Long-Term Follow-Up of Patients with Scleritis After Rituximab Treatment Including B Cell Monitoring.

Purpose: We report the long-term effect of rituximab (RTX) in scleritis and determine the value of B-cell monitoring for the prediction of relapses.

Methods: We retrospectively studied 10 patients with scleritis, who were treated with RTX. Clinical characteristics were collected, and blood B-cell counts were measured before the start of RTX, and at various time points after treatment.

Vitreous proteomics, a gateway to improved understanding and stratification of diverse uveitis aetiologies.

Purpose: The vitreous proteome might provide an attractive gateway to discriminate between various uveitis aetiologies and gain novel insights into the underlying pathophysiological processes. Here, we investigated 180 vitreous proteins to discover novel biomarkers and broaden disease insights by comparing (1). primary vitreoretinal lymphoma ((P)VRL) versus other aetiologies, (2).

Parafoveal Microvascular Alterations in Ocular and Non-Ocular Behҫet's Disease Evaluated With Optical Coherence Tomography Angiography.

Purpose: To compare quantitative optical coherence tomography angiography (OCT-A) measurements of the parafoveal microvasculature in retinal capillary plexuses among Behҫet uveitis (BU) patients, non-ocular Behҫet's disease (NOBD) patients, and healthy volunteers (HVs).

Methods: Sixty-eight subjects were enrolled in this prospective observational cross-sectional study. OCT-A imaging was performed using the Heidelberg Engineering Spectralis OCT.

Reactivation of CNV after Discontinuation of Bevacizumab Treatment of Age-Related Macular Degeneration.

Introduction: Treatment of exudative age-related macular degeneration (ARMD) has shifted to pro re nata and treat-extend-stop strategies. However, a rational discontinuation strategy is lacking. To develop such a strategy, it is important to determine choroidal neovascularization (CNV) recurrence rates after anti-VEGF treatment is discontinued.

Development of a Genotype Assay for Age-Related Macular Degeneration: The EYE-RISK Consortium.

Purpose: To develop a genotype assay to assess associations with common and rare age-related macular degeneration (AMD) risk variants, to calculate an overall genetic risk score (GRS), and to identify potential misdiagnoses with inherited macular dystrophies that mimic AMD.

Design: Case-control study.

Participants: Individuals (n = 4740) from 5 European cohorts.

Six and eight weeks injection frequencies of bevacizumab are non-inferior to the current four weeks injection frequency for quality of life in neovascular age-related macular degeneration: a randomized controlled trial.

Purpose: Patients with neovascular age-related macular degeneration (nARMD) will not deteriorate on visual acuity and retinal thickness when treated with bevacizumab injection frequencies of 6 or 8 weeks compared to 4 weeks. This study aimed to investigate this non-inferiority in quality of life (QoL). We hypothesized that less frequent bevacizumab injections are not inferior regarding patients reported QoL.

A randomized clinical trial comparing prompt photodynamic therapy with 3 months observation in patients with acute central serous chorioretinopathy with central macular leakage.

Purpose: The purpose of the study was to demonstrate whether photodynamic therapy in patients with acute central serous chorioretinopathy, with the leakage point within one optic disk diameter from the fovea, can be safely deferred.

Methods: A single-center, randomized, controlled trial was conducted. Patients were randomized to photodynamic therapy within a week after presentation (Group I, 26 patients) or observation during 3 months (Group II, 26 patients).

Outcomes in patients with chronic uveitis: which factors matter to patients? A qualitative study.

Purpose: Outcome measurements currently used in chronic uveitis care fail to cover the full patient perspective. The aim of this study is to develop a conceptual model of the factors that adult patients with chronic uveitis consider to be important when evaluating the impact of their disease and treatment.

Methods: A qualitative study design was used.

ALPK1 missense pathogenic variant in five families leads to ROSAH syndrome, an ocular multisystem autosomal dominant disorder.

Purpose: To identify the molecular cause in five unrelated families with a distinct autosomal dominant ocular systemic disorder we called ROSAH syndrome due to clinical features of retinal dystrophy, optic nerve edema, splenomegaly, anhidrosis, and migraine headache.

Methods: Independent discovery exome and genome sequencing in families 1, 2, and 3, and confirmation in families 4 and 5. Expression of wild-type messenger RNA and protein in human and mouse tissues and cell lines.

A joint model for dynamic prediction in uveitis.

Uveitis is characterised as a recurrent inflammation of the eye and an ongoing inflammation can have severe impact on the visual acuity of the patient. The Rotterdam Eye Hospital has been collecting data on every uveitis patient visiting the hospital since 2000. We propose a joint model for the inflammation and visual acuity with the purpose of making dynamic predictions.

Bevacizumab in age-related macular degeneration: a randomized controlled trial on the effect of on-demand therapy every 4 or 8 weeks.

Purpose: Intravitreal anti-vascular endothelial growth factor (VEGF) injections are an effective treatment for neovascular age-related macular degeneration (nARMD). Bevacizumab appears to be a cost-effective off-label anti-VEGF alternative to ranibizumab, but an optimal injection schedule has not yet been determined. In this study, we investigate whether on-demand bevacizumab treatment every 8 weeks is non-inferior to on-demand bevacizumab every 4 weeks in treating nARMD.

Treatment Effects in Retinal Angiomatous Proliferation Imaged with OCT Angiography.

Purpose: This prospective case series is aimed at exploring optical coherence tomographic angiography (OCT-A) as a treatment monitoring tool in patients treated for retinal angiomatous proliferation (RAP).

