Subclinical choroidal involvement in Systemic Lupus Erythematosus.

PURPOSE: To assess the choroidal status of Systemic Lupus Erythematosus (SLE) patients using Optical Coherence Tomography (OCT) and OCT-Angiography. METHODS: SLE patients with disease duration < 10 years, no disease activity and no ocular involvement were recruited and cross-sectionally evaluated. A demographically similar cohort of healthy subjects was used for comparison. The main outcome is choroidal vascularity index (CVI). As secondary outcomes, choriocapillaris parameters and choroidal thickness (CT) were evaluated. RESULTS: Forty eyes of 40 subjects (20 SLE patients and 20 healthy subjects) were studied with a mean ± SD age of 36.7 ± 9.9 years. In the SLE group, the mean ± SD duration of disease was 7.35 ± 2.21 years. Increased CVI was found in the SLE group (p = 0.022). Considering the choriocapillaris, SLE patients presented a lower number (p = 0.037) and a smaller total area (p = 0.041) of signal voids. No differences between groups were found in CT. For SLE patients, CT at subfoveal, temporal and inferior locations presented a negative moderate correlation with disease duration. A strong correlation between choriocapillaris parameters and age was demonstrated for both groups. CONCLUSIONS: This study provides evidence of subclinical choroidal changes in adult SLE patients with inactive disease and no overt ocular manifestation. Increased CVI and fewer and smaller flow voids in choriocapillaris with normal CT suggest increased choroidal vascularity in SLE.

Optic Neuritis and Vertebral Osteomyelitis: An Uncommon Presentation of Cat-Scratch Disease.

In cat-scratch disease (CSD), hematogenous spread may result in atypical presentations. Ocular manifestations develop in a minority of patients, with treatment being important in reducing long-term visual sequelae. Bone infection is rare. We present the case of a 52-year-old woman, with close contact with cats, reporting acute unilateral blurred vision and presenting papilledema, optic disc pallor, and peripapillary hemorrhage. Etiologic study of optic neuritis revealed an elevated positive Bartonella IgG; hence, treatment for CSD with doxycycline plus rifampin and corticosteroids was started. Concomitant lumbar pain of increasing intensity warranted magnetic resonance imaging, which revealed L3-L4 vertebral osteomyelitis with spondylodiscitis. Given the temporal link with CSD diagnosis and the significant clinical improvement since its treatment was started, an etiologic link was presumed and antibiotics were prolonged. This case stands out for the presence of distinct atypical CSD manifestations in the same patient. Further studies are needed to determine the optimal treatment for rare manifestations, particularly bone infection.

Design and Development of a Web-Based Prospective Nationwide Registry for Ocular Inflammatory Diseases: UVEITE.PT - The Portuguese Ocular Inflammation Registry.

Uveitis is a heterogeneous collection of infrequent diseases, which poses significant challenges to cost-effective research in the field. Medical registries are being increasingly recognized as crucial tools to provide high-quality data, thus enabling prospective clinical research. This paper describes the design and technical structure development of an innovative countrywide electronic medical record for uveitis, Uveite.pt, and gives an overview of the cohort registered since its foundation, March 2020.Uveite.pt is an electronic medical record platform developed by the Portuguese Ocular Inflammation Group (POIG), a scientific committee of the Portuguese Ophthalmology Society. This is a nationwide customized web-based platform for uveitis patients useful for both clinical practice and real-world-based research, working as a central repository and reporting tool for uveitis. This paper describes the technical principles, the design and the development of a web-based interoperable registry for uveitis in Portugal and provides an overview of more than 400 patients registered in the first 18 months since inception.In infrequent diseases, the existence of registries enables to gather evidence and increase research possibilities to clinicians. The adoption of this platform enables standardization and improvement of clinical practice in uveitis. It is useful to apprehend the repercussion of medical and surgical treatments in uveitis and scleritis, supporting clinicians in the strict monitoring of drug adverse reactions and surgical outcomes.

Detection of subclinical microvascular changes in systemic lupus erythematous using optical coherence tomography angiography: A systematic review and meta-analysis.

