Therapeutic window of opportunity in the acute uveitic phase of Vogt-Koyanagi-Harada disease: Prevention of late autoimmune complications by early intervention.

Purpose: To determine relationship between timing of treatment initiation and disease outcomes and whether a therapeutic window of opportunity exists in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease.

Methods: Retrospective analysis of 112 patients (224 eyes). Main outcome measures were final visual acuity, progression to chronic recurrent evolution, development of complications, particularly 'sunset glow fundus', and drug-free remission cure of uveitis.

Multiple evanescent white dot syndrome (MEWDS): update on practical appraisal, diagnosis and clinicopathology; a review and an alternative comprehensive perspective.

Background: Multiple evanescent white dot syndrome (MEWDS) is a rare inflammatory eye condition affecting the outer retina as a consequence of choriocapillaris non perfusion. The pathophysiology of MEWDS will be discussed based clinical appraisal and on multimodal imaging appraisal.

Methods: Narrative review and perspective opinion.

Varicella-zoster virus reactivation causing herpes zoster ophthalmicus (HZO) after SARS-CoV-2 vaccination - report of three cases.

Purpose: We are reporting 3 patients who presented acute zoster ophthalmicus (HZO), an activation of varicella-zoster virus, after mRNA anti-SARS-CoV-2 vaccination, seen directly or referred to our center.

Cases: A 73-year-old woman with history of ocular sarcoidosis presented HZO in the right V1 dermatome 16 days after a single booster dose of vaccination (Pfizer BioNTech). A 69-year-old woman presented HZO in her V1 left dermatome, occurring 10 days after her first dose of Pfizer BioNTech vaccine.

Initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease presenting with unilateral exudative retinal detachment despite bilateral choroidal involvement.

Purpose: To investigate the frequency of initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease presenting with unilateral exudative retinal detachment.

Methods: A retrospective case series.

Results: During the period between January 1998 and December 2020, we identified 135 patients with initial-onset acute uveitis associated with VKH disease.

Acute Zonal Occult Outer Retinopathy (AZOOR) Results from a Clinicopathological Mechanism Different from Choriocapillaritis Diseases: A Multimodal Imaging Analysis.

Background And Aim: AZOOR is a rare disease characterized by loss of zones of outer retinal function, first described by J Donald Gass in 1993. Symptoms include acute onset photopsias and subjective visual field losses. The syndrome is characterized by a normal fundus appearance, scotomas and electroretinographic changes pointing towards outer retinal dysfunction.

Classification of Non-Infectious and/or Immune Mediated Choroiditis: A Brief Overview of the Essentials.

The choroid was poorly accessible to imaging investigation until the last decade of the last century. With the availability of more precise imaging methods such as indocyanine green angiography (ICGA) and, later, optical coherence tomography (OCT), enhanced depth OCT (EDI-OCT), and OCT angiography (OCTA), appraisal of choroidal inflammation has substantially gained in accuracy. This allowed to precisely determine which structures were touched in the different non-infectious choroiditis entities and made it possible to classify this group of diseases, ICGA signs, mainly hypofluorescent lesions, were identified and described.

When HIV Immunodeficiency and Heterochromia Confuse the Issue: Recurrent Zoster Uveitis Mistaken for Fuchs' Uveitis.

Purpose: We report a case with iris heterochromia misdiagnosed as Fuchs' uveitis which finally turned out to be a unilateral zoster uveitis in an HIV-positive patient.

Case Report: A 45-year old patient was seen for a recurrent right anterior uveitis treated with prednisolone 1% drops BID. The iris of the right eye was hypochromic and atrophic and several small granulomatous keratic precipitates (KPs) were present.

Granulomatous Features in Juvenile Idiopathic Arthritis-Associated Uveitis is Not a Rare Occurrence.

Background: Juvenile idiopathic arthritis (JIA)-associated uveitis is the most common form of intraocular inflammation with systemic involvement in childhood. Textbooks tend to describe it as non-granulomatous anterior uveitis. Here, we report the percentage of granulomatous features in JIA-related uveitis among patients at a uveitis referral center.

