Search

Background: The aim of the Posner-Schlossman Syndrome European Study Group (PSS-ESG) is to acquire a comprehensive dataset of European patients with PSS. Here, we present the first report on the study protocol and the clinical findings of the patients at baseline., Methods: The PSS-ESG is a retrospective, multicentre study designed to evaluate patients with PSS. The study, designed and driven by a European Expert Committee includes three datasets: (1) the baseline, (2) the follow-up and (3) the intraocular pressure (IOP)/glaucoma dataset., Results: A total of 11 centres adhered to the PSS-ESG and 107 patients were included (68 males, 39 females) mostly Caucasian (93.4%). At uveitis onset, the patient's age ranged between 11 and 76 years, (mean age: 42±15 years).Best-corrected visual acuity was >0.5 in 80.3% of the eyes, IOP was >40 mm Hg in 44% of the eyes. Keratic precipitates were found in 78.5% of the eyes. No flare or cells in anterior chamber were detected in 56% and 53% of the cases, respectively. PCR analysis on aqueous sample was positive for cytomegalovirus-DNA in 50.6% out of the 81 tested patients., Conclusions: The PSS-ESG is the first multicentre study aimed to collect a comprehensive dataset of patients with PSS in non-Asian countries. A middlde-aged Caucasian male with a low-grade anterior chamber inflammation, keratic precipitates, preserved visual acuity and marked increased in IOP seemed to be the standard PSS patient across the 11 uveitis and glaucoma centres participating in the PSS-ESG., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: The survey aims to explore the use of existing nomenclature and current clinical and multimodal imaging (MMI) approach in diagnosing white dot syndromes (WDS) among uveitis and retina specialists., Methods: The members of the International Uveitis Study Group (IUSG) task force MUV (Multimodal imaging in UVeitis) developed a survey. The questionnaire, created using Qualtrics, consisted of 22 questions. The responses were compared against regions, workplace setting, sub-specialty, and experience of the participants., Results: A total of 432 participants initiated the background section; 343 initiated the investigation section and 263/343 completed the survey (76.7%). The majority (43.7%) reported a specialty/practice focus mostly on uveitis, 32.2% on uveitis and retina, and 20.1% mostly on retina. Specifically, 55.7% were in practice > 10 years post-fellowship and 65.8% worked in academic settings. The term WDS was not universally used in clinical practice, with no significant differences by region, subspecialty, experience, workplace setting or number of WDS patients managed in the prior year ( p  > 0.01). Nearly 90% of participants reported using MMI to diagnose WDS. More than 70% advocated redefining the nomenclature and classification of WDS based on the primary anatomical location of disease using MMI without significant regional or professional differences ( p  > 0.01)., Conclusion: These results underscore the widespread adoption of MMI among uveitis and retina specialists in the characterization of entities traditionally grouped under the term WDS. Respondents strongly agree that MMI provides a precise distinction between these posterior uveitis, advocating for the overcoming of the clinical term WDS in favor of a patho-anatomic redefinition.

Objectives : To evaluate the long-term anatomical and functional prognosis of patients with retinal detachment (RD) secondary to acute retinal necrosis (ARN) treated with pars plana vitrectomy (PPV). Methods : This retrospective case series included 21 eyes from 21 patients with RD secondary to ARN. The study analyzed vitreous or aqueous biopsy results, the impact of antiviral therapy, time to retinal detachment, changes in visual acuity (VA), and anatomical and surgical outcomes. All cases underwent 23-gauge PPV with silicone oil tamponade, and an episcleral encircling band was used in 11 cases. All patients received systemic antiviral therapy at diagnosis. Results : Retinal reattachment was achieved in 91% of cases during follow-up, with an average follow-up period of 39.5 ± 36.8 months. The average time from ARN diagnosis to RD onset was 33.3 ± 27.5 days. VZV was detected in 10 eyes through PCR analysis. Significant differences in visual prognosis were found between macula-off and macula-on RD ( p = 0.048). Eyes with optic nerve head inflammation had worse final VA ( p = 0.010). No significant difference was observed between preoperative VA and VA at the end of follow-up ( p = 0.665). Conclusions : VZV was the primary virus associated with ARN-related RD. Early involvement of the macula and optic nerve in retinitis negatively impacted the final visual prognosis.

Vogt-Koyanagi-Harada (VKH) is a rare multisystem inflammatory disease affecting the eyes, ears, brain, skin, and hair. The Coronavirus Disease 2019 (COVID-19) is a new contagious infection that might trigger the onset of VKH disease, as previously proposed for other viruses. Moreover, after the mass vaccination against SARS-CoV-2 worldwide, cases of VKH disease associated with COVID-19 vaccination have been reported. We present an overview of VKH and a comprehensive literature revision of all the VKH cases described after COVID-19 infection and vaccination, adding our experience. No differences have been found considering epidemiology and clinical findings of the disease compared to those reported in the no-COVID era. All of the patients promptly responded to systemic and local corticosteroid therapy with a good final visual prognosis. Different possible pathogenetic mechanisms underlying the onset of VKH after COVID-19 vaccination are discussed, while the presence of the HLA DR4 antigen as a genetic predisposition for the onset of the disease after COVID-19 infection and vaccination is proposed. VKH disease is one of the most frequently reported uveitic entities after COVID-19 vaccination, but a good response to therapy should not discourage vaccination. Nevertheless, ophthalmologists should be alerted to the possibility of VKH occurrence or relapse after COVID-19 vaccination, especially in genetically predisposed subjects., Competing Interests: the Authors declare no conflict of interest

