-old immunocompetent man developed herpes zoster ophthalmicus (HZO) in the right V1 dermatome with acute anterior uveitis (AAU) treated with oral valaciclovir and topical steroid and a chalazion in the upper eyelid with associated madarosis. Four months later, he presented recurrence of the AAU and multiple areas of chorioretinal atrophy on fundoscopy. Biopsy of the upper eyelid lesion revealed granulomatous inflammation of the eyelid margin and polymerase chain reaction study (PCR) tested positive for VZV-specific DNA. The iridocyclitis was resolved with oral valaciclovir at maximum doses with minimal choroidal pigmentary changes. DISCUSSION: VZV ophthalmic infection starts by reactivation from the trigeminal ganglion, and it spreads to the isthmus of the pilosebaceous follicles and the epidermis, which can cause involvement of follicle and sebaceous glands. Chorioretinopathy is a rare form of late-onset non-necrotizing herpetic uveitis characterized by atrophic-appearing hypopigmented lesions, the pathogenesis of which is unknown. A direct viral infection or secondary to occlusive choroidal vasculitis is postulated at the level of the choriocapillaris and more recently it has been referred to as "choroidal vitiligo" due to possible involvement of choroidal melanocytes, as occurs in cases of cutaneous vitiligo due to VZV infection.

Optic coherence tomography angiography findings in fuchs heterochromic iridocyclitis.

PURPOSE: To evaluate macular capillary perfusion in patients with fuchs heterochromic iridocyclitis (FHI) by using optical coherence tomography angiography (OCTA). MATERIAL AND METHOD: A total of 19 eyes of 19 patients with unilateral FHI underwent detailed eye examination. OCTA (RTVue-XR Avanti) images were obtained from both eyes. OCTA parameters, including foveal avascular zone, superficial capillary plexus and deep capillary plexus vessel densities, were compared between the involved and fellow control eyes. RESULTS: The median age of the patients (11 females, 8 males) was 42.0 ± 9.63 (range 24-57) years. DCP and SCP densities at the parafoveal and perifoveal area were significantly lower in the FHI eyes compared to the control eyes (44.80 ± 5.24% vs. 54.70 ± 3.76% and 43.30 ± 5.10% vs. 53.70 ± 2.73%, respectively; p < 0.05). The median FAZ was 0.29 ± 0.12 (0.11-0.42) mm2 in the FHI eyes and 0.26 ± 0.09 (0.10-0.40) mm2 in the control eyes. This difference did not reach statistical significance (p = 0.199). CONCLUSION: Macular capillary perfusion was significantly reduced in both SCP and DCP in the eyes with FHI. FHI, which is known to affect the choroid layer, could also compromise macular capillary perfusion of the retina.

Development of revised diagnostic criteria for Fuchs' uveitis syndrome in a Chinese population.

BACKGROUND/AIMS: Fuchs' uveitis syndrome (FUS) is one of the frequently misdiagnosed uveitis entities, which is partly due to the absence of internationally recognised diagnostic criteria. This study was performed to develop and evaluate a set of revised diagnostic criteria for FUS. METHODS: The clinical data of Chinese patients with FUS and patients with non-FUS were collected and analysed from a tertiary referral centre between April 2008 and December 2020. A total of 593 patients with FUS and 625 patients with non-FUS from northern China were enrolled for the development of diagnostic criteria for FUS. Three hundred and seventy-seven patients with FUS and 503 patients with non-FUS from southern China were used to validate the criteria. Clinical symptoms and ocular signs were collected from all patients with FUS and patients with non-FUS. Multivariate two-step cluster analysis, logistic regression and decision tree algorithms in combination with the clinical judgement of uveitis experts were used to revise diagnostic criteria for FUS. RESULTS: Three essential findings including diffuse iris depigmentation, absence of posterior synechiae, mild inflammation in the anterior chamber at presentation and five associated findings including mostly unilateral involvement, cataract, vitreous opacities, absence of acute symptoms and characteristic iris nodules were used in the development of FUS diagnostic criteria. All essential findings were required for the diagnosis of FUS, and the diagnosis was further strengthened by the presence of associated findings. CONCLUSION: Revised diagnostic criteria for FUS were developed and validated by analysing data from Chinese patients and showed a high sensitivity (96.55%) and specificity (97.42%).

Subfoveal Choroidal Thickness and Its Intereye Differences in Fuchs Uveitis Syndrome Evaluated Using Optical Coherent Tomography.