Systemic lupus erythematosus (SLE) is a chronic, systemic, autoimmune connective tissue disease that affects several vascular territories. We sought to assess the role of optical coherence tomography angiography in detecting subclinical microvascular alterations in SLE patients. PubMed, Scopus, and Web of Science databases were systematically searched until January 21, 2023. Studies using optical coherence tomography angiography as a primary diagnostic method to evaluate the macular microvasculature of SLE patients versus healthy controls were included. Primary outcomes were macular vessel density and foveal zone parameters. A meta-analysis was performed using a random-effects model. Of 301 screened abstracts, 15 were found eligible, enrolling 1,246 eyes from 1,013 patients. SLE patients presented a reduction of macular vessel density at both plexuses in all zones (whole scan, fovea, parafovea, and perifovea), and of foveal density compared with healthy controls. No differences were found at foveal avascular zone parameters. SLE patients presented a reduction of macular vessel density without signs or symptoms of SLE ocular involvement. Optical coherence tomography angiography application for the assessment of subclinical microvascular changes needs to be elucidated with longitudinal studies.

Comparison of Color Fundus Photography and Multicolor Fundus Imaging for Detection of Lesions in Diabetic Retinopathy and Retinal Vein Occlusion.

Purpose: To evaluate the agreement between conventional fundus photography (CFP) and multicolor fundus imaging (MFI) for the detection of lesions of diabetic retinopathy (DR) and retinal vein occlusion (RVO). Methods: Cross-sectional analysis of eyes with DR or RVO who underwent CFP and MFI. All images were independently analyzed by two observers (O1 and O2), and the evaluated lesions were classified as "present" or "absent". Then, a paired comparison between both exams of the same eye was performed, to assess which made it easier to detect the lesions. Results: Considering DR, the agreement was substantial for cotton wool spots and photocoagulation scars for both observers (O1: κ=0.75 and κ=0.67; O2: κ=0.71 and κ=0.64, respectively) and for hard exudates for O1 (κ=0.80). These lesions were detected more frequently on MFI. Regarding RVO, the agreement was considered substantial for venous sheathing by O1 (κ=0.64) and moderate for optociliary shunts by O2 (κ=0.60). Optociliary shunts were detected more frequently in CPF by both observers and venous sheathing on MFI by O1. For microaneurysms, retinal hemorrhages, retinal neovascularization, and proliferative membranes, in DR, and retinal hemorrhages, venous engorgement, and retinal neovascularization in RVO, the agreement was almost perfect (κ>0.82). In the paired analysis, both observers considered that, in DR, microaneurysms and retinal hemorrhages were easier to detect on CFP and that retinal neovascularization, cotton wool spots, and photocoagulation scars were easier to identify on MFI. Regarding RVO, optocilliary shunts were easier to identify on CFP and venous engorgement on MFI. Conclusion: The agreement of MFI and CFP was substantial to almost perfect for most lesions. MFI seems better to detect cotton wool spots and photocoagulations scars in DR and venous sheathing in RVO. Optocilliary shunts seem easier to detect on CFP.

Retinal Changes After COVID-19 Infection and COVID-19 Vaccination.

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) disease was first reported in 2019 and was initially associated with respiratory pathology. With the improvement of knowledge about this disease, it was noticed that, among other symptoms, some patients presented visual acuity changes associated with retinal vascular changes, mainly associated with thrombotic phenomena. Later, with the development of vaccines against SARS-CoV-2 disease, cases of visual acuity alterations secondary to thrombotic phenomena were also reported. Case Presentation: In this article, a series of clinical cases with retinal vascular alterations after COVID-19 infection and vaccination are described. Conclusion: COVID-19 infection and vaccination increase the risk of retinal vascular events. The purpose of this article is to present a set of clinical cases with various manifestations of vascular changes in the retina associated with COVID-19 infection and COVID-19 vaccination observed in the Department of Ophthalmology of Centro Hospitalar Universitário de Santo António, in Porto, Portugal.

Application of optical coherence tomography angiography for microvascular changes in patients treated with hydroxychloroquine: a systematic review and meta-analysis.