Purpose: to study the clinical features of uveitis-glaucoma-hyphema (UGH) syndrome, particularly those useful for a differential diagnosis from unilateral hypertensive acute anterior uveitis., Methods: A retrospective chart review was conducted on the clinical features of 9 patients with UGH syndrome. These features were then compared with those detected in 50 patients with unilateral hypertensive acute anterior uveitis., Results: Fine and pigmented keratic precipitates ( p = .0002 and p = .00004, respectively), iris atrophy ( p = .0122), hyphema and vitreous opacities > 2+ ( p = .0003), and cystoid macular edema ( p = .009) were statistically associated with UGH syndrome. These clinical signs show a high specificity, ranging from 58 to 100%; the presence of pigmented keratic precipitates in the setting of a unilateral acute hypertensive anterior uveitis has a sensitivity and specificity of 89% and 84%, respectively., Conclusion: In patients operated on for cataract, UGH syndrome can be differentiated from unilateral hypertensive acute anterior uveitis considering specific clinical signs.

Purpose: To report a relapse of Vogt-Koyanagi-Harada (VKH) disease in a patient after COVID-19 vaccination., Case Report: A VKH disease patient, well controlled on azathioprine therapy, presented a uveitis relapse eleven days after the first vaccination for COVID-19. She received an induction high-dose intravenous corticosteroid therapy, followed by oral therapy, which led to a complete recovery from the uveitis in two weeks. No relapses occurred in the following five months of follow-up. Despite high-dose corticosteroid therapy and azathioprine, and one dose only of vaccination, the patient resulted positive for anti-RBD spike COV19 antibody., Conclusion: Relapse of VKH disease can occur after COVID-19 vaccination, despite an appropriate immunosuppressive therapy is ongoing. It responds to the classic therapy for VKH, and a serological response to an incomplete COVID-19 vaccination can also be found.

Background: The purpose of this study was to evaluate the association between a novel psychometric 12-item questionnaire (U-qest) and other validated questionnaires to assess quality of life and work impairment in patients with non-infectious uveitis., Methods: Data were collected at baseline and 3 months postbaseline using U-qest and two other validated questionnaires: The National Eye Institute 25-Item Visual Function Questionnaire (VFQ-25) and the 12-Item Short-Form Health Survey (SF-12)., Results: A total of 136 patients (52.2% female) aged 47.9 ± 14.8 years (mean ± SD) were enrolled in 14 uveitis referral centres. U-qest correlated moderately with VFQ-25 and SF-12 at baseline and at 3 months. Both U-qest and VFQ-25 scores improved as disease improved; however, U-qest also detected improvement in patients for whom VFQ-25 scores did not improve. Disease activity was shown to significantly affect activity impairment. Patients and physicians expressed positive perceptions regarding the use and benefit of this instrument. U-qest showed very good reliability in terms of internal consistency (Cronbach's alpha = 0.91)., Conclusions: U-qest can be considered a useful tool to assess the burden of uveitis on quality of life.

Purpose: To study the epidemiological and clinical features of uveitis post-COVID-19 vaccination., Patients and Methods: Retrospective chart review of patients presenting with uveitis after COVID-19 vaccination in tertiary uveitis services., Results: In total, 25 patients, 76% females, mean age 43.2 years, were included. Uveitis occurred after Pfizer, Moderna, Astra-Zeneca and Covaxin vaccination. Anterior uveitis was the most frequent type of uveitis (56%). History of uveitis was found in 19 cases (76%), among whom 90.9% of the tested patients developed anti-Sars-CoV-2 spike antibodies after vaccination. In a mean follow-up of 5 months, one-line decrease in visual acuity was found in 12% of patients. During post-vaccine uveitis, 15.8% of cases needed an increase in their systemic therapy. According to Naranjo score, new-onset uveitis had a higher probability of being associated with vaccination (p < .01)., Conclusion: COVID-19 vaccination can cause uveitis but has no significant impact on the visual prognosis after resolution.

Background: Vogt−Koyanagi−Harada (VKH) disease is a form of uveitis that is rare in Western countries. The aim of this study was to report on the long-term real-life treatment and prognosis of VKH in Italy. Methods: The clinical features, complications, and final visual acuity were retrospectively evaluated in 38 patients with VKH (mean follow-up: 120 months) globally, according to oral or intravenous corticosteroid treatment at onset and subsequent immunosuppressive therapy. Results: The mean final visual acuity was 0.13 ± 0.4 logMAR, which was a significant increase from the baseline (p < 0.0001). The patients who received intravenous rather than oral corticosteroids relapsed less (p = 0.026), with fewer relapses/patient/month of follow-up (p < 0.0001), and showed less frequent sunset glow fundus (33.3% versus 55%) and more relapse-free cases after induction therapy (p = 0.007). Delayed immunosuppressive therapy (median: 180 days from the onset of symptoms) reduced the rate of sunset glow fundus. The onset of sunset glow fundus was associated with a worse final visual acuity (p = 0.006). Conclusion: The long-term prognosis of VKH is quite good. Intravenous corticosteroids given at the onset of VKH are more effective than oral corticosteroids. Even if it is not given immediately after symptoms onset, immunosuppressive therapy is able to reduce the incidence of sunset glow fundus and to improve the final visual prognosis.

