2023

Jump to: 2022, 2021

27 pages, 7400 KiB

Open AccessFeature PaperReview

by Priscilla Manni, Maria Carmela Saturno and Massimo Accorinti

J. Clin. Med. 2023, 12(19), 6242; https://doi.org/10.3390/jcm12196242 - 27 Sep 2023

Viewed by 1524

Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease affecting the eyes, ears, brain, skin, and hair. The Coronavirus Disease 2019 (COVID-19) is a new contagious infection that might trigger the onset of VKH disease, as previously proposed for other viruses. Moreover, after the [...] Read more.

Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease affecting the eyes, ears, brain, skin, and hair. The Coronavirus Disease 2019 (COVID-19) is a new contagious infection that might trigger the onset of VKH disease, as previously proposed for other viruses. Moreover, after the mass vaccination against SARS-CoV-2 worldwide, cases of VKH disease associated with COVID-19 vaccination have been reported. We present an overview of VKH and a comprehensive literature revision of all the VKH cases described after COVID-19 infection and vaccination, adding our experience. No differences have been found considering epidemiology and clinical findings of the disease compared to those reported in the no-COVID era. All of the patients promptly responded to systemic and local corticosteroid therapy with a good final visual prognosis. Different possible pathogenetic mechanisms underlying the onset of VKH after COVID-19 vaccination are discussed, while the presence of the HLA DR4 antigen as a genetic predisposition for the onset of the disease after COVID-19 infection and vaccination is proposed. VKH disease is one of the most frequently reported uveitic entities after COVID-19 vaccination, but a good response to therapy should not discourage vaccination. Nevertheless, ophthalmologists should be alerted to the possibility of VKH occurrence or relapse after COVID-19 vaccination, especially in genetically predisposed subjects. Full article

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9 pages, 263 KiB

Open AccessArticle

Systemic Disease Associations in a Cohort of Hispanic Patients with Scleritis

by Cristina Arruza, Guillermo A. Requejo Figueroa, Javier Colón, Estefania Ramirez Marquez, Guillermo Puebla, Daniela Ortega, Mariella Pappaterra Rodriguez, José López Fontanet, Alexandra Colón, Sofía C. Ayala Rodríguez, Erick Rivera Grana, Frances Marrero, Emilio Báez, Carmen Santos and Armando L. Oliver

J. Clin. Med. 2023, 12(5), 1969; https://doi.org/10.3390/jcm12051969 - 02 Mar 2023

Viewed by 1171

Abstract

(1) Purpose: A patient with scleritis may have an associated systemic disease, which is often autoimmunological and seldom infectious in origin. The data regarding such associations in Hispanic populations are scarce. Therefore, we evaluated the clinical characteristics and systemic-disease associations of a cohort [...] Read more.

(1) Purpose: A patient with scleritis may have an associated systemic disease, which is often autoimmunological and seldom infectious in origin. The data regarding such associations in Hispanic populations are scarce. Therefore, we evaluated the clinical characteristics and systemic-disease associations of a cohort of Hispanic patients with scleritis. (2) Methods: A retrospective review of the medical records (January 1990–July 2021) of two private uveitis practices in Puerto Rico was performed. Clinical characteristics and systemic-disease associations observed either at presentation or diagnosed as a consequence of the initial workup were recorded. (3) Results: A total of 178 eyes of 141 patients diagnosed with scleritis were identified. An associated autoimmune disease was present in 33.3% of the patients (rheumatoid arthritis, 22.7%; Sjögren’s syndrome, 3.5%; relapsing polychondritis, 2.8%; sarcoidosis, 1.4%; systemic lupus erythematosus, 1.4%; and systemic vasculitis, 0.7%). An associated infectious disease was present in 5.7% of the patients (2.13%, syphilis; 1.41%, herpes simplex; 1.14%, herpes zoster; and 0.71%, Lyme disease). One patient had all-trans retinoic-acid-associated scleritis. Statistical analysis revealed that patients with nodular anterior scleritis were less likely to have an associated immune-mediated disease (OR: 0.21; p = 0.011). (4) Conclusion: Rheumatoid arthritis was the most common systemic autoimmune disease association, while syphilis was the most common infectious disease associated with scleritis patients. Our study suggests that patients with nodular scleritis have a lower risk of having an associated immune-mediated disease. Full article

2022

Jump to: 2023, 2021

5 pages, 1204 KiB

Open AccessCase Report

Treatment of Various Ocular Manifestation of Systemic Lupus Erythematosus with Therapeutic Plasma Exchange

by Ji-Hye Lee, Ho-Wook Jeon, Su-Jin Moon and Mee-Yon Lee

J. Clin. Med. 2022, 11(22), 6632; https://doi.org/10.3390/jcm11226632 - 09 Nov 2022

Cited by 1 | Viewed by 1616

Abstract

The goal of this study is to describe a rare case of acute systemic lupus erythematosus (SLE) ocular involvement, followed by a rapid deterioration of the overall condition, and to then describe its successful treatment with therapeutic plasma exchange (TPE). In our case, [...] Read more.