BACKGROUND: Retinal toxicity with long-term hydroxychloroquine (HCQ) treatment is a major concern. This systematic review aims to assess the application of optical coherence tomography angiography (OCTA) to detect microvascular alterations in patients under HCQ. METHODS: PubMed, Scopus, Web of Science, and Cochrane Library databases were systematically searched until January 14, 2023. Studies using OCTA as a primary diagnostic method to evaluate the macular microvasculature of HCQ users were included. Primary outcomes were macular vessel density (VD) and foveal avascular zone (FAZ) at the superficial (SCP) and deep (DCP) capillary plexus. Meta-analysis was performed using a random-effects model. RESULTS: Of 211 screened abstracts, 13 were found eligible, enrolling 989 eyes from 778 patients. High-risk patients due to longer duration of treatment presented lower VD in the retinal microvasculature than those with low-risk in SCP (P = 0.02 in fovea; P = 0.004 in parafovea) and in DCP (P = 0.007 in fovea; P = 0.01 in parafovea). When compared with healthy controls, HCQ users had lower VD in both plexus-no quantitative synthesis was presented. CONCLUSIONS: Microvascular changes were found in autoimmune patients under HCQ treatment without any documented retinopathy. However, the evidence produced so far does not allow to draw conclusion concerning the effect of drug as studies were not controlled for disease duration.

Posterior Capsular Rupture during Cataract Surgery in Eyes Previously Treated with Intravitreal Injections.

INTRODUCTION: Intravitreal injections (IVIs) are currently the most common intraocular surgical procedure worldwide. Some studies have reported a higher risk of intraoperative complications, namely, posterior capsular rupture (PCR), during cataract surgery. The aim of this retrospective and observational study, conducted at Department of Ophthalmology, Centro Hospitalar Universitário do Porto, was to assess the risk of PCR during cataract surgery in eyes previously treated with IVIs with anti-vascular endothelial growth factor (anti-VEGF) and/or corticosteroids. METHODS: Eyes undergoing cataract surgery between June 2019 and May 2021 were included. Combined surgeries, such as glaucoma surgery and pars plana vitrectomy, were excluded. The occurrence of PCR during cataract surgery in treated and previously untreated eyes with IVI was analyzed. RESULTS: A total of 5,813 cataract surgeries were analyzed; 4.1% of the cases had previously undergone IVI. The PCR rate in cataract surgery was 1.8%: 6.7% in eyes previously treated with IVI and 1.6% without previous IVI (OR = 4.5, 95% CI: 2.6-7.7, p < 0.001). The combined therapy (anti-VEGF with corticosteroids) presents a higher risk compared to the two therapies alone as monotherapy (OR = 11.6, 95% CI: 4.7-28.5, p < 0.001), as well as treated eyes treated with ≥10 IVI (OR = 2.1, 95% CI: 0.8-6.1, p = 0.144) and a time interval between the last IVI and cataract surgery was ≤6 months (OR = 1.9, 95% CI: 0.6-6.1, p = 0.296). CONCLUSION: These results demonstrate that eyes that require IV treatment prior to cataract surgery are at increased risk of CPA during cataract surgery, and careful assessment of the characteristics of the cataract and posterior capsule is critical.

Long-Term Bilateral Poorly Treated Acute Syphilitic Posterior Placoid Chorioretinitis Complicated with Choroidal Neovascularization: A Case Report.

Purpose: To describe a case of long-term poorly treated bilateral acute syphilitic posterior placoid chorioretinitis (ASPPC) complicated with choroidal neovascularization, with a multimodal imaging approach. Patients and Methods: Retrospective case report. Analysis of patient's clinical data. Case Report: A healthy 66-year-old man complained of decreased visual acuity in the right eye (RE). He had a past history of bilateral exudative maculopathy of unknown etiology, lasting for more than 10 years and leading to severe and irreversible vision loss on the left eye. The corrected distance visual acuity (CDVA) was 20/63 on the RE and <20/400 on the left eye (LE). On slit-lamp, no anterior chamber reaction was observed, a vitreous haze was present on the RE as well as large, bilateral yellowish lesions in the macula, with exuberant macular atrophy, particularly on the LE. These well-defined lesions were confined to the posterior pole. The spectral domain optical coherence tomography (SD-OCT) showed a flat retinal pigmented epithelium (RPE) detachment with hyperreflective material beneath the RPE and cystoid macular edema on the RE and an exuberant macular atrophy on the LE, with both eyes showing anomalous enlargement of choroidal vessels. The fluorescein angiography showed general hypofluorescence and indocyanine green angiography revealed a heterogeneous pattern. The OCT angiography (OCTA) of the RE demonstrated an anomalous vascular network related to the presence of choroidal neovascularization (CNV). An etiological study was performed and a positive treponemal test (TPHA) was found. The patient experienced visual acuity improvement to 20/25 after systemic treatment with Penicillin and anti-VEGF injections. Conclusion: Clinical data and multimodal imaging information suggest that this case represents a complication of long-term untreated bilateral acute syphilitic posterior placoid chorioretinitis (ASPPC) with associated choroidal neovascularization. Once the RE presented signs of active disease, besides intravitreal anti-VEGF, treatment of the underlying disease was essential for visual recovery.

