Clinical manifestations and immune markers of non-HIV-related CMV retinitis.

BACKGROUND: Since the HIV epidemic in the 1980s, CMV retinitis has been mainly reported in this context. CMV retinitis in persons living with HIV is usually observed when CD4 + cells are below 50 cells/mm3. This study aims to describe the immune markers of non-HIV-related CMV retinitis as well as to describe its clinical manifestations and outcomes. METHODS: Retrospective chart review of consecutive patients with CMV retinitis not related to HIV seen at the uveitis clinic of Jules Gonin Eye Hospital between 2000 and 2023. We reported the clinical manifestations and outcomes of the patients. We additionally assessed immune markers during CMV retinitis (leukocyte, lymphocyte, CD4 + cell and CD8 + cell counts as well as immunoglobulin levels). RESULTS: Fifteen patients (22 eyes) were included. Underlying disease was hematologic malignancy in 9 patients, solid organ transplant in 3 patients, rheumatic disease in 2 patients and thymoma in one patient. The median time between the onset of underlying disease and the diagnosis of retinitis was 4.8 years. Lymphopenia was observed in 8/15 patients (mild = 3, moderate = 4, severe = 1), and low CD4 counts were observed in 9/12 patients, with less than 100 cells/mm3 in 4 patients. Hypogammaglobulinemia was detected in 7/11 patients. Retinitis was bilateral in 7/15 patients, and severe visual loss was frequent (5/19 eyes). Disease recurrence was seen in 7/13 patients at a median time of 6 months after initial diagnosis. No differences in immune markers were observed in patients with vs. without recurrence. CONCLUSION: CMV retinitis is a rare disorder that can affect patients suffering any kind of immunodeficiency. It is associated with a high visual morbidity despite adequate treatment. CD4 + cell counts are usually higher than those in HIV patients, but B-cell dysfunction is common.

Eyes Wide Shut: A Cohort Study Questioning the Role of Fundoscopy in Infective Endocarditis Diagnosis.

In this retrospective/prospective study, we assessed the role of fundoscopy in 711 episodes with suspected infective endocarditis (IE); 238 (33%) had IE. Ocular embolic events (retinal emboli or chorioretinitis/endophthalmitis) and Roth spots were found in 37 (5%) and 34 (5%) episodes, respectively, but had no impact on IE diagnosis.

Efficacy of TNF-Alpha Inhibitors to Control Inflammation and Prevent Secondary Complications in Non-Infectious Uveitis: A Real-Life Experience from Switzerland.

PURPOSE: To evaluate the efficacy of systemic tumor necrosis factor-alpha inhibitors (TNFi) in the treatment of non-infectious uveitis (NIU). METHODS: This Swiss multicenter retrospective cohort study included patients with NIU requiring TNFi during the period from 2001 to 2018. Risk factors for the occurrence of new complications were identified using Cox regression analysis and hazard ratios (HR). RESULTS: Seventy-one patients (126 eyes; mean age 40.6 ± 14.4 years, mean duration of uveitis 46.0 ± 61.8 months) were followed for 40.2 ± 17.3 months after addition of TNFi. Under TNFi, visual acuity improved from 0.2 ± 0.3 to 0.1 ± 0.3 logMAR (p < 0.001). The portion of patients under systemic corticosteroids decreased from 81.7% to 25.4% (p < 0.001), while that for conventional synthetic disease-modifying anti-rheumatic drugs insignificantly decreased from 63.4% to 50.7% (p > 0.05). In 80.2% of eyes, complications were present at baseline with epiretinal gliosis (39.7%), cataract (41.3%) and macular edema (ME; 27.8%) being the most common. New complications under TNFi were encountered in 49.2% of eyes, also including recurrence (5 eyes) or new onset of ME (14 eyes). The need for switching of TNFi was associated with further complications (HR 3.78, p = 0.012). CONCLUSION: Although the efficacy and tolerability of TNFi in a real-life setting are favorable, treatment is often initiated late, i.e., after many eyes have already developed complications. Even with TNFi, new complications, particularly ME, cannot be completely avoided. Further research is needed to assess the impact of earlier initiation of TNFi therapy.

Long-term Efficacy of TNF-alpha Inhibitors on Persistent Uveitic Macular Edema: A Swiss Multicenter Cohort Study.

