Front Ophthalmol (Lausanne)
February 2024
Autoinflammatory diseases include disorders with a genetic cause and also complex syndromes associated to polygenic or multifactorial factors. Eye involvement is present in many of them, with different extent and severity. The present review covers ophthalmological lesions in the most prevalent monogenic autoinflammatory diseases, including FMF (familial Mediterranean fever), TRAPS (TNF receptor-associated periodic syndrome), CAPS (cryopyrin-associated periodic syndromes), Blau syndrome, DADA2 (deficiency of adenosine deaminase 2), DITRA (deficiency of the interleukin-36 receptor antagonist), other monogenic disorders, including several ubiquitinopathies, interferonopathies, and the recently described ROSAH (retinal dystrophy, optic nerve edema, splenomegaly, anhidrosis, and headache) syndrome, and VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome.
View Article and Find Full Text PDFPurpose: Hydroxychloroquine is currently recommended for the treatment of systemic lupus erythematosus (SLE), but it can cause irreversible retinal toxicity. This study aimed to identify factors associated with early hydroxychloroquine-induced retinal toxicity in patients with SLE from a single centre for 20 years.
Methods: SLE patients diagnosed between 1998 and 2017 and followed up for at least 1 year were included.
Objective: To compare multimodal structural and functional diagnostic methods in patients with systemic lupus erythematosus (SLE) treated with hydroxychloroquine, to identify the best complementary approach for detecting subclinical retinal toxicity.
Methods: A cross-sectional, unicentric study was conducted on patients with SLE treated with hydroxychloroquine. Each patient underwent a comprehensive ophthalmic evaluation, comprising structural tests (spectral-domain optical coherence tomography (SD-OCT), OCT, OCT angiography (OCTA), fundus autofluorescence (FAF)) and functional tests (automated perimetry for visual field (VF) testing, multifocal electroretinography (mfERG)).
Background: Microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are the two major antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
Objectives: To characterize a homogenous AAV cohort and to assess the impact of clinicopathological profiles and ANCA serotypes on clinical presentation and prognosis. Clinical differences in GPA patients according to ANCA serotype and the diagnostic yield for vasculitis of biopsies in different territories were also investigated.
Purpose: This study assessed the effectiveness of the 0.19-mg fluocinolone acetonide (FAc) implant by multimodal measurements in patients with non-infectious uveitis (NIU) in a real-world setting in Spain.
Methods: A prospective study of patients who had NIU including uveitic macular oedema (UME) with ≥ 12 months follow-up was done.
Objective: The aim of the present study was to detect preclinical changes in SLE patients in retinal microvascularization or retinal and optical nerve structure by optical coherence tomography.
Methods: This cross-sectional, single-centre study aimed to describe structural changes [macular and retinal nerve fibre layer (RNFL) thickness] by structural spectral-domain optical coherence tomography (SD-OCT) and perifoveal vascular [vessel density (VD) and vascular perfusion (VP) and foveal avascular zone (FAZ) structural parameters] findings by OCT angiography (OCTA) in 78 SLE patients and 80 healthy volunteers. In addition, we analysed their association with clinical and laboratory parameters, medications received, disease duration, and SLE activity and damage.
Background: Because of the efficacy and good safety profile of antimalarials in systemic lupus erythematosus (SLE), hydroxychloroquine (HCQ) is currently recommended in all SLE patients. However, patients' compliance was reported as suboptimal. This study aims to elucidate the reasons for discontinuing antimalarials in a large series of SLE patients followed in a single centre during the last 50 years
Material And Methods: Among all patients diagnosed between 1968 and 2017 at our reference centre, retrospective data were obtained from electronic medical records of SLE patients consecutively visited during 2015-2017 and controlled for at least 1 year.
Purpose: To describe clinical and ophthalmologic features and outcomes of patients with coronavirus disease-19 with retinal vascular occlusions.
Methods: Retrospective multicenter case series and PubMed review of cases reported from March 2020 to September 2021. Outcome measures are as follows: type of occlusion, treatments, best-corrected visual acuity, and central macular thickness on optical coherence tomography.