Background: Juvenile idiopathic arthritis (JIA) is a rheumatic condition of childhood that is frequently associated with anterior chronic uveitis. Evidence suggests that uveitis may persist up to adulthood in some cases, possibly causing severe visual impairment. Methods: We conducted a retrospective study on a series of patients aged 16 years or older with JIA-related active uveitis who were referred to the Uveitis Service of Sapienza University of Rome from 1990 to 2019 to evaluate the characteristics of ocular disease in patients with JIA-associated uveitis (JIA-U) who still exhibit uveitis in adulthood. Data on clinical features, treatment, complications and visual outcomes were collected. Results: Twenty adults (85% female; median age 23.4 ± 6.6 years, range 16−38 years) with ongoing uveitis (35 eyes) were identified. The median age at JIA onset was 6.15 ± 2.9 years (range 2−10), and uveitis onset was 8.7 ± 4.7 years (range 3−20). The patients were observed in a median follow-up of 16 ± 7.7 years (range 4−35). Fifty-seven percent of affected eyes (20 eyes) had good visual acuity (>0.4 logMAR), while eleven percent of affected eyes (4 eyes) were blind (≤20/200). Uveitis required topical steroids and mydriatic/cycloplegic in all cases. Orbital steroid injection was performed in 13 eyes. Systemic corticosteroids and biologic drugs were used in 14 patients. Conclusions: Although the visual prognosis of JIA-U has improved in recent years, persistent uveitis up to adulthood is still observed. Therefore, protracted follow-up of JIA-U patients is warranted because of the high burden of delayed visual complications.

Purpose: to study in patients with Behçet disease and ocular involvement (OBD) the presence of SARS-CoV-2 infection and its influence on the course of OBD., Methods: all the patients with OBD living in Lazio, one Italian region, and attending the Uveitis center of the Sapienza University of Rome were included in the study., Results: SARS-CoV-2 infection was found in 12.3% of 54 patients with OBD and in 3.84% of Lazio inhabitants (p  =  0.001, OR 3.51), and it was unrelated to the use of immunosuppressive drugs. COVID-19 symptoms in OBD patients were mild, with one patient only requiring hospitalization for interstitial pneumonia. None of the SARS-CoV-2 infected patients presented any uveitis relapses during the infection and in a subsequent median follow-up of 6 months., Conclusion: OBD seems to be a risk factor for developing SARS-CoV-2 infection. Usually this infection has a mild course and does not impact negatively on the course of uveitis.

Purpose : To assess the frequency of clinical signs in patients with viral acute anterior uveitis (AAU), and their ability to differentiate viral versus non-viral AAU. Methods : 168 patients with AAU, including 84 with presumed viral etiology, were evaluated. Sensitivity, specificity, area under the curve (AUC), positive and negative predictive value were calculated for each clinical sign. The model built with these parameters was tested on a validation group comprising 66 patients with AAU. Results : The most useful clinical signs were unilaterality (sensitivity: 98.8%, specificity: 57.1%), intraocular pressure (IOP) ≥24 mmHg (sensitivity: 68.7%, specificity: 91.7%), and the association between the two (sensitivity: 68.7%, specificity: 95.2%). In the validation group, the model built with these parameters presented AUC of 0.939. Adding iris atrophy AUC increased to 0.97. Considering these signs, it was possible to diagnose viral uveitis in 93.9% of the patients. Conclusion : Unilaterality, IOP≥24 mmHg and iris atrophy are significant predictors of possible viral etiology in AAU.

Background: This report describes the clinical course of choroidal neovascular membrane (CNV) in West Nile virus-associated chorioretinitis., Case Presentation: A 28-year-old Italian woman was referred to our institution because of reduced visual acuity in the left eye dating back 4 months. A diagnosis of retinal vasculitis in the right eye and chorioretinitis with CNV in the left eye was made. A complete workup for uveitis revealed positivity only for anti-West Nile virus immunoglobulin M (IgM), while immunoglobulin G (IgG) was negative. Whole-body computed tomography and nuclear magnetic resonance imaging of the brain were also negative. Therefore, the patient was treated with a combination of oral prednisone (starting dose 1 mg/kg per day) and three intravitreal injections of bevacizumab 1.25 mg/0.05 ml, 1 month apart. Fourteen days from starting corticosteroid therapy and after the first intravitreal injection, the patient experienced increased visual acuity to 0.4. Response to therapy was monitored by clinical examination, ocular coherence tomography (OCT), OCT angiography and retinal fluorescein angiography. Three months later, resolution of CNV in the left eye was achieved and no signs of retinal vasculitis were detected in the right eye, while serum IgM for West Nile virus turned negative and IgG positive., Conclusion: CNV may be a complication of West Nile virus-associated chorioretinitis, and only subclinical retinal vasculitis may also be found even in non-endemic regions.

Purpose: To provide recommendations for diagnosis of vitreoretinal lymphoma (VRL)., Methods: Literature was reviewed for reports supporting the diagnosis of VRL. A questionnaire (Delphi 1 round) was distributed to 28 participants. In the second round (Delphi 2), items of the questionnaire not reaching consensus (75% agreement) were discussed to finalize the recommendations., Results: Presenting symptoms include floaters and painless loss of vision, vitreous cells organized into sheets or clumps. Retinal lesions are usually multifocal creamy/white in the outer retina. Other findings include retinal lesions with "leopard-skin" appearance and retinal pigment epithelium atrophy. Severe vitreous infiltration without macular edema is the most likely presentation. Diagnostic vitrectomy should be performed. Systemic corticosteroid should be discontinued at least 2 weeks before surgery. An interleukin (IL)-10:IL-6 ratio > 1, positive mutation for the myeloid differentiation primary response 88 gene and monoclonality are indicators of VRL. Multi-modal imaging (optical coherence tomography, fundus autofluorescence) are recommended., Conclusions: A consensus meeting allowed the establishment of recommendations important for the diagnosis of VRL.