Clinical Course and Treatment Paradigms for JIA-related Uveitis and Pars Planitis Uveitis Using Precise Ocular Investigational Methods.

Purpose: The aim of this study was to report on the disease course and management modalities in patients with juvenile idiopathic arthritis-related uveitis (JIA uveitis) or intermediate uveitis of the pars planitis type (PP) who were followed up using precise ocular investigational techniques.

Methods: This is a retrospective single-centre study. All charts of patients seen in our centre over 15 years (2005 - 2019) with a diagnosis of JIA uveitis or PP were retrieved and analysed for clinical course, severity of inflammation, type of management, and the role played by precise investigational techniques (laser flare photometry [LFP], optical coherence tomography [OCT] and fluorescein angiography [FA]) in therapeutic decisions.

Choriocapillaris Involvement in Acute Syphilis Posterior Placoid Chorioretinitis is Responsible for Functional Impairment and Points towards an Immunologic Mechanism: A Comprehensive Clinicopathological Approach.

Purpose: To evaluate the multimodal imaging of acute syphilitic posterior placoid chorioretinitis (ASPPC) lesions in order to elucidate their pathophysiology which seems to resemble choriocapillaritis as in primary inflammatory choriocapillaropathies such as multifocal choroiditis (MFC) and acute posterior multifocal placoid pigment epitheliopathy (APMPPE).

Methods: Charts of patients with ASPPC seen in the Centre for Ophthalmic Specialised Care, Lausanne, Switzerland, were retrieved. Fundus autofluorescence (FAF), spectral domain optical coherence tomography (SD-OCT), fluorescein angiography (FA), indocyanine green angiography (ICGA), and when available, OCT angiography were analyzed and compared to a case of MFC.

Vogt-Koyanagi-Harada is a Curable Autoimmune Disease: Early Diagnosis and Immediate Dual Steroidal and Non-Steroidal Immunosuppression are Crucial Prerequisites.

Purpose: It is crucial to subdivide Vogt-Koyanagi-Harada (VKH) disease into two subentities, initial-onset disease versus chronically evolving disease. For early diagnosis and precise follow-up of VKH choroiditis, the "Revised criteria for VKH" are no more sufficient for the appraisal of VKH and new biomarkers for disease activity are needed. It has been shown that, if initial-onset disease is treated promptly within the "therapeutic window of opportunity" and long enough with dual steroidal and non-steroidal immunosuppression, the disease can be cured in a large proportion of cases, an approach still contested.

Susac syndrome (Retino-cochleo-cerebral vasculitis), the ophthalmologist in the role of the whistleblower.

Background/purpose: Susac syndrome is a rare microangiopathy of suspected autoimmune origin affecting arteries of the retina, the cochlea and the brain. The aim of the study was to give a review of the disease entity and determine the proportion of cases and their characteristics in a uveitis referral centre.

Patients And Methods: Charts of patients with the diagnosis of Susac syndrome seen in the Uveitis Clinic of the Centre for Ophthalmic Specialised Care (COS), Lausanne, Switzerland were reviewed retrospectively to determine the frequency of such cases in a uveitis referral centre.

Initial-onset acute and chronic recurrent stages are two distinctive courses of Vogt-Koyanagi-Harada disease.

Purpose: To describe distinctive stages of Vogt-Koyanagi-Harada (VKH) disease: initial-onset acute versus chronic recurrent disease.

Methods: A comprehensive literature review regarding stages and clinical presentations of VKH disease was conducted.

Results: Despite a list of signs that has been described as characteristic features of early or late phases of VKH disease, the current classification -developed by an international committee and published in 2001- does not consider a distinction regarding the time from onset of disease symptoms, and specific findings observed at certain time point from the symptoms presentation and outcomes related to the stage of VKH disease.

Definition of Uveitis Refractory to Treatment: A Systematic Review in the Absence of a Consensus.

Purpose: To evaluate the different definition of refractoriness in uveitis in the literature.

Methods: We systematically searched the literature in order to identify definitions of refractory noninfectious uveitis in adult patients. A search strategy in the databases of MEDLINE and Scopus was used to find articles published between January 2005 and October 2018.