The goal of this study is to describe a rare case of acute systemic lupus erythematosus (SLE) ocular involvement, followed by a rapid deterioration of the overall condition, and to then describe its successful treatment with therapeutic plasma exchange (TPE). In our case, a 21-year-old female, previously diagnosed with SLE, presented with a bilateral decreased vision for one week. Fundus examination and optical coherence tomography revealed subretinal fluid accumulation in both eyes and severe disc swelling with diffuse subretinal hemorrhages and perimacular whitening in the left eye. Despite systemic high-dose steroid therapy, the patient became worse, but immunosuppressive treatment was postponed due to fever and elevated serum leukocytes with the chance of systemic infection. She had undergone therapeutic plasma exchange (TPE) and was successfully treated. Preceding SLE ocular manifestation can be an indicator of the exacerbation of SLE, and TPE can be a treatment option for such progression. Full article

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8 pages, 3887 KiB

Open AccessPerspective

Assessing Patients with Alpha-1 Antitrypsin Deficiency for Corneal Refractive Surgery: A Review and Clinical Experience

by Majid Moshirfar, Neil Kelkar, Yasmyne C. Ronquillo and Phillip C. Hoopes

J. Clin. Med. 2022, 11(14), 4175; https://doi.org/10.3390/jcm11144175 - 19 Jul 2022

Cited by 1 | Viewed by 1525

Abstract

Alpha-1 Antitrypsin Deficiency (AATD) is an autosomal inheritable disorder that impairs the protease inhibitor alpha-1 antitrypsin. This disorder presents with various systemic effects, including liver cirrhosis, centrilobular emphysema, and ocular manifestations. Performing corneal refractive surgery in patients with AATD raises concerns regarding the [...] Read more.

Alpha-1 Antitrypsin Deficiency (AATD) is an autosomal inheritable disorder that impairs the protease inhibitor alpha-1 antitrypsin. This disorder presents with various systemic effects, including liver cirrhosis, centrilobular emphysema, and ocular manifestations. Performing corneal refractive surgery in patients with AATD raises concerns regarding the increased rates of corneal erosions, corneal ulcerations, potential developing descemetoceles, and other ocular manifestations. Patient outcomes for laser-assisted in situ keratomileuses (LASIK), photorefractive keratectomy (PRK), small incision lenticule extraction (SMILE), and other ocular corrective surgeries are lacking in this population. This article provides experiences performing corneal refractive surgery, discusses the current understanding of AATD, including its ocular manifestations, and explores factors to consider when evaluating patients for corneal procedures. The aim of this paper is to address the manifestations of AATD prior to performing corrective vision surgery. Full article

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15 pages, 4445 KiB

Open AccessArticle

Morphology and Vessel Density of the Macula in Preterm Children Using Optical Coherence Tomography Angiography

by Agnieszka Czeszyk, Wojciech Hautz, Maciej Jaworski, Dorota Bulsiewicz and Justyna Czech-Kowalska

J. Clin. Med. 2022, 11(5), 1337; https://doi.org/10.3390/jcm11051337 - 28 Feb 2022

Cited by 6 | Viewed by 1806

Abstract

Background: Retinal morphology changes may be associated with prematurity and can lead to visual impairment. Optical coherence tomography angiography may contribute to understanding the pathomechanism of structural and vascular retinal impairment in premature children. The aim of this study was to assess an [...] Read more.

