Clinical Spectrum and Perspective in Bilateral Acute Retinal Necrosis: Systematic Review.

Bilateral acute retinal necrosis (BARN) represents a broad ophthalmological field of severe retinal pathologies associated with poor visual prognosis and blindness. The purpose of this review is to examine the clinical spectrum in detail over the past few years, exploring laboratory and instrumental diagnosis, and providing useful and up-to-date guidance in this field. A systematic review of this field has been performed through the PRISMA guidelines, searching in the PUBMED database.

Impact of HLA-B51 on Uveitis and Retinal Vasculitis: Data from the AIDA International Network Registries on Ocular Inflammatory Disorders.

Purpose: The clinical relevance of human leukocyte antigen (HLA) subtypes such as HLA-B51 on Behçet's disease (BD)-related uveitis and non-infectious uveitis (NIU) unrelated to BD remains largely unknown.

Methods: Data were prospectively collected from the International AIDA Network Registry for BD and for NIU. We assessed differences between groups (NIU unrelated to BD and positive for HLA-B51, BD-related uveitis positive for HLA-B51 and BD-related uveitis negative for HLA-B51) in terms of long-term ocular complications, visual acuity (VA) measured by best corrected visual acuity (BCVA), anatomical pattern, occurrence of retinal vasculitis (RV) and macular edema over time.

Mast cells in human choroid and their role in age-related macular degeneration (AMD).

The role of mast cells in physiologic and pathological processes extends far beyond the allergy processes: they are involved in wound healing, chronic inflammation, and tumor growth. This short article emphasizes the role played by mast cells in age-related macular degeneration (AMD). Mast cells can induce angiogenesis and are present around Bruch's membrane during the early and late stages of choroidal neovascularization in AMD.

Aqueous humor as eye lymph: A crossroad between venous and lymphatic system.

Aqueous humor (AQH) is a transparent fluid with characteristics similar to those of the interstitial fluid, which fills the eyeball posterior and anterior chambers and circulates in them from the sites of production to those of drainage. The AQH volume and pressure homeostasis is essential for the trophism of the ocular avascular tissues and their normal structure and function. Different AQH outflow pathways exist, including a main pathway, quite well defined anatomically and referred to as the conventional pathway, and some accessory pathways, more recently described and still not fully morphofunctionally understood, generically referred to as unconventional pathways.

Ocular manifestations of cryoglobulinemia: a reappraisal.

Background/objectives: To describe frequency and type of ocular manifestations in patients with cryoglobulinemic vasculitis (CV), as well as management approaches and outcomes.

Subjects/methods: This was a retrospective, observational, cohort study of patients who were diagnosed with CV at a single center and regularly underwent a comprehensive ocular assessment.

Results: Ophthalmologic manifestations were recorded in 16 patients (28%).

The eye is a common site of granulomatosis with polyangiitis. A collaborative study.

Background: Ocular manifestations of granulomatosis with polyangiitis (GPA) have been reported in a limited number of studies and with largely variable frequency. Here we report on the clinical, diagnostic, and therapeutic features of a cohort of 63 GPA patients, with particular regard to 22 of them with ophthalmic involvement (35%).

Methods: Clinical manifestations, results of immunological findings, histopathological pictures, imaging data, Birmingham Vasculitis Activity Score, therapeutic regimens, and outcomes were retrospectively analyzed.

Retrograde Optic Nerve Degeneration in Pituitary Adenoma: A Study with RE-PERG.

Purpose: RE-PERG is altered in presence of primary neuronal degeneration of retinal ganglion cells, both in glaucoma and other diseases. A previous study showed that in a model of retrograde degeneration (vascular dementia) RE-PERG was normal. In this study, we enrolled patients with pituitary adenoma (PA) to evaluate RE-PERG findings in another model of retrograde degeneration compared with healthy controls (HC).

Development and implementation of the AIDA International Registry for patients with Behçet's disease.

Purpose of the present paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients with Behçet's disease (BD). The Registry is a clinical physician-driven non-population- and electronic-based instrument implemented for the retrospective and prospective collection of real-life data about demographics, clinical, therapeutic, laboratory, instrumental and socioeconomic information from BD patients; the Registry is based on the Research Electronic Data Capture (REDCap) tool, which is thought to collect standardised information for clinical real-life research, and has been realised to change over time according to future scientific acquisitions and potentially communicate with other existing and future Registries dedicated to BD. Starting from January 31st, 2021, to February 7th, 2022, 110 centres from 23 countries in 4 continents have been involved.

Macular ganglion cell-inner plexiform layer defect patterns in multiple sclerosis patients without optic neuritis: A Spectral-Domain-Optical Coherence Tomography Cross-Sectional, Case-Control, Pilot Study.

Purpose: Spectral-domain optical coherence tomography (SD-OCT) was used to evaluate, in patients with multiple sclerosis without a history of optic neuritis (MSNON), the proportion of the different macular ganglion cell-inner plexiform layer complex (mGCIP) defect patterns. The results were compared with those of healthy controls (HCs).

Methods: In this cross-sectional case-control study, 34 eyes of 34 individuals, 17 with MSNON and 17 HCs, were evaluated.

Microvascular changes in the recurrent cystoid macular edema secondary to posterior noninfectious uveitis on optical coherence tomography angiography.

Background: Posterior uveitis represents the second most frequent type of uveitis (15-30% of all uveitis). Noninfectious posterior uveitis complicated with secondary cystoid macular edema (CME) affects the visual prognosis negatively. The objective of the current study is to detect possible microvascular changes causing relapsing uveitis-related CME using optical coherence tomography angiography (OCTA).