Purpose: To measure the subfoveal choroidal thickness (SFCT) and assess intereye subfoveal choroidal thickness difference (ISFCTD) in patients with unilateral Fuchs Uveitis Syndrome (FUS) compared with healthy controls.Methods: Forty-two patients with unilateral FUS were included in this observational retrospective study. SFCT in both eyes was measured in patients and controls using optical coherent tomography. The measurements were analyzed and compared as follows: for SFTC-affected eye vs fellow eye (FUS); affected eye (FUS) vs right control eye; fellow eye (FUS) vs left control eye; for ISFCTD - FUS patients vs controls. In addition, measurement error analysis was performed.Results: No significant differences in SFCT between the compared eyes were found (p > .05). The mean ISFCTD was 57.24 ± 40.8 µm in FUS patients and 30.33 ± 25.48 µm in controls (p < .,001).Conclusion: The ISFCTD was higher in FUS patients than in controls. There were no statistically significant differences in SFCT between the compared eyes.

Bullous central serous chorioretinopathy and retinal pigment epithelium sequelae postblunt trauma.

A 68-year-old man presented with diminution of distance and near vision in the right eye for a duration of 1 month postblunt trauma with a stick. On examination, his visual acuity in the right eye was 20/320 and near vision was

Choroid vascularity index as a parameter for chronicity of Fuchs' uveitis syndrome.

PURPOSE: This study aimed to compare the choroidal vascularity index (CVI) of eyes having Fuchs' uveitis syndrome (FUS) with healthy fellow eyes (N). METHODS: This prospective, cross-sectional study included unilateral FUS cases and an age- and gender-matched healthy control group. Thirty-nine participants were included in the FUS group, and 24 age- and gender-matched individuals were randomly selected for the control group. Endothelial cell density (ECD) was measured using Tomey specular microscopy. Spectral-domain optical coherence tomography was used to acquire the choroidal images, and binarization was applied to the images. Two blinded investigators analyzed the CVI in both eyes of the FUS cases and the right eyes of the healthy control group. RESULTS: CVI was found to be significantly decreased in FUS (p < 0.001). Additionally, ECD had a strong positive correlation with CVI (r = 0.383, p = 0.008). CONCLUSION: CVI may provide information about the chronicity of the disease.

Corneal endothelium in unilateral Fuchs heterochromic iridocyclitis.

Purpose: To assess the corneal endothelium in patients with Fuchs heterochromic iridocyclitis (FHI) and compare it with the normal fellow eye. Methods: Retrospective, observational, cross-sectional study of 31 patients seen between Jan 2016 to Dec 2018, with clinical diagnosis of Fuchs heterochromic iridocyclitis, was performed. Specular microscopic examination was documented in both eyes. The affected eyes formed the study group and the fellow healthy eyes served as controls. Results: The mean age of the patients was 29.9 ± 8.2 years. The endothelial cell density (P = 0.0001) was significantly lower, whereas average cell size (P = 0.0001), coefficient of variation (P = 0.004), and maximum cell area (P = 0.01) were significantly higher in the affected eye compared to the control eye. In three patients, the affected eye showed guttae, while the healthy fellow eye revealed a normal specular mosaic. Conclusion: Specular microscopic analysis shows endothelial alterations in the affected eyes in FHI.

Analysis of retinal microvasculature in Fuchs' uveitis syndrome. Retinal microvasculature in Fuchs' uveitis.

PURPOSE: The objective of this study is to quantitatively analyse the foveal microvasculature in eyes with Fuchs' Uveitic Syndrome (FUS), or Fuchs' Heterochromic Iridocyclitis (FHI), by Optical coherence tomography angiography (OCTA). METHODS: Thirty patients with FUS and 30 healthy volunteer patients (control group) were enrolled in the study. Vascular density (VD) in the superior and deep capillary plexuses (SCP, DCP) were reported and compared between eyes with FUS (FU), fellow eyes (FE) and the control group. RESULTS: Foveal VD and parafoveal VDs in all quadrants of the SCP were significantly lower in the FU group than the FE group and normal eyes (P<0.05). Foveal VDs in the DCP were similar between the three groups (P>0.05); however, parafoveal VDs in all quadrants of the DCP were significantly lower in the FU group than in the FE and control eyes (P<0.05). Foveal and parafoveal VDs in both the SCP and DCP were similar between fellow eyes and the control group. CONCLUSION: Fuchs' Uveitic Syndrome (Fuchs' Heterochromic Iridocyclitis) affects not only the anterior uvea and vitreous but also the retinal microvasculature. Analysis by OCT-A may enable us to understand the extent of this disease.