PURPOSE: To assess the efficacy of tumor necrosis factor-alpha inhibitors (TNFi) on uveitic macular edema (ME) unresponsive to conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs). METHODS: This multicenter retrospective study included patients with uveitic ME persisting despite csDMARDs. The effect of an additional TNFi on central retinal thickness (CRT), best corrected visual acuity (BCVA) and corticosteroid need was evaluated. RESULTS: Thirty-five eyes (26 patients, mean age 42.9 ± 15.2 years) were included. CRT decreased from 425 ± 137 µm to 294 ± 66 µm (p < .001) and 280 ± 48 µm (p < .001) at 1 and 4 years of follow-up, respectively. BCVA improved from 0.28 ± 0.22 to 0.21 ± 0.48 (1 year, p = .013) and 0.08 ± 0.13 logMAR (4 years, p = .002). The proportion of patients requiring systemic corticosteroids decreased from 88.5% to 34.8% (1 year) and 15.4% (4 years). CONCLUSION: The addition of a TNFi resulted in an improvement of CRT and BCVA for up to 4 years in uveitic ME but rescue treatments were needed for some patients.

Susac Syndrome: A Case Series.

BACKGROUND: Susac syndrome (SS) is an autoimmune disorder that involves the eyes, the brain, and the ears. It is a rare cause of recurrent branch retinal artery occlusion. The purpose of this study was to report cases of SS, highlighting the clinical presentations, therapeutic options, and their outcome. PATIENTS AND METHODS: Retrospective case series of patients seen at our institution for SS between 2005 and 2020. Demographics, clinical characteristics, treatment, and outcome were studied. RESULTS: Four patients (3 females, mean age 29 years old) were included in the study. According to the recently revised diagnostic criteria, three patients had definite and one patient had probable SS (distinctive ophthalmological and brain involvement without ear involvement). Initial visual acuity (VA) was normal in all eyes, but two patients had unilateral visual field impairment. Gass plaques (defined as yellow-white plaques found in the arteriolar wall away from arterial bifurcations) were observed on fundus examination in all patients. Fluorescein angiography revealed arteriolar wall hyperfluorescence and branch retinal arterial occlusions (BRAOs) in the absence of other signs of intraocular inflammation in all patients. Initial treatment consisted of a high-dose corticosteroid (intravenous or oral) with additional immunosuppressive therapy (azathioprine, intravenous immunoglobulins, mycophenolate mofetil, and/or cyclophosphamide). Residual symptoms were present in all patients and included scotoma (n = 2) and hearing loss (n = 3). CONCLUSION: SS is a rare disease with characteristic ophthalmological manifestation. The majority of patients present a crude form of the triad, and retinal findings may be the first initial manifestation. Ophthalmologists should consider the possibility of an SS in all young patients presenting with BRAOs.

The Clinical Spectrum and Outcome of Uveomeningitis: A Comprehensive Analysis of 110 Cases.

PURPOSE: Uveitis can be associated with meningitis (uveomeningitis) and the inflammation shared with the central nervous system. We aimed to describe the characteristics and outcome of uveomeningitis. METHODS: We retrospectively analyzed 110 consecutive adult patients with uveomeningitis. RESULTS: The main causes of uveomeningitis were Vogt-Koyanagi-Harada (31%), syphilis (16%), sarcoidosis (12%), Behçet's disease (7%), and multiple sclerosis (5%). Sixteen percent of uveomeningitis remained of undetermined origin. Compared to etiology-matched uveitis without meningitis, patients with uveomeningitis were younger, had more frequent neurological manifestations, and had more frequent abnormal cerebral magnetic resonance imaging findings. In contrast, no ocular feature upon examination was significantly associated with the presence of meningitis. Patients with uveomeningitis were more frequently treated with immunosuppressants but uveitis relapse and systemic complications did not differ between groups. CONCLUSION: Uveomeningitis is associated with a limited spectrum of diseases. Meningitis does not seem to impact ocular and extraocular outcomes. Therefore, lumbar puncture should be performed on an individual basis during the diagnostic workup of uveitis.

Acute retinal necrosis: A mini review.

Acute retinal necrosis is a rare but potentially devastating disease. Even in the era of modern medicine, retinal detachment is a frequent complication leading to vison loss, as well as phthisis bulbi. Whereas IV acyclovir still remains the standard of care, high doses of valacyclovir with/without additional intravitreal injections of foscarnet have been used. In an attempt to reduce the retinal detachment rate, prophylactic laser treatment and early vitrectomy have been proposed. In this article, we aim to review current diagnostic and treatment modalities.