Cystoid macular edema (CME) is the most common cause of decreased visual acuity after both vitrectomy and cataract surgery. Various strategies have been used for its treatment, such as intravitreal corticosteroids. The intravitreal fluocinolone acetonide implant (Iluvien) is approved for the treatment of persisting diabetic macular edema and for the prevention of recurrence of non-infectious uveitis affecting the posterior segment.
View Article and Find Full Text PDFAim: To describe and evaluate the efficacy of Ahmed glaucoma valve implantation (AGV) combined with pars plana vitrectomy (PPV) in a single surgical act for the treatment of advanced neovascular glaucoma (NVG).
Methods: Retrospective observational case series included 51 eyes from 50 patients with severe NVG treated with PPV, AGV, and panretinal photocoagulation and/or cryotherapy in a single surgical act during a 13-year period (2005-2018). Preoperative, intraoperative and postoperative data at day 1 and months 1, 3, 6, 21, and 24 were systematically collected.
Background: This study aims at describing the therapeutic outcome of patients carrying the R92Q variant in the gene treated with anakinra (ANA) or canakinumab (CAN) and identifying any factors predictive of complete response to IL-1 inhibition.
Methods: Clinical data of patients treated with ANA or CAN for recurrent inflammatory attacks due to the presence of the R92Q variant were retrospectively collected and analysed.
Results: Data about 20 treatment courses with IL-1 inhibitors (16 with ANA and 4 with CAN) from 19 patients were collected.
Objective: To assess the efficacy of biologic drugs, beyond tumor necrosis factor- (TNF-) inhibitors, in the management of noninfectious refractory scleritis, either idiopathic or associated with systemic immune-mediated disorders. . This is a retrospective study assessing the efficacy of several biologic agents (rituximab, anakinra, tocilizumab, and abatacept) and the small molecule tofacitinib in the treatment of scleritis through assessment of scleral inflammation and relapses, as well as treatment impact on best-corrected visual acuity (BCVA) and safety profile.
View Article and Find Full Text PDFObjectives: To evaluate the efficacy of tumour necrosis factor (TNF)-α inhibitors in refractory non-infectious scleritis.
Methods: We carried out a retrospective study assessing the efficacy of TNF-α inhibitors in the treatment of scleritis, scleritis relapses, glucocorticoid (GC)-sparing effect, impact on best-corrected visual acuity (BCVA) and safety profile.
Results: Nineteen patients (28 eyes) were eligible for analysis.
: To study the risk factors for visual loss in presumed tuberculosis-related uveitis (TRU).: Retrospective observational cohort study of patients with TRU, either treated or not for tuberculosis, from January 2005 to January 2017. Clinical and demographic variables were recorded.
View Article and Find Full Text PDFA 37-year-old female presented with severe apraxia of lid opening (ALO) affecting the right upper lid associated with Becker congenital myotonia (MC). The patient had a history of right upper lid ptosis for 25 years that was exacerbated over the previous month with severe incapacity to open her right eye. No other associated neurological or ophthalmic symptoms were observed.
View Article and Find Full Text PDFIntroduction: Isolated retinal vasculitis (IRV) is an inflammatory condition of unknown etiology confined to the retinal vessels. In contrast to secondary retinal vasculitis (RV), IRV has not been well characterized.
Objective: To describe and characterize isolated forms of RV.
To develop a disease activity index for patients with uveitis (UVEDAI) encompassing the relevant domains of disease activity considered important among experts in this field. The steps for designing UVEDAI were: (a) Defining the construct and establishing the domains through a formal judgment of experts, (b) A two-round Delphi study with a panel of 15 experts to determine the relevant items, (c) Selection of items: A logistic regression model was developed that set ocular inflammatory activity as the dependent variable. The construct "uveitis inflammatory activity" was defined as any intraocular inflammation that included external structures (cornea) in addition to uvea.
View Article and Find Full Text PDFA 72-year-old man presented with signs on uveítis. Biopsy of an iris lesion established the diagnosis of uveal MALT lymphoma with presumed choroidal involvement.
View Article and Find Full Text PDFPurpose: To identify clinical outcomes and treatment patterns of intravitreal dexamethasone implant (Ozurdex; Allergan, Inc) in noninfectious uveitis in the clinical setting.
Design: Multicenter retrospective cohort study.
Methods: Eighty-two eyes (63 patients) receiving 142 implant injections over 35 months were included.