Purpose : To standardize a nomenclature system for defining clinical phenotypes, and outcome measures for reporting clinical and research data in patients with ocular tuberculosis (OTB). Methods : Uveitis experts initially administered and further deliberated the survey in an open meeting to determine and propose the preferred nomenclature for terms related to the OTB, terms describing the clinical phenotypes and treatment and reporting outcomes. Results : The group of experts reached a consensus on terming uveitis attributable to tuberculosis (TB) as tubercular uveitis. The working group introduced a SUN-compatible nomenclature that also defines disease "remission" and "cure", both of which are relevant for reporting treatment outcomes. Conclusion : A consensus nomenclature system has been adopted by a large group of international uveitis experts for OTB. The working group recommends the use of standardized nomenclature to prevent ambiguity in communication and to achieve the goal of spreading awareness of this blinding uveitis entity.

Purpose: To study macular microvascular changes in ocular Behçet disease (OBD) using optical coherence tomography angiography (OCTA)., Methods: Quantitative and qualitative analyses of OCTA were performed on 23 OBD patients with active or inactive uveitis and compared with healthy controls., Results: Deep capillary plexus (DCP) is the most frequently involved in OBD (p < 0.001). Its vessel density (VD) is reduced compared with controls in both active (p < 0.007) and inactive uveitis (p = 0.03). In inactive uveitis, VD is inversely related to the number of uveitis relapses (superficial capillary plexus: r = -0.694, p = 0.004; DCP: r = -0.541, p = 0.037) and it is significantly reduced in patients with a uveitis-free period ≥5 years compared with healthy controls (p < 0.038)., Conclusions: Macular VD is reduced in Behçet patients with active and inactive uveitis, especially in DCP. In inactive uveitis, VD is inversely related to the number of ocular relapses and cannot be restored during time.

Purpose: To describe the epidemiology of macular edema (ME) in patients with uveitis., Methods: Review of articles listed on PubMed pertaining to uveitic ME., Results: Reported rates of uveitic ME ranging from 20% to 70%, depending on the ancillary tests used (fundus examination, fluorescein angiography, optical coherence tomography). Macular edema might develop due to uveitis itself, or occur as an adverse effect of drugs taken for different diseases. It is more frequently observed in adults than in children, in chronic uveitis, and in intermediate uveitis. Males with ankylosing spondylitis are more prone to develop ME than females. Three patterns of uveitic ME are observed, either isolated or in combination: cystoid ME, the most frequently encountered pattern seen in up to 80% of cases, diffuse ME and serous retinal detachment., Conclusion: Older age, chronicity of uveitis and intermediate uveitis are risk factors for the development of ME in patients with uveitis.

Purpose : To study epidemiology and clinical findings of cataract in HIV+ patients. Methods : A total of 32 HIV+ patients, 11 with uveitis/retinitis before surgery and 21 without, mean follow-up 44.9 ± 36.6 months, and 114 HIV- patients, 57 with uveitis/retinitis before surgery and 57 without, were retrospectively compared. Results : Visual acuity improved in all HIV+ patients ( p  < 0.001), who were younger ( p  = 0.01) and more frequently males ( p  = 0.027). HIV+ patients with uveitis prior surgery improved less ( p  = 0.046) than HIV- ( p  < 0.001); their anterior chamber inflammation was similar to baseline. Male sex ( p  = 0.005), younger age ( p  < 0.001), dyslipidaemia ( p  = 0.058), HBV+ ( p  = 0.037), and unilateral cataract ( p  = 0.001) were more frequent in HIV+ patients with senile cataract, but they showed the same postoperative course as HIV- patients. Conclusion : Cataract surgery in HIV+ patients is safe and effective. Uveitis prior to surgery did not significantly affect the postoperative course. Systemic comorbidities are more frequent in HIV+ patients with senile cataract than in HIV- subjects.

Ocular involvement is a common manifestation of inflammatory rheumatic diseases, often requiring a multidisciplinary collaboration between rheumatologists and ophthalmologists. The aim of this study was to standardize "red flags" for referral for rheumatologists and ophthalmologists using a Delphi consensus for the management of rheumatic diseases with ocular involvement. The scientific board comprised 11 Italian hospital-based rheumatologists (N = 6) and ophthalmologists (N = 5). A systematic review identified potential red flags for referral. The panel developed 19 statements consisting of (a) referral from ophthalmologist to rheumatologist (b) referral from rheumatologist to ophthalmologist and (c) overarching principles relating to multidisciplinary roles/goals and management. Voting was performed anonymously using an online Delphi method. Each participant expressed a level of agreement on each statement using a 5-point scale (1="strongly disagree"; 5="strongly agree"). Total cumulative agreement was defined as the sum of the percentage of response to items 4 ("agree") and 5 ("absolutely agree"), consensus defined as ≥ 80% cumulative agreement for each statement. Positive consensus among 11 participants was reached for 15/19 (78.9%) statements. Statements not reaching consensus were discussed in a face-to-face meeting prior to the second vote (10 participants). Positive consensus was reached for all 19 statements, with final total cumulative agreement of 90-100%. This is the first Delphi consensus undertaken to standardize red flags for referral to rheumatologists and ophthalmologists for patients with rheumatic diseases and ocular involvement.