Tick-borne encephalitis related uveitis: a case report.

BACKGROUND: Tick-borne encephalitis (TBE) is an infectious disease of the central nervous system caused by the TBE virus (TBEV), which is usually transmitted by a tick-bite, with increasing incidence in northeastern Europe and eastern Asia during the past decade. Ocular involvement has not been described in the literature to date. CASE PRESENTATION: A 58-year-old patient presented to the emergency department with occipital headaches and poor balance for 5 days. He reported a tick-bite 6 weeks before without erythema migrans followed by a flu-like syndrome. Serological testing was negative for Borreliosis and TBEV. At presentation, he was febrile with neck stiffness and signs of ataxia. Three days later, he presented unilateral visual loss in his right eye. Examination revealed non granulomatous anterior uveitis, vitreous inflammation, and retinal haemorrhages at the posterior pole without macular oedema or papillitis. Polymerase chain reaction (PCR) of the cerebrospinal fluid returned negative for all Herpes family viruses. No clinical evidence of other infection nor malignancy was identified. A seroconversion of the TBEV- immunoglobulin titres was observed 2 weeks later while the serum antibodies for Borrelia were still not detected. Magnetic resonance imaging was unremarkable. We concluded to the diagnosis of TBE-related uveitis. Under supportive treatment, there was complete resolution of the neurological symptoms and the intraocular inflammation without sequelae within the following weeks. CONCLUSIONS: We describe a new association of TBEV with uveitis. In view of the growing number of TBE cases and the potential severity of the disease we aim at heightening awareness to achieve prompt recognition, prevention, and treatment.

Epidemiology of Childhood Uveitis in a Tertiary Care Center: A 20-Year Study.

PURPOSE: To investigate demographics and causes of pediatric uveitis in a Swiss tertiary reference center over a 20-year period. MATERIALS AND METHODS: Retrospective cohort study on patients with uveitis aged less than 16 years seen at Jules-Gonin Eye Hospital between 1 January 2000 and 31 December 2019. RESULTS: Out of 2846 patients with uveitis seen in the Jules-Gonin Eye Hospital Ocular Immune-Infectiology Department, 317 (11.1%) were under 16 years of age and were included in this study. Median age at onset of the uveitis was 8.9 years (range 0 - 16). Anterior uveitis was the most frequent presentation (45.1%) followed by posterior uveitis in 26.2%, intermediate uveitis in 23.3%, and panuveitis in 5.4%. The inflammation was most frequently bilateral and non-granulomatous. A systemic inflammatory disease was found in 34% of the cases and an infectious cause in 24%. CONCLUSION: The repartition of the location of the uveitis was similar to previous reports from Western countries. Uveitis in juvenile idiopathic arthritis is the most frequent etiology related to a systemic disease in children. An infectious cause was found in 24% of our patients, which is a greater proportion than in adult cohorts.

[Ocular manifestations of Covid-19]. / Ophtalmologie - Manifestations du Covid-19.

The most frequent ocular manifestation of the SARS-CoV-2 is a conjunctivitis. It is found in 1-3% of patients and has unusually a benign course. In those patients but also in patients without ocular involvement, the virus can be detected by PCR in conjunctival swabs or in the tears. Whereas the presence of the virus in the conjunctiva is proven, its transmission through the conjunctiva is still discussed. Despite the poor level of evidence, the use of protective eyewear is recommended. Ocular thromboembolic events have been described in Covid-19. They can be found in the context of the Covid-related coagulopathy. A multidisciplinary approach should be provided in these cases. In the ICU, severe ocular complications might be indirectly related to Covid-19 in ventilated patients.

La manifestation oculaire la plus fréquente du SARS-CoV-2 est une conjonctivite. Elle est retrouvée dans 1 à 3 % des cas et est généralement d'évolution bénigne. Dans le cas de conjonctivite mais aussi chez les patients asymptomatiques au niveau oculaire, le virus peut être retrouvé par PCR dans les larmes/le frottis conjonctival. Une transmission par la conjonctive reste toutefois incertaine. Par mesure de précaution, le port de lunettes de protection est recommandé. Les atteintes ophtalmologiques sévères de type thromboembolique peuvent être retrouvées dans le cadre de la coagulopathie liée au Covid-19. Dans ces cas, une prise en charge pluridisciplinaire est nécessaire. Des événements oculaires graves peuvent également être indirectement liés au Covid-19 chez les patients sous ventilation aux soins intensifs.