Síndrome de despigmentación aguda bilateral de iris y transiluminación aguda bilateral de iris / Bilateral acute iris depigmentation and bilateral acute iris transillumination síndrome

Varón de 32 años que acude con un cuadro agudo bilateral caracterizado por visión borrosa, ojo rojo, fotofobia severa y dolor ocular tras un cuadro seudogripal. El paciente presentaba un cuadro con afectación bilateral caracterizado por pupilas en midriasis media, escasamente reactivas a la luz, transiluminación del iris, despigmentación difusa del estroma iridiano, dispersión de pigmento en la cámara anterior e hipertensión ocular. Tras el examen ocular se descartó un cuadro inflamatorio y un glaucoma pigmentario. El paciente presentaba características tanto de la despigmentación como de la transiluminación bilateral de iris. Ambas entidades podrían formar parte del espectro de la misma enfermedad

The case is presented of a 32 year-old male who arrived with acute bilateral symptoms with blurred vision, red eye, severe photophobia and severe ocular pain after suffering from a flu-like syndrome. The patient presented with a clinical picture of bilateral involvement characterised by pupils in mid-mydriasis, scarcely reactive to light, iris transillumination, diffuse depigmentation of the iridian stroma, pigment dispersion in the anterior chamber, and ocular hypertension. After the eye examination an inflammatory syndrome and pigmentary glaucoma were ruled out. The patient showed depigmentation characteristics as well as bilateral iris transillumination. Both conditions could form part of the spectrum of the same disease

Predictive Factors of Intraocular Pressure Level Evolution Over Time and Glaucoma Severity in Fuchs' Heterochromic Iridocyclitis.

Purpose: To investigate the clinical and virologic-associated and predictive factors of intraocular pressure (IOP) evolution over time and its severity in Fuchs' heterochromic iridocyclitis (FHC). Methods: Consecutive patients with both clinical FHC and intraocular synthesis of rubella virus (RV)-specific antibodies were included in this study. Specific ocular production of RV antibodies was confirmed using the quotient of serum/aqueous humor ratio of RV IgGs (Crv) and control antiviral IgGs (Cctl), using quantitative serology methods. Epidemiologic, clinical, biological, and virologic data at referral were collected and correlated with IOP values over time, occurrence, and severity of glaucoma. Results: Sixty-eight eyes of 68 patients were included. Mean age at diagnosis was 40.7 ± 11.1 years. Mean follow-up was 4.3 ± 4.3 years. Mean baseline Crv and Cctl values were 12.34 ± 14.67 and 216.70 ± 98.4, respectively. Mean baseline IOP was 17.2 ± 7.2 mm Hg (range, 9-40) and 15.6 ± 5.6 (range, 3-30) 5 years after referral. The predictive factors for pejorative IOP evolution over time and glaucoma severity were male sex (P = 0.03) and decreased Crv (P = 0.04) and presence of iris nodules (P < 0.001) and decreased Cctl (P = 0.02), respectively. Diagnostic delay was associated with increased likelihood of undergoing glaucoma surgery (P = 0.02). Conclusions: Time to diagnosis, male sex, presence of iris nodules at baseline, and decreased Crv and Cctl ratios were associated with increased likelihood of pejorative IOP evolution over time. Given the aggressiveness of glaucoma in FHC, these results provide interesting insight into what category of patients should need the closest screening.

Bilateral acute iris depigmentation and bilateral acute iris transillumination syndrome. / Síndrome de despigmentación aguda bilateral de iris y transiluminación aguda bilateral de iris.

The case is presented of a 32 year-old male who arrived with acute bilateral symptoms with blurred vision, red eye, severe photophobia and severe ocular pain after suffering from a flu-like syndrome. The patient presented with a clinical picture of bilateral involvement characterised by pupils in mid-mydriasis, scarcely reactive to light, iris transillumination, diffuse depigmentation of the iridian stroma, pigment dispersion in the anterior chamber, and ocular hypertension. After the eye examination an inflammatory syndrome and pigmentary glaucoma were ruled out. The patient showed depigmentation characteristics as well as bilateral iris transillumination. Both conditions could form part of the spectrum of the same disease.