Long-Term Effect of Anti-Vascular Endothelial Growth Factor (Anti-VEGF) Injections in Choroidal Neovascularization Secondary to Angioid Streaks.

Purpose: This study aimed to evaluate the long-term effectiveness of intravitreal anti-vascular endothelial growth factor (VEGF) injections in the treatment of choroidal neovascularization (CNV) associated with angioid streaks. Methods: Multicenter retrospective cohort study, including eyes with CNV secondary to angioid streaks treated with anti-VEGF injections, were performed. Best-corrected visual acuity (BCVA) in ETDRS letters; qualitative and quantitative (foveal thickness) OCT parameters; anti-VEGF type; and number of injections were collected at baseline and at 3, 6, 12, 24, 36, 48, 60, and 72 months. Results: Thirty-nine eyes from 29 patients, 17 (58.6%) females, were included. The mean follow-up time was 69.4 ± 34.5 months. BCVA was 59.3 ± 23.3 letters at baseline and 63.7 ± 21.9 letters at 48 months. At 3 months, BCVA improved 6.9 ± 11.7 letters (P=0.003). Then, BCVA remained stable. The mean foveal thickness decreased from 343.3 ± 120.2 µm at baseline to 268.3 ± 65.4 at 48 months (P=0.021). The mean number of injections was 4.6 ± 2.1 at 12 months, decreasing to 1.7 ± 2.4 injections between 36 and 48 months (P=0.093). Conclusion: This real-world study suggests that the functional and morphologic response to anti-VEGF therapy for CNV related to angioid streaks is generally satisfactory and maintained in the long term.

The 2021 Portuguese Society of Ophthalmology joint guidelines with Paediatric Rheumatology on the screening, monitoring and medical treatment of juvenile idiopathic arthritis-associated uveitis.

AIM: To develop the first Ophthalmology joint guidelines with Paediatric Rheumatology with recommendations on the screening, monitoring and medical treatment of juvenile idiopathic arthritis-associated uveitis (JIA-U), endorsed by the Portuguese Society of Ophthalmology (SPO). METHODS: A systematic literature review was conducted to include publications up to July 14th 2020, with no language restrictions, in order to include all the international position papers/guidelines concerning the medical management of JIA-U and randomised clinical trials assessing the efficacy and safety of medical treatment in this field. We searched through MEDLINE (PubMed), Scopus, Web of Science and Cochrane Library. The Delphi modified technique to generate consensus was used. Preliminary evidence statements were subject to an anonymous agreement assessment and discussion process using an online survey, followed by further discussion and update at a national meeting. A draft of the manuscript with all recommendations was then circulated among all participants and suggestions were incorporated. The final version was again circulated before publication. RESULTS: Twenty-six recommendations were developed focusing on the following topics: general management (3), screening and follow-up of uveitis (4), treatment (17) and health education in JIA-U among patients and families (2). CONCLUSION: These guidelines were designed to support the shared medical management of patients with JIA-U and emphasize the need for a multidisciplinary approach between Ophthalmology and Paediatric Rheumatology regarding the comprehensive care of JIA-U. We acknowledge that updating these recommendations will be warranted in the future, as more evidence becomes available. KEY-WORDS: juvenile idiopathic arthritis, uveitis, biological treatment, conventional immunosuppressive treatment, multidisciplinary management, guidelines, consensus, review, Delphi Technique.