Background: Retinal morphology changes may be associated with prematurity and can lead to visual impairment. Optical coherence tomography angiography may contribute to understanding the pathomechanism of structural and vascular retinal impairment in premature children. The aim of this study was to assess an influence of prematurity, neonatal clinical characteristics, and a history of retinopathy of prematurity (ROP) on the morphology and retinal vascularity of macula in children. Methods: A case–control study of 123 preterm children and 86 full-term children was performed. The age of the subjects was 10.45 years (IQR: 8.12–12.77), while the age of the control group was 11.78 years (IQR: 8.81–13.79). Optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA, angio-OCT) were performed using RTVueXR Avanti (Optovue, Fremont, CA, USA). Ganglion cell complex (GCC), foveal thickness (FT), parafoveal thickness (PFT), size of foveal avascular zone (FAZ) in superficial plexus, superficial capillary vessel density (sVD), deep capillary vessel density (dVD), central choroidal thickness (CCT), and presence of macular hypoplasia were analyzed. The association between OCT/angio-OCT results and clinical characteristics including the degree of ROP and therapy requirements was assessed in preterm infants. Results: Foveal morphology was affected in preterm children with high incidence of foveal hypoplasia (24.77%). GCC was thinner in preterm children compared to controls: avgGG 93 μm vs. 100 μm, p < 0.001. No associations between GCC and gestational age (R = −0.085; p = 0.228) and birth weight (R = −0.054; p = 0.446) were found. FAZ in preterm group was smaller than in controls (0.13 ± 0.09 vs. 0.22 ± 0.09; p < 0.001). FAZ area correlated with gestational age (R = 0.456; p < 0.001) and birth weight (R = 0.472; p < 0.001). Deep vessel density in the fovea was higher in preterm children than in control group (p < 0.001). PFT was significantly lower in preterm children compared to control group. However, increased thickness in the fovea was noted in preterm children (p < 0.001). FT was inversely correlated with gestational age (R = −0.562; p < 0.001) and birth weight (R = −0.508, p < 0.001). CCT was lower in preterm children (312 μm vs. 337.5 μm, p < 0.001) Parameters of GCC and FT were higher in patients with ROP required treatment compared to patients without ROP and spontaneously regressed retinopathy. FAZ was smaller in patients with retinopathy than in preterm children without ROP. Conclusion: Prematurity has a significant negative impact on GCC, macular morphology, and vascularization. In premature children, decreased FAZ, increased FT, and vessel density were strongly associated with gestational age, birth weight, Apgar score, ROP stage, and treatment requirement. Optical coherence tomography angiography is a useful tool for detecting retinal changes in premature children. Full article

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2021

Jump to: 2023, 2022

12 pages, 17518 KiB

Open AccessArticle

Subclinical Signs of Retinal Involvement in Hereditary Angioedema

by Paola Triggianese, Matteo Di Marino, Carolina Nesi, Elisabetta Greco, Stella Modica, Maria Sole Chimenti, Paola Conigliaro, Raffaele Mancino, Carlo Nucci and Massimo Cesareo

J. Clin. Med. 2021, 10(22), 5415; https://doi.org/10.3390/jcm10225415 - 19 Nov 2021

Cited by 2 | Viewed by 1399

Abstract

To explore retinal abnormalities using spectral domain optical coherence tomography (SD-OCT) and OCT-angiography (OCT-A) in a highly selective cohort of patients with type I hereditary angioedema (HAE). This prospective case-control study included 40 type I HAE patients and 40 age-/sex-matched healthy subjects (HC). [...] Read more.

To explore retinal abnormalities using spectral domain optical coherence tomography (SD-OCT) and OCT-angiography (OCT-A) in a highly selective cohort of patients with type I hereditary angioedema (HAE). This prospective case-control study included 40 type I HAE patients and 40 age-/sex-matched healthy subjects (HC). All participants underwent SD-OCT-scanning of retinal posterior pole (PP), peripapillary retinal nerve fiber layer (pRNFL), and optic nerve head (ONH). Superficial/deep capillary density was analyzed by OCT-A. A total of 80 eyes from 40 HAE and 40 eyes from HC were evaluated. The pRNFL was thicker in HAE than in HC in nasal superior (p < 0.0001) and temporal quadrants (p = 0.0005 left, p = 0.003 right). The ONH thickness in HAE patients was greater than in HC in the nasal (p = 0.008 left, p = 0.01 right), temporal (p = 0.0005 left, p = 0.003 right), temporal inferior (p = 0.007 left, p = 0.0008 right), and global (p = 0.005 left, p = 0.007 right) scans. Compared to HC, HAE showed a lower capillary density in both superficial (p = 0.001 left, p = 0.006 right) and deep (p = 0.008 left, p = 0.004 right) whole images, and superficial (p = 0.03 left) and deep parafoveal (p = 0.007 left, p = 0.005 right) areas. Our findings documented subclinical retinal abnormalities in type I HAE, supporting a potential role of the retinal assessment by SD-OCT/OCT-A as a useful tool in the comprehensive care of HAE patients. Full article