Methods: Twelve treatment-naïve RAP patients were included, with a median age of 79 years (range 65-90). Patients were imaged with an experimental 1,040-nm swept-source phase-resolved OCT-A instrument before and after treatment.

Functionally distinct ERAP1 and ERAP2 are a hallmark of HLA-A29-(Birdshot) Uveitis.

Birdshot Uveitis (Birdshot) is a rare eye condition that affects HLA-A29-positive individuals and could be considered a prototypic member of the recently proposed 'MHC-I (major histocompatibility complex class I)-opathy' family. Genetic studies have pinpointed the endoplasmic reticulum aminopeptidase (ERAP1) and (ERAP2) genes as shared associations across MHC-I-opathies, which suggests ERAP dysfunction may be a root cause for MHC-I-opathies. We mapped the ERAP1 and ERAP2 haplotypes in 84 Dutch cases and 890 controls.

Clinical characteristics and long-term visual outcome of severe phenotypes of chronic central serous chorioretinopathy.

Purpose: The aim of this study was to investigate disease onset and disease progression in patients with severe chronic central serous chorioretinopathy (cCSC).

Patients And Methods: The medical records of 143 cCSC patients (199 eyes) were reviewed. All cases had visual complaints for >6 months and showed signs of a severe disease phenotype on optical coherence tomography (OCT) and fluorescein angiography (FA).

Aberrant leukocyte telomere length in Birdshot Uveitis.

Purpose: Birdshot Uveitis (BU) is an archetypical chronic inflammatory eye disease, with poor visual prognosis, that provides an excellent model for studying chronic inflammation. BU typically affects patients in the fifth decade of life. This suggests that it may represent an age-related chronic inflammatory disease, which has been linked to increased erosion of telomere length of leukocytes.

SF-6D utility values for the better- and worse-seeing eye for health states based on the Snellen equivalent in patients with age-related macular degeneration.

Objective: Economic evaluations in wet age-related macular degeneration (ARMD) is hampered as often utility values for solely one eye are used, mostly the better-seeing eye (BSE). Moreover, frequently chosen methods rely on patient values and/or disease specific measures, while economic evaluations prefer generic quality of life (QoL) measures based on societal preferences. The generic QoL utility instrument EQ-5D has shown to be insensitive for differences in visual acuity.

Three Cases of Erdheim-Chester Disease With Intraocular Manifestations: Imaging and Histopathology Findings of a Rare Entity.

Purpose: To report intraocular manifestations of Erdheim-Chester Disease (ECD) with multimodal imaging.

Design: A retrospective observational case series.

Methods: This was a multicenter case series of 3 patients with confirmed tissue diagnosis of ECD that showed intraocular manifestations and were imaged at baseline and follow-up visits.

Early surgical treatment of retinal hemangioblastomas.

Purpose: To evaluate the clinical course after early surgical treatment with excision of retinal hemangioblastomas (RHs) before development of major complications.

Methods: Interventional case series of four eyes (four patients) with a peripheral RH that had not yet been treated by laser or cryotherapy prior to surgery. All eyes underwent 23-gauge vitrectomy with lesion excision.

Clinical Manifestations and Outcome of Syphilitic Uveitis.

Purpose: To analyze visual outcome, effectiveness of various modes of antibiotic treatment, and prognostic factors in patients with serologically proven syphilitic uveitis.

Methods: The clinical records of 85 patients (139 eyes) diagnosed with syphilitic uveitis between 1984 and 2013 at tertiary centers in The Netherlands were retrospectively analyzed.

Results: Mean age was 47 years (range, 27-73 years), 82.

Diagnosis and treatment of uveitis; not restricted to the ophthalmologist.

Uveitis is associated with a wide range of underlying causes. Familiarity with its clinical manifestations, referral indications, and treatment strategies are required for the optimal use of current therapeutic options. Uveitis can be caused by infectious and non-infectious factors, resulting in differing prognoses and treatments.

Somatostatin receptor scintigraphy patterns in patients with sarcoidosis.

Purpose: Sarcoidosis is a multisystem granulomatous disorder, most frequently involving the lungs, skin, or eyes. Somatostatin receptor scintigraphy (SRS) can visualize sarcoid granulomas through binding of a radionuclide-coupled somatostatin analog to somatostatin receptors that are expressed in sarcoidosis. Uptake and patterns on SRS were studied and correlated to clinical and conventional findings.

Updated cannulation technique for tissue plasminogen activator injection into peripapillary retinal vein for central retinal vein occlusion.

Purpose: To update the surgical technique in which a vitrectomy is performed and a retinal branch vein is cannulated and infused with recombinant tissue plasminogen activator (RTPA) to treat central retinal vein occlusion (CRVO) in patients who present with very low visual acuity (VA).

Methods: Twelve consecutive patients (12 eyes) with CRVO and low VA (logMAR >1.00) at presentation were treated using this method.

Phase-Resolved Doppler Optical Coherence Tomographic Features in Retinal Angiomatous Proliferation.

Purpose: To study patients diagnosed with retinal angiomatous proliferation (RAP) based on conventional imaging techniques with phase-resolved Doppler optical coherence tomography (OCT) to detect and localize blood flow in RAP lesions; and to compare these findings to conventional imaging, which are mostly invasive and give limited information concerning intra- and transretinal blood flow.

Design: Single-center, consecutive observational case series.

Methods: Twelve treatment-naïve patients diagnosed with RAP based on fundus examination, fluorescein angiography, and indocyanine green angiography were included.