Choroidal neovascularization secondary to pathological myopia-macular Bruch membrane defects as prognostic factor to anti-VEGF treatment.

PURPOSE: To evaluate the prevalence and visual outcomes of macular Bruch membrane (BM) defects in patients treated with anti-vascular endothelial growth factors (VEGF) for choroidal neovascularization secondary to pathological myopia (mCNV). METHODS: Single-center retrospective observational case series of 68 eyes from 62 patients with mCNV treated with one anti-VEGF injection followed by a pro re nata (1 + PRN) regimen. A minimum follow-up of 6 months was defined. Chorioretinal atrophy was assessed by fundus examination, fluorescein angiography, and SD-OCT. RESULTS: Median follow-up was 28.5 (range 6-89) months with a median number of 5 anti-VEGF injections. At baseline, 27.9% of eyes had macular BM defects increasing to 36.8% during follow-up (p<0.001). Eyes without macular BM defects at the baseline had higher BCVA at the last observation than patients with BM defects (p=0.003). An increase of 5 or more ETDRS letters was more frequent in eyes without BM defects (p=0.001). At the end of follow-up, mCNV-related macular atrophy was present in 44.1%; out of which, 83.3% presented macular BM defects (p<0.001). Eyes with mCNV-related macular atrophy without BM defects had a significant increase of best-corrected visual acuity compared with eyes with mCNV-related macular atrophy and BM defect (p=0.002). CONCLUSIONS: Macular Bruch membrane defects are often seen in mCNV and have a significant impact in visual acuity and prognosis. Eyes with macular BM defects have a poorer response and worse visual outcomes after anti-VEGF therapy.

Macular edema secondary to retinal vein occlusion in a real-life setting: a multicenter, nationwide, 3-year follow-up study.

PURPOSE: To evaluate the presence of macular edema secondary to retinal vein occlusion (RVO)-both central retinal vein occlusion (CRVO) and branch retinal vein occlusion (BRVO)-3 years after diagnosis in patients who underwent intravitreal therapy and to identify potential prognostic factors and biomarkers of persistent macular edema. METHODS: National multicenter, observational, exploratory, retrospective cohort study of 104 consecutive patients with macular edema secondary to RVO diagnosed from January 2014 to December 2015 with minimum 3-year follow-up time. Data analyzed included best-corrected visual acuity (BCVA), clinical and demographic data, and spectral domain optical coherence tomography parameters. RESULTS: At final observation, median baseline central retinal thickness significantly improved from baseline 538 to 290 µm (p < 0.001) and complete macular edema resolution was achieved in 51.0% of patients (56.3% and 42.5% in BRVO and CRVO patients, respectively). BCVA also improved (p < 0.01). Logistic regression analysis revealed a relationship between recurrence of macular edema and disorganization of retinal inner layers (DRIL) at baseline (odds ratio = 2.88; p = 0.013). CONCLUSION: Good long-term anatomical and functional outcomes are achieved with intravitreal treatments in RVO patients. Anatomical success and visual gains seen in the first year were maintained throughout the entire follow-up, though DRIL is a major risk factor for recurrence.

Serum vitamin D and age-related macular degeneration: Systematic review and meta-analysis.

Vitamin D may be implicated in the pathophysiology of several ocular diseases, but its role in age-related macular degeneration (AMD) remains uncertain. We sought to review systematically the existing evidence to evaluate the association between serum 25-hydroxyvitamin D 25(OH)D levels and AMD. A four-database search (PubMed, ISI Web of Science, Cochrane, and Scopus) was performed from inception to May 2020 using the MeSH terms: ("Macular Degeneration" OR "Age-related macular degeneration" OR "Retinal degeneration" OR "Macula lutea") AND ("Vitamin D" OR "Ergocalciferols" OR "Cholecalciferol" OR "25-Hydroxyvitamin D"). Random-effects meta-analyses were performed to compute 1) the standard mean difference in 25(OH)D concentration between AMD and non-AMD patients and 2) the AMD risk according to serum 25(OH)D levels. Eighteen observational studies enrolling 75,294 patients after a selection process among 375 original abstracts were selected. No significant differences were found, but there appears to exist a trend for late AMD among subjects with a serum 25(OH)D level below 50 nmol/L (odds ratio, 1.8; 95% confidence interval: 1.00-3.24, P = 0.05). There is no clear evidence of a definitive association between serum 25(OH)D and AMD risk, mainly due to heterogeneity in study procedures and lack of longitudinal designs.