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17 pages, 4283 KiB

Open AccessReview

Ocular Manifestations of Behçet’s Disease: An Update on Diagnostic Challenges and Disease Management

by Hanna Zając and Anna Turno-Kręcicka

J. Clin. Med. 2021, 10(21), 5174; https://doi.org/10.3390/jcm10215174 - 05 Nov 2021

Cited by 11 | Viewed by 4777

Abstract

Behçet’s disease is a systemic vasculitis that affects multiple organs. The most common manifestations are oral and genital ulcerations and recurrent uveitis. Uveitis can be an initial symptom in 10–20% of cases and leads to blindness in 16–25% of patients. The management of [...] Read more.

Behçet’s disease is a systemic vasculitis that affects multiple organs. The most common manifestations are oral and genital ulcerations and recurrent uveitis. Uveitis can be an initial symptom in 10–20% of cases and leads to blindness in 16–25% of patients. The management of this disease is evolving due to the clinical phenotypes recently described in the literature and increasing focus on the detection of subclinical inflammation to enable correct therapeutic decisions. The first line treatment is azathioprine, followed by various immunosuppressive and biological agents as alternatives in severe or refractory cases. This review summarizes scientific articles about the etiology of, diagnostic tools for and treatment of the ocular manifestations of Behçet’s disease available in the PubMed database from 1 January 2016 to 1 May 2021. A multidisciplinary approach is necessary to effectively prevent permanent damage and thus improve the life quality of the patients. Therefore, it is crucial to raise awareness of the common clusters of symptoms, use of modern imaging methods, such as ocular computed tomography and fluorescein angiography, and novelty treatment algorithms to enable early diagnosis and appropriate management. Full article

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13 pages, 13940 KiB

Open AccessReview

Ocular Involvement in Relapsing Polychondritis

by Ken Fukuda, Tomoka Mizobuchi, Isana Nakajima, Tatsuma Kishimoto, Yusaku Miura and Yoshinori Taniguchi

J. Clin. Med. 2021, 10(21), 4970; https://doi.org/10.3390/jcm10214970 - 26 Oct 2021

Cited by 12 | Viewed by 3116

Abstract

Relapsing polychondritis (RPC) is a rare systemic immune-mediated disease characterized by recurrent inflammation of cartilaginous and proteoglycan-rich tissues throughout the body. Auricular, nasal, tracheal, and articular chondritis and arthritis are common systemic symptoms in patients with RPC. Ocular tissues are also targets of [...] Read more.

Relapsing polychondritis (RPC) is a rare systemic immune-mediated disease characterized by recurrent inflammation of cartilaginous and proteoglycan-rich tissues throughout the body. Auricular, nasal, tracheal, and articular chondritis and arthritis are common systemic symptoms in patients with RPC. Ocular tissues are also targets of inflammation in RPC, and a variety of ocular symptoms are observed in approximately half of the patients with RPC. Scleritis/episcleritis, uveitis, and conjunctivitis are common symptoms associated with RPC. Less frequently, keratitis, retinopathy, optic neuropathy, muscle palsy, and orbital inflammation are also observed. Ocular inflammation could also be the first manifestation of RPC. Although RPC is a potentially fatal and sight-threatening disease, the rarity of the disease and its protean clinical presentation may lead to delayed diagnosis or misdiagnosis. Given the high prevalence of ocular involvement in RPC, to avoid misdiagnosis, physicians should be suspicious of RPC when they see patients with recurrent ocular inflammatory conditions and various systemic symptoms. In this article, we provide a comprehensive review of ocular manifestations associated with RPC. Full article

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11 pages, 1368 KiB

Open AccessArticle

Correlation between Ultra-Wide-Field Retinal Imaging Findings and Vascular Supra-Aortic Changes in Takayasu Arteritis

by Barthelemy Poignet, Philippe Bonnin, Julien Gaudric, Ismael Chehaibou, Mathieu Vautier, Ramin Tadayoni, Alain Gaudric, Michel Paques, Bahram Bodaghi, David Saadoun and Sophie Bonnin

J. Clin. Med. 2021, 10(21), 4916; https://doi.org/10.3390/jcm10214916 - 24 Oct 2021

Cited by 4 | Viewed by 1812

Abstract

(1) Background: Takayasu arteritis (TA) is a chronic inflammatory large-vessel vasculitis. Ultra-wide-field imaging allows describing the retinal lesions in these patients and correlating them with vascular supra-aortic stenosis. (2) Methods: In total, 54 eyes of 27 patients diagnosed with TA were included, and [...] Read more.