Indocyanine green angiographic findings in presumed intraocular tuberculosis.

Purpose: To study features of Indocyanine green angiography (ICGA) in patients with presumed intraocular tuberculosis.

Methods: Retrospective study of 48 consecutive patients (77 eyes) who underwent ICGA. The following signs were analysed: choroidal perfusion inhomogeneity, early hyperfluorescent stromal vessels, round or oval hypofluorescent dark dots (HDDs), hypofluorescent geographic lesions (HGLs), fuzzy or lost pattern of large stromal choroidal vessels, disc hyperfluorescence and diffuse late choroidal hyperfluorescence.

Advances and potential new developments in imaging techniques for posterior uveitis Part 2: invasive imaging methods.

The aim of this review was to identify the imaging methods at our disposal to optimally manage posterior uveitis at the present time. The focus was put on methods that have become available since the 1990s, some 30 years after fluorescein angiography had revolutionised imaging of posterior uveitis in particular imaging of the retinal vascular structures in the 1960s. We have focussed our review on precise imaging methods that have been standardised and validated and can be used universally thanks to commercially produced and available instruments for the diagnosis and follow-up of posterior uveitis.

Advances and potential new developments in imaging techniques for posterior uveitis. Part 1: noninvasive imaging methods.

The aim of this review was to identify the imaging methods at our disposal to optimally manage posterior uveitis at the present time. The focus was put on methods that have become available since the 1990s, some 30 years after fluorescein angiography had revolutionized imaging of posterior uveitis in particular imaging of the retinal vascular structures in the 1960s. We have focussed our review on precise imaging methods that have been standardized and validated and can be used universally thanks to commercially produced and available instruments for the diagnosis and follow-up of posterior uveitis.

Vogt-Koyanagi-Harada disease: recurrence rates after initial-onset disease differ according to treatment modality and geographic area.

Unlabelled: Background/Purpose Vogt-Koyanagi-Harada (VKH) disease is a primary autoimmune stromal choroiditis producing a spill-over panuveitis. For initial-onset VKH disease, it is increasingly thought that corticosteroid therapy is not sufficient and additional non-steroidal immunosuppressive therapy is needed. At the 11th workshop on VKH, the disease was said to be well controlled with corticosteroids alone in Japanese patients.

Sensitivity of indocyanine green angiography compared to fluorescein angiography and enhanced depth imaging optical coherence tomography during tapering and fine-tuning of therapy in primary stromal choroiditis: A case series.

Purpose: To investigate indocyanine green angiography (ICGA), fluorescein angiography (FA), and enhanced depth imaging optical coherence tomography measured choroidal thickness (EDI-OCT-CT) in the follow-up of inflammatory activity in stromal choroiditis [Vogt-Koyanagi-Harada disease (VKH) and birdshot retinochoroiditis (BRC)] under treatment in order to monitor tapering of therapy or readjustment of therapy in case of subclinical disease recurrence.

Methods: Patients with initial onset disease and/or treatment-naive stromal choroiditis (VKH & BRC) at entry, quiet under therapy, and having had a follow-up of at least four years monitored with dual FA and ICGA and EDI-OCT-CT measurements were analyzed retrospectively. ICGA and FA scores and EDI-OCT-CT values were correlated with therapy, and significant changes of each modality were correlated with disease evolution.

Catching the therapeutic window of opportunity in early initial-onset Vogt-Koyanagi-Harada uveitis can cure the disease.

Purpose: Vogt-Koyanagi-Harada (VKH) disease is a primary autoimmune granulomatous choroiditis that begins in the choroidal stroma. The aim of this review was to gather a body of evidence for the concept of a window of therapeutic opportunity, defined as a time interval following initial-onset disease during which adequate treatment will substantially modify the disease outcome and possibly even lead to cure, similar to what has been described for rheumatoid arthritis.

Methods: We reviewed the literature and consulted leading experts in VKH disease to determine the consensus for the notion of a therapeutic window of opportunity in VKH disease.