Optical Coherence Tomography Findings (SD-OCT and OCTA) in Early-Stage Type 3 Neovascularization.

A 76-year-old male presented with a small hyperreflective density in the outer nuclear layer with subtle retinal pigment epithelium (RPE) elevation and few intraretinal cysts on spectral-domain optical coherence tomography (SD-OCT). Optical coherence tomography angiography (OCTA) confirmed the presence of a tuft-shaped intraretinal neovascular lesion. SD-OCT performed 2 months before showed a smaller RPE elevation at the same location without intraretinal fluid. A 79-year-old male presented with a small hyperreflective density in the outer retina surrounded by scant intraretinal fluid on SD-OCT and a bright vessel on OCTA, suggesting early-stage type 3 neovascularization. SD-OCT performed 2 months before showed a smaller hyperreflectivity at the same location, without intraretinal fluid. An 84-year-old female presented with hyperreflective foci in the outer retina overlying a serous pigment epithelium detachment (PED) with focal RPE disruption on SD-OCT. SD-OCT performed 2 months before showed the same hyperreflective lesion associated with a shallower PED. No neovascular lesions were found on OCTA after six injections of bevacizumab. To conclude, careful evaluation of SD-OCT allows for early detection of type 3 neovascularization at a pre-exudative stage. OCTA may be useful in confirming the presence of intraretinal neovascular lesion and monitoring response to anti-vascular endothelial growth factor agents.

Purtscher-like retinopathy.

Purtscher-like retinopathy is associated with retinal hemorrhages and ischaemia probably due to the complement-mediated leukoembolization. It is a rare and severe angiopathy found in conditions such as acute pancreatitis. Case. We present a case of a 53-year-old man who presented with a Purtscher-like retinopathy associated with the development of acute pancreatitis in the context of a Klatskin tumour (a hilar cholangiocarcinoma). The ophthalmologic evaluation revealed the best corrected visual acuity (BCVA) of 20/32 in the right eye (RE) and of 20/40 in the left eye (LE); biomicroscopy of anterior segment showed scleral icterus and fundoscopy revealed peripapillary cotton-wool spots, optic disc edema, and RPE hypo- and hyperpigmentation in the middle peripheral retina in both eyes with an intraretinal hemorrhage in the LE. 15 months after the initial presentation, without ophthalmological treatment, there was an improvement of BCVA to 20/20 in both eyes and optical coherence tomography (OCT) revealed areas of reduction of retinal nerve fiber layer thickness corresponding to the previous cotton-wool spots. Conclusion. Purtscher-like retinopathy should not be neglected in complex clinical contexts. Its unclear pathophysiology determines an uncertain treatment strategy, but a meticulous follow-up is compulsory in order to avoid its severe complications.

Bilateral proliferative retinopathy as the initial presentation of chronic myeloid leukemia.

The authors report a rare case of a 48-year-old male with chronic myeloid leukemia (CML) who initially presented with a bilateral proliferative retinopathy. The patient complained of recent visual loss and floaters in both eyes (BE). Ophthalmologic evaluation revealed a best corrected visual acuity (BCVA) of 20/50 in the right eye and 20/200 in the left eye (LE). Fundoscopy showed the presence of bilateral peripheral capillary dropout with multiple retinal sea fan neovascularisations, which were confirmed on fluorescein angiography. Full blood count revealed hyperleukocytosis, thrombocytosis, anemia, and hyperuricemia. Bone marrow aspiration and biopsy showed the reciprocal chromosomal translocation t (9;22), diagnostic of CML. The patient was started on hydroxyurea, allopurinol and imatinib mesylate. He received bilateral panretinal laser photocoagulation and a vitrectomy was performed in the LE. The patient has been in complete hematologic, cytogenetic, and major molecular remission while on imatinib and his BCVA is 20/25 in BE.