(1) Background: Takayasu arteritis (TA) is a chronic inflammatory large-vessel vasculitis. Ultra-wide-field imaging allows describing the retinal lesions in these patients and correlating them with vascular supra-aortic stenosis. (2) Methods: In total, 54 eyes of 27 patients diagnosed with TA were included, and a complete ophthalmological examination was performed, including UWF color fundus photography (UWF-CFP), fluorescein angiography (UWF-FA), and computed tomography angiography measuring supra-aortic stenosis. Eleven patients underwent Doppler ultrasound imaging assessing the blood flow velocity (BFV) in the central retinal artery (CRA). (3) Results: Microaneurysms were detected in 18.5% of eyes on fundus examination, in 24.4% of eyes on UWF-CFP, and in 94.4% of eyes on UWF-FA. The number of microaneurysms significantly correlated with the presence of an ipsilateral supra-aortic stenosis (p = 0.026), the presence of hypertension (p = 0.0011), and the duration of the disease (p = 0.007). The number of microaneurysms per eye negatively correlated with the BFV in the CRA (r = −0.61; p = 0.003). (4) Conclusions: UWF-FA improved the assessment of TA-associated retinal findings. The significant correlation between the number of microaneurysms and the BFV in the CRA gives new insight to our understanding of Takayasu retinopathy. The total number of microaneurysms could be used as an interesting prognostic factor for TA. Full article

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9 pages, 1143 KiB

Open AccessArticle

Complex Rearrangement of the Entire Retinal Posterior Pole in Patients with Relapsing Remitting Multiple Sclerosis

by Alessio Martucci, Doriana Landi, Massimo Cesareo, Emiliano Di Carlo, Giovanni Di Mauro, Roberto Pietro Sorge, Maria Albanese, Carolina Gabri Nicoletti, Giorgia Mataluni, Nicola Biagio Mercuri, Matteo Di Marino, Francesco Aiello, Diego Centonze, Carlo Nucci, Girolama Alessandra Marfia and Raffaele Mancino

J. Clin. Med. 2021, 10(20), 4693; https://doi.org/10.3390/jcm10204693 - 13 Oct 2021

Cited by 2 | Viewed by 1567

Abstract

There are consolidated data about multiple sclerosis (MS)–dependent retinal neurodegeneration occurring in the optic disk and the macula, although it is unclear whether other retinal regions are affected. Our objective is to evaluate, for the first time, the involvement of the entire retinal [...] Read more.

There are consolidated data about multiple sclerosis (MS)–dependent retinal neurodegeneration occurring in the optic disk and the macula, although it is unclear whether other retinal regions are affected. Our objective is to evaluate, for the first time, the involvement of the entire retinal posterior pole in patients diagnosed with relapsing remitting multiple sclerosis (RRMS) unaffected by optic neuritis using Spectral Domain–Optical Coherence Tomography (SD–OCT). The study protocol was approved by Tor Vergata Hospital Institutional Ethics Committee (Approval number 107/16), and conforms to the tenets of the Declaration of Helsinki. After a comprehensive neurological and ophthalmological examination, 53 untreated RRMS patients (aged 37.4 ± 10) and 53 matched controls (aged 36.11 ± 12.94) were enrolled. In addition, each patient underwent an examination of the posterior pole using the SD-OCT built-in Spectralis posterior pole scanning protocol. After segmentation, the mean thickness, as well as the thickness of the 64 single regions of interest, were calculated for each retinal layer. No statistically significant difference in terms of average retinal thickness was found between the groups. However, MS patients showed both a significantly thinner ganglion cell layer (p < 0.001), and, although not statistically significant, a thinner inner nuclear layer (p = 0.072) and retinal nerve fiber layer (p = 0.074). In contrast, the retinal pigment epithelium (p = 0.014) and photoreceptor layers p < 0.001) resulted significantly thicker in these patients. Interestingly, the analysis of the region of interest showed that neurodegeneration was non-homogeneously distributed across each layer. This is the first report that suggests a complex rearrangement that affects, layer by layer, the entire retinal posterior pole of RRMS retinas in response to the underlying neurotoxic insult. Full article

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11 pages, 1250 KiB

Open AccessArticle

Changes in Eyelid Pressure and Dry Eye Status after Orbital Decompression in Thyroid Eye Disease

by Yasuhiro Takahashi, Aric Vaidya and Hirohiko Kakizaki

J. Clin. Med. 2021, 10(16), 3687; https://doi.org/10.3390/jcm10163687 - 20 Aug 2021

Cited by 7 | Viewed by 2341

Abstract

The aim of this prospective observational study was to examine changes in eyelid pressure and dry eye status after orbital decompression in thyroid eye disease (TED). In 16 patients (29 sides), upper eyelid pressure at plateau phase and maximum pressure were measured. TED [...] Read more.