Ocular tuberculosis remains a presumptive clinical diagnosis, as the gold standard tests for diagnosing ocular tuberculosis are often not useful: Mycobacterium tuberculosis cultures require weeks to process on Lowenstein-Jenson media and have low yield from ocular samples; while acid-fast bacilli smears or polymerase chain reaction detection of M. tuberculosis DNA have low sensitivities. Thus, diagnosis is often based on suggestive clinical signs, which are supported by positive investigations: tuberculin skin test or interferon-gamma release assays; chest X-ray findings suggestive of pulmonary tuberculosis, and/or evidence of associated systemic tuberculosis infections in the absence of other underlying disease. The aim of this review is to provide an update on the methods of diagnosing ocular tuberculosis, and discuss the challenges of its diagnosis. We also suggest a step-ladder approach to a more accurate diagnosis of ocular tuberculosis by combining the available diagnostic tests.

Purpose: To investigate demographic, clinical features and complications of Italian patients with ocular Behçet's disease (BD) over time., Methods: Retrospective study of 385 patients examined from 1968 to 2011, 265 of whom had follow-up ≥ 12 months., Results: A significant increase in the proportion of females (p = 0.03) and anterior uveitis (p < 0.001), and a decrease in the complete type of BD in all patients (p = 0.003) and in males (p = 0.002), and in hypopyon (p = 0.024) were observed over time. Optic neuropathy (p < 0.0001), maculopathy (p = 0.002), retinal detachment, and retinal neovascularization (p = 0.02) decreased over time, with no difference between genders, concomitant with an increase in the use of immunosuppressive drugs (p = 0.003). Visual acuity ≤1/10 was detected more often in males than females (p = 0.003)., Conclusions: A significant shift in clinical manifestations of BD patients was observed over 44 years. Immunosuppressive therapy succeeded in lowering ocular complications. The incidence of ocular BD is increasing in females, but visual prognosis is still worse in males.

Purpose: To report on clinical features and outcome of severe bacterial keratouveitis., Methods: Twenty patients with severe bacterial keratouveitis treated with topical tobramycin and levofloxacin and oral levofloxacin were included. Main outcome measures were ulcers location, bacterial isolates, risk factors, visual prognosis., Results: Centrally located ulcer/abscess was present in 65% of patients. Contact lens (CL) wear was the most common risk factor (70%). Bacterial isolates were observed in 58% of patients, none resistant to tobramycin and levofloxacin. Pseudomonas aeruginosa was found in 47% of positive cases and in 64% of CL wearers. After therapy, the mean visual acuity improved significantly (p < 0.0001), particularly in contact lens wearers (p = 0.04) and in patients younger than 60 years old (p < 0.001)., Conclusions: Pseudomonas aeruginosa is the most frequent cause of bacterial keratouveitis and CL wear the most common risk factor. Topical tobramycin and levofloxacin and oral levofloxacin are effective in the treatment of bacterial keratouveitis.

Purpose . To study epidemiology, clinical findings and visual prognosis of patients with Fuchs' Heterochromic Iridocyclitis (FHI). Methods . A retrospective analysis was performed on 158 patients with FHI. Thirty-five patients were observed only once; the remaining 123 had a mean follow-up of 30.7 months (50 of them had a mean follow-up of 63.5 months) and in those we assessed complications, medical and surgical treatment, and long-term visual prognosis. Results . Average age at uveitis diagnosis was 27.2 years and 18.3% of patients were children. Blurred vision (54.5%) and floaters (40.5%) were the most frequent presenting symptoms. Small to medium-sized keratic precipitates (95.6%), iris atrophy (86.8%), and vitreous opacities (91.2%) were the most common signs; the prevalence of cataract and IOP increase was 63.5% and 20.1%, respectively, and their incidence was 0.1 and 0.06 eye/year. Significant risk factor for visual loss was IOP increase at presentation ( p = 0.02). At final examination 98% of the eye had a visual acuity ≥ 0.6, and topical ( p < 0.001) and systemic ( p < 0.001) corticosteroids therapy were used less frequently than before referral. Conclusions . FHI has a good visual prognosis, despite the significant incidence of cataract and glaucoma. A correct and prompt diagnosis might avoid unnecessary therapies and provide excellent visual outcomes.

Objectives: To report on the clinical course of ocular and extraocular involvement in a multidrug-resistant patient with Behçet's disease (BD)., Methods: A 22-year-old male with BD (bilateral panuveitis and retinal vasculitis, oral ulcers, erythema nodosum, arthralgia, epididymitis) was followed-up from 1999 to 2014. He was treated continuously with corticosteroids in combination with different immunosuppressive therapies (cyclosporine, azathioprine, methotrexate, interferon, infliximab, mycophenolate), which exerted numerous side-effects such as nephrotoxicity, nephrolithiasis, increase of liver enzymes, severe depression with suicidal ideation, severe leucopenia, pulmonary tuberculosis, pulmonary legionellosis, recurrent bronchopneumonia., Results: Despite immunosuppressive and corticosteroid therapies, the patient showed multiple relapses of uveitis and systemic BD lesions and developed severe osteoporosis with multiple vertebral fractures, bilateral cataracts and steroid-associated glaucoma until 2007. Since then he has been treated with prednisone alone, currently at low dosage, remaining free from uveitis and systemic symptoms. His final visual acuity is 9/10 in the right eye and counting fingers in the left one., Conclusions: BD patients are usually responsive to immunosuppressive drugs. The possibility of a multi-drug resistance as well as of multiple drug-related side effects cannot be disregarded and continuous therapy should be given in order to preserve a useful visual acuity until the disease, either spontaneously or drug-induced, runs into remission.