Ocular Manifestations in an Italian Cohort of Patients with Takayasu Arteritis.

Purpose: We describe ophthalmic manifestations, therapy, and outcomes in 16 patients with Takayasu arteritis (TA).

Methods: Takayasu retinopathy was detected in 15 eyes of 9 patients and hypertensive retinopathy in 14 eyes of 7 patients.

Results: Visual acuity was normal in 7 eyes, 20/40 to 20/200 in 20 eyes, counting fingers in 2 eyes, hand motion in 2 eyes, and no light perception in 1 eye.

Natural and iatrogenic ocular manifestations of rheumatoid arthritis: a systematic review.

Purpose: To provide an overview of the ocular features of rheumatoid arthritis (RA) and of the ophthalmic adverse drug reactions (ADRs) that may be associated with the administration of antirheumatic drugs.

Methods: A systematic literature search was performed using the PubMed, MEDLINE, and EMBASE databases. In addition, a cohort of 489 RA patients who attended the Authors' departments were examined.

A 10-year-old immunocompetent girl with endogenous fungal endophthalmitis: Report of a case and review of the literature.

Endogenous Endophthalmitis (EE) is a rare cause of blindness in the pediatric age group and this may account for the paucity of management guidelines in the literature. In this report, we describe our experience with a 10-year-old immunocompetent female who developed EE and became blind because of rapidly progressive and destructive inflammatory changes in her eye in spite of seemingly timely treatment.

The Spectrum of Ocular Manifestations in Patients with Waldenström's Macroglobulinemia.

Purpose: To investigate the ocular manifestations in 91 Waldenström's macroglobulinemia (WM) patients.

Methods: Retrospective, cross-sectional, observational analysis.

Results: Ocular impairments, detected in 19 patients, included flame-shaped hemorrhages, venous sausaging, papilledema, macular detachments, or central retinal vein occlusion in 16 patients; paraproteinemic keratopathy in 2; and a CANOMAD syndrome in 1.

Takayasu arteritis: a cohort of Italian patients and recent pathogenetic and therapeutic advances.

Takayasu arteritis (TAK) is a rare granulomatous vasculitis of unknown etiology that mainly affects the aorta and its major branches. The aim is to describe the clinical features, diagnostic procedures, pathogenesis, and management of TAK in a longitudinal cohort of patients recruited within a single region of southern Italy. The cohort included 43 patients who were diagnosed with TAK and followed up according to a standard protocol, in a collaboration between four university tertiary referral centers and a regional hospital.

RE-PERG in early-onset Alzheimer's disease: A double-blind, electrophysiological pilot study.

Purpose: To evaluate the ability of re-test pattern electroretinogram (RE-PERG), a non-invasive and fast steady-state PERG, to detect inner retinal bioelectric function anomalies in patients with early-onset Alzheimer's disease (AD).

Methods: The study population consisted of 17 patients with AD-related mild cognitive impairment (MCI), 16 patients with vascular dementia (VD)-related MCI, both assessed using the neuropsychological Mini-Mental State Examination (MMSE) and by structural magnetic resonance imaging, and 19 healthy, age-matched normal controls (NC). All participants were visually asymptomatic, had normal or near-normal general cognitive functioning and no or minimal impairments in daily life activities.

Ocular sarcoidosis: clinical experience and recent pathogenetic and therapeutic advancements.

Purpose: To describe the ocular manifestations in a cohort of patients with systemic sarcoidosis (SS). Recent advances in the pathophysiology, diagnosis, and therapy of SS are also discussed.

Methods: Data from 115 Italian patients diagnosed between 2005 and 2016 were retrospectively reviewed.

Amyloidosis and Ocular Involvement: an Overview.

: To describe the ophthalmic manifestations of amyloidosis and the corresponding therapeutic measures.: The 178 patients included in the study had different types of amyloidosis, diagnosed at a single internal medicine institution (Bari, Italy). To provide a comprehensive review of the types of amyloidosis that can be associated with ocular involvement, the images and clinical descriptions of patients with amyloidosis structurally related to gelsolin, keratoepithelin and lactoferrin were obtained in collaborations with the ophthalmology departments of hospitals in Mainz (Germany) and Helsinki (Finland).

RE-PERG, a new paradigm for glaucoma diagnosis, in myopic eyes.

Purpose: To evaluate reliability of steady-state pattern electroretinogram (ssPERG) phase variability in re-test (procedure called RE-PERG) in the presence of myopia, which is known to affect ssPERG amplitude, in glaucomatous patients (GP), normal controls (NC), and myopic patients (MY).

Methods: The procedure was performed on 50 GP, 35 NC, and 19 MY. All subjects were examined with RE-PERG, spectral-domain coherence tomography (SD-OCT), and standard automated perimetry (SAP).

Pemphigus and mucous membrane pemphigoid: An update from diagnosis to therapy.

Pemphigus diseases (PDs) and mucous membrane pemphigoid (MMP) are a group of immune-mediated mucocutaneous disorders clinically characterized by the formation of blisters, erosions and ulcers. The skin and mucous membranes are predominantly affected, with the oropharyngeal mucosa as the initially involved site. Ocular involvement is also a frequent feature of these diseases.

Ocular Involvement in Systemic Lupus Erythematosus: The Experience of Two Tertiary Referral Centers.

Purpose: To assess the prevalence of the ocular manifestations related to the disease and/or ascribable to the administration of potentially toxic drugs in a cohort of 98 patients with systemic lupus erythematosus (SLE).

Methods: Retrospective, observational study reporting the experience of two tertiary referral centers.

Results: Overall, an ocular involvement was detected in 29 patients (29.