Purpose: To report on Vogt-Koyanagi-Harada disease that occurred after stapedotomy., Methods: Case report., Results: A 46-year-old woman developed bilateral choroiditis, papillitis, and serous retinal detachment 10 days after an uneventful surgical stapedotomy. The diagnosis was confirmed on the basis of the clinical features, fundus fluorescein angiography and optical coherence tomography findings, and a clinical course with subsequent involvement of the anterior segment, uveitis relapses with serous retinal detachment, and negative laboratory work-up for uveitis., Conclusions: Vogt-Koyanagi-Harada disease may appear after an uneventful surgical intervention of stapedotomy, suggesting that surgical trauma in the inner ear, a melanocyte-containing organ, may induce an inflammatory response within the eye.

Behçet disease (BD) is a multisystem inflammatory disorder that is an important cause of morbidity worldwide. BD is most common along the ancient "Silk Road" route in the Far East and Mediterranean basin. The eye is the most commonly involved organ in BD patients.The prototypical form of involvement is a relapsing remitting panuveitis and retinal vasculitis. Less commonly, BD may present in the form of conjunctivitis, conjunctival ulcers, keratitis, episcleritis, scleritis, and extraocular muscle paralysis. Uveitis in BD carries significant implications for the patient, because it is a chronic recurrent disease characterized by explosive attacks of severe inflammation that may cause significant, cumulative damage to the intraocular structures. This review summarizes the epidemiology of systemic and ocular clinical features of BD with particular focus on risk factors, clinical characteristics, complications, and prognosis of BD-associated uveitis.

Aim: To assess the morphological characteristics of uveitic macular edema studied with Spectralis optical coherence tomography (OCT) and to investigate the correlation between the tomographic features and visual acuity., Methods: 71 eyes of 55 patients underwent examination with Spectralis OCT (Heidelberg Engineering, Germany). Data was correlated with logMAR best-corrected visual acuity (BCVA)., Results: Two morphological patterns were observed: cystoid macular edema (CME) in 69% and diffuse macular edema in 31% of eyes. BCVA was 0.2 in CME, 0.1 in diffuse edema (p = 0.008). Foveal thickness was 413.4 ± 212 µm in CME, 311.27 ± 53 µm in diffuse edema (p = 0.03). BVCA was 0.3 in eyes with serous retinal detachment (SRD), 0.2 in eyes without SRD (p = 0.02). BCVA was 0.4 in eyes with inner segment/outer segment (IS/OS) disruption, 0.1 in eyes with integrity of the IS/OS junction (p = 0.01)., Conclusions: BCVA is negatively correlated with cystoid pattern, foveal thickening and SRD. Disruption of the IS/OS junction is associated with poor vision in uveitic macular edema., (Copyright © 2012 S. Karger AG, Basel.)

Purpose: The aim of the present study is to evaluate the roles of TGFs β1 and β2, glial cell line-derived neurotrophic factor (GDNF), and nerve growth factor (NGF) in the pathogenesis of idiopathic epiretinal membrane (ERM)., Methods: Eight patients, six males and two females, with an average age of 60.25 ± 17.16 years (range, 33-75 years) who were affected by idiopathic ERM were enrolled in the study. All patients underwent standard pars plana vitrectomy surgery with membrane removal and specific ELISA was performed to evaluate TGFβ1, TGFβ2, GDNF, and NGF in the vitreous samples. This was repeated after acidification of the samples with hydrochloric acid., Results: Before acidification, ELISA analysis revealed a significant increase of TGFβ2 in the samples with idiopathic ERM (327.98 ± 99.58 pg/mL; range, 206.864-466.235 pg/mL) compared to the control group (187.17 ± 58.20 pg/mL; range, 132.758-271.707 pg/mL; t = 3.4; P < 0.05). A statistically significant difference was also obtained after acidification of the samples (618.15 ± 201.43 pg/mL; range, 409.795-866.215 pg/mL compared to 265.04 ± 98.15 pg/mL; range, 152.478-352.101 pg/mL; t = 4.5; P < 0.05). Notably, before acidification the differences in NGF between the two groups were not statistically significant (t = 0.79; P = 0.46), while after acidification a significant increase of the NGF levels in ERM samples was found in comparison with the control group (723.41 ± 235.4 vs. 242.84 ± 104.61; t = 3; P < 0.05)., Conclusions: The present study reveals that TGFβ2 and NGF are associated with idiopathic ERMs, suggesting a novel compensatory mechanism so far never proposed.

We report a case of a 53-year-old man with a 2-year history of progressive gait and balance disturbance, supranuclear ophthalmoparesis, mild dysarthria and dysmetria. EMG revealed a lower limb axonal sensory-motor neuropathy, while MR imaging demonstrated a small focal lesion in the right frontal lobe, mild diffuse hyperintensity of the periventricular white matter and diffuse brain atrophy. Magnetic resonance spectroscopy revealed a mild decrease in N-acetyl-aspartate peak and an increase in the choline peak in the small right frontal lesion and within 6 voxels of interest in normal appearing cerebral tissue. According to the clinical picture the diagnosis of WD was made by the positivity of PCR for T. whipplei DNA on CSF. After treatment the patient showed a mild clinical improvement although MR images and laboratory test remained unchanged. The MRS findings suggest that the pathological process of the disease diffusely involves the brain. Despite the absence of gastrointestinal involvement WD should be suspected in all complex and atypical neurological pictures, even in presence of peripheral involvement, in order to be able to start treatment promptly., (Copyright 2010 Elsevier B.V. All rights reserved.)

Purpose: To report clinical features and prognosis of HLA B27 acute anterior uveitis., Methods: 165 patients with recurrent acute anterior uveitis were divided into two groups: HLA B27-positive (group 1, 60 patients) and HLA B27-negative (group 2, 105 patients). A comparison between the two groups was performed., Results: Unilateral involvement was higher in group 1 (p = .046), and more simultaneous bilateral cases occurred in group 2 (p = .004). Group 1 was more correlated to systemic diseases than group 2 (50 versus 17.1%, p < .001), particularly to ankylosing spondylitis (35 versus 3.8%, p < .001). Uveitis was diagnosed before spondylitis in 57% of patients. In groups 1 and 2 an immunosuppressive therapy was administered to 20 and 9.5% of patients but was exclusively given to control uveitis in 1.6 and in 2.8% of cases, respectively., Conclusions: A strong association between HLA B27 uveitis and ankylosing spondylitis was confirmed and usually uveitis preceded rheumatological involvement.

Purpose: To determine the frequency of occurrence of limited clinical features which distinguish patients with Vogt-Koyanagi-Harada (VKH) disease from those with non-VKH uveitis., Design: Comparative case series., Participants: We included 1147 patients., Methods: All patients with bilateral ocular inflammatory disease presenting to any of 10 uveitis centers in the 3-month period between January 1 and March 31, 2006 (inclusive), were asked to participate. The clinical and historical features of disease were obtained from the participants via direct interview and chart review. Patients were stratified based on whether they were diagnosed with VKH disease or non-VKH uveitis for statistical analysis., Main Outcome Measures: Presence or absence of various clinical features in the 2 populations., Results: Of 1147 patients, 180 were diagnosed with VKH disease and 967 with non-VKH uveitis. Hispanics and Asians were more likely to be diagnosed with VKH than non-VKH disease compared with other ethnicities. In acute disease, the finding of exudative retinal detachment was most likely to be found in VKH disease with a positive predictive value (PPV) of 100 and negative predictive value (NPV) of 88.4, whereas in chronic disease, sunset glow fundus was most likely to be found, with a PPV of 94.5 and NPV of 89.2., Conclusions: Numerous clinical findings have been described in the past as important in the diagnosis of VKH. The current study reveals that of these, 2 are highly specific to this entity in an ethnically and geographically diverse group of patients with nontraumatic bilateral uveitis. These clinical findings are exudative retinal detachment during acute disease and sunset glow fundus during the chronic phase of the disease., (Copyright 2010 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To evaluate the clinical features of ocular toxoplasmosis (OT)., Methods: Retrospective review of 3080 new patients with uveitis was performed to identify patients with active OT. The main outcome measures were ocular features and ocular complications., Results: Active OT was diagnosed in 88 patients (2.85% of all uveitis), 39 male (44.3%) and 49 female (55.7%), at an average age of 20.4+/-14.6 years, lower than in other types of uveitis (p<0.0001). Acquired OT diagnosis was possible in 4 cases (4.5%), while in the others a differentiation between acquired or congenital OT was impossible. Unilateral OT occurred in 76 patients (86.36%), with lesions located inside the vascular arcade in 64 of them (84.2%). Among bilateral cases (12 patients, 13.63%), at least one lesion was central in 9 (75%). Isolated peripheral lesions were observed in 12 out of 88 patients (13.6%). During an average follow-up of 70+/-73.5 months, OT recurrences appeared in 70 patients (79.54%). Mean interval between diagnosis and first relapse was 43.57+/-48.46 months, while it shortened between subsequent relapses (24.44+/-26.5 months; p=0.001)., Conclusions: Ocular toxoplasmosis is a highly recurrent disease, mainly unilateral, with an average age at onset lower than those observed in other types of uveitis. Time between relapses shortens significantly over time.

Purpose: To study use of optical coherence tomography (OCT) for macular edema in uveitis., Methods: In total, 44 patients with uveitis and macular edema underwent OCT. Data obtained were correlated with anatomic type, duration of uveitis, and visual acuity (VA)., Results: Both cystoid macular edema (CME) and diffuse macular edema (DME) correlate negatively with VA, although statistically CME showed stronger correlation (p = .02). Also CME had higher mean foveal thickness than DME (p < .01). Negative correlation between foveal thickness and VA (p < .05) was observed. CME correlates with uveitis duration (p < .01)., Conclusions: CME and macular thickening correlate with VA reduction and uveitis duration.

Purpose: To assess the efficacy and safety of infliximab in the treatment of sight-threatening uveitis and extraocular manifestations in patients with Behçet's disease., Methods: Twelve patients with Behçet's disease and uveitis were treated with infliximab after unsuccessful therapy with other immunosuppressive drugs. The main outcome measures were as follows: the number of uveitis relapses, the number of Behçet's disease-related extraocular lesions, and the amount of corticosteroids administered during the treatment as well as during an equal prior period of time while the patients were on other immunosuppressive agents. Visual acuity was recorded at the beginning of infliximab therapy and at the end of follow-up, and was defined as stable if it did not change from baseline, increased if it showed at least one line of improvement from baseline, and decreased if it showed at least a one line decrease from baseline., Results: During an average follow-up of 16.67 +/- 7.63 months (median, 15 months), 11 patients (91.6%) showed a reduction in the number of ocular relapses (relapse/month, from 0.35 +/- 0.17 to 0.12 +/- 0.17, P < 0.001). All of the patients (n = 11) who were taking corticosteroids before infliximab were able to reduce the amount of corticosteroids taken daily during infliximab treatment (from 24.33 +/- 10.84 mg/prednisone per day to 8.97 +/- 6.81 mg/prednisone per day, P < 0.001), and all presented with a reduced onset of extraocular manifestations of Behçet's disease (mean total number, from 2.83 +/- 3.61 to 1.51 +/- 2.35, P = 0.039). One patient, who had to stop treatment 2 months after starting because of the onset of pulmonary tuberculosis, showed the same number of relapses during infliximab treatment but was able to reduce the mean daily corticosteroid dose. Visual acuity increased by one or more lines in three eyes (12.5%) and remained unchanged in 87.5% of the eyes. Infliximab-related side effects appeared in four patients (33.3%)., Conclusions: Infliximab was effective in the treatment of uveitis in these Behçet's disease patients, significantly reducing the number of ocular relapses and making possible a significant reduction in the daily dose of corticosteroids administered. Extraocular manifestations of Behçet's disease were also controlled by infliximab. Nevertheless, side effects were not uncommon, and an extensive study of systemic conditions before infliximab administration had to be carried out to exclude systemic infection, particularly prior tuberculosis., (Copyright (c) Japanese Ophthalmological Society 2007.)

Purpose: To compare the effect on outcomes of the route of administration of corticosteroids in acute Vogt-Koyanagi-Harada disease., Design: Retrospective comparative interventional case series., Settings: Nine international uveitis specialty clinics., Study Population: Forty-eight patients presenting over a three-year period to a study center with acute Vogt-Koyanagi-Harada disease., Intervention: Initial treatment with corticosteroid either orally (Oral only group) or intravenously followed by an oral taper (IV+Oral group)., Main Outcome Measures: Change in visual acuity with treatment; development of ocular complications, including visually significant cataract, choroidal neovascularization, subretinal fibrosis, fundus pigment migration, nummular hypopigmented lesions, and diffuse fundus depigmentation; use of immunosuppressive therapy., Results: The Oral only group comprised 15 patients (31%) and the IV+Oral group 33 patients (69%). Median follow-up was 15 months. There was no difference in duration of follow-up between groups (P = .234). There was no difference in the change in visual acuity between groups, adjusting for initial visual acuity (P = .402). There were no differences in the rates of development of visually significant cataract, fundus pigmentary changes, or in the rate of use of subsequent immunosuppressive therapy between treatment groups. No patients developed choroidal neovascularization or subretinal fibrosis over the study period., Conclusions: Route of administration of corticosteroid had no detectable effect on change in visual acuity nor on the development of visually significant complications over the study period. Prospective trials are necessary to address speed of resolution and definitively answer outcome questions.

Purpose: To present and evaluate research criteria for the diagnosis of birdshot chorioretinopathy., Design: Consensus statement., Methods: Investigators who attended an international conference on birdshot chorioretinopathy drafted diagnostic criteria, which were refined after comment by conference attendees. Sensitivity and specificity of the criteria were evaluated by review of 82 previously diagnosed cases and evaluation of 80 consecutive patients with other forms of uveitis., Results: Criteria, including presence of "birdshot lesions" and mild intraocular inflammation in both eyes, are based on clinical findings in the absence of systemic disease; HLA-A29 is supportive of diagnosis, but not required. Diagnostic criteria were found to be 100% specific and 97.5% sensitive. Positive predictive value was 100%; negative predictive value was 97.6%., Conclusions: Standardized criteria for the diagnosis of birdshot chorioretinopathy will facilitate clinical research and the comparison of results from studies performed at different institutions by ensuring a homogeneous population of study patients.

Background: Turner's syndrome is a chromosomal abnormality where phenotypic females have either a missing X chromosome or a structural aberration of the X chromosome. Several ocular diseases have been associated with Turner's syndrome in the past, including one case only of proven iridocyclitis., Methods: In this study we report the clinical findings of three females with Turner's syndrome and uveitis followed up for a mean period of 12.6 months. Two were observed in childhood or adolescence (10 and 16 years old)., Results: All the patients showed iridocyclitis, in one case complicated by the onset of papilledema and cystoid macular edema. Associated systemic diseases (psoriasis and juvenile seronegative arthritis) were diagnosed in two cases. The third patient showed positive antinuclear antibody and HLA-DR11, without any clinical or radiologic signs of arthritis. The iridocyclitis tended to become chronic with time, and this may be partially due to the endocrinologic contraindications to the use of systemic steroids. Nevertheless, the final visual acuity was > or =8/10 in all three cases., Conclusions: Iridocyclitis should be included in the list of ocular manifestations in Turner's syndrome. It may tend to become chronic and may be found especially in those patients presenting other associated autoimmune systemic disease.