The aim of this prospective observational study was to examine changes in eyelid pressure and dry eye status after orbital decompression in thyroid eye disease (TED). In 16 patients (29 sides), upper eyelid pressure at plateau phase and maximum pressure were measured. TED status was evaluated through the Hertel exophthalmometric value and margin reflex distance (MRD)-1 and 2. Dry eye status was quantified through corneal fluorescein staining, tear break-up time, Schirmer test I results, meibomian gland dysfunction (MGD), tear meniscus height, and superior limbic keratoconjunctivitis (SLK). Patients were classified into two groups: patients with decreased eyelid pressure (Group 1) and those with elevated pressure (Group 2). Consequently, neither the maximum upper eyelid pressure nor pressure at plateau phase significantly changed after surgery (p > 0.050). Some parameters about MGD improved after surgery, but the other parameters on dry eye, MGD, and SLK worsened or did not change. MRD-1 decreased more (p = 0.028), and the ratio of patients in whom SLK improved after surgery was larger in Group 1 (p = 0.030). These results indicate that upper eyelid pressure tends to decrease postoperatively in patients with a high upper eyelid position, resulting in improvement of SLK. Full article

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22 pages, 669 KiB

Open AccessReview

Ocular Complications of Obstructive Sleep Apnea

by Pei-Kang Liu, Tzu-Yu Chiu, Nan-Kai Wang, Sarah R. Levi and Ming-Ju Tsai

J. Clin. Med. 2021, 10(15), 3422; https://doi.org/10.3390/jcm10153422 - 31 Jul 2021

Cited by 11 | Viewed by 3945

Abstract

Obstructive sleep apnea (OSA), the most common form of sleep-disordered breathing, is characterized by repetitive episodes of paused breathing during sleep, which in turn induces transient nocturnal hypoxia and hypercapnia. The high prevalence of OSA and its associated health consequences place a heavy [...] Read more.

Obstructive sleep apnea (OSA), the most common form of sleep-disordered breathing, is characterized by repetitive episodes of paused breathing during sleep, which in turn induces transient nocturnal hypoxia and hypercapnia. The high prevalence of OSA and its associated health consequences place a heavy burden on the healthcare system. In particular, the consequent episodic oxygenic desaturation/reoxygenation series and arousals from sleep in patients with OSA have the potential to trigger oxidative stress, elevated systemic inflammatory responses, and autonomic dysfunction with sympathetic activation. Given these adverse side-effects, OSA is highly correlated to many eye diseases that are common in everyday ophthalmic practices. Some of these ocular consequences are reversible, but they may permanently threaten a patient’s vision if not treated appropriately. Here, this article seeks to review the ocular consequences and potential pathophysiologic associations in patients with OSA. Understanding these OSA-related eye diseases may help clinicians provide comprehensive care to their patients. Full article

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18 pages, 7477 KiB

Open AccessReview

Ocular and Systemic Manifestations in Paediatric Multisystem Inflammatory Syndrome Associated with COVID-19

by Tzu-Chen Lo and Yu-Yen Chen

J. Clin. Med. 2021, 10(13), 2953; https://doi.org/10.3390/jcm10132953 - 30 Jun 2021

Cited by 13 | Viewed by 2582

Abstract

This study aimed to achieve a better understanding of the epidemiological and clinical characteristics of multisystem inflammatory syndrome in children (MIS-C) following coronavirus disease 2019 (COVID-19). We searched PubMed and Embase between December 2019 and March 2021 and included only peer-reviewed clinical studies [...] Read more.

This study aimed to achieve a better understanding of the epidemiological and clinical characteristics of multisystem inflammatory syndrome in children (MIS-C) following coronavirus disease 2019 (COVID-19). We searched PubMed and Embase between December 2019 and March 2021 and included only peer-reviewed clinical studies or case series. The proportions of patients who had conjunctivitis, systemic symptoms/signs (s/s), Kawasaki disease (KD), and exposure history to suspected/confirmed COVID-19 cases were obtained. Moreover, positive rates of the nasopharyngeal real-time reverse transcriptase polymerase chain reaction (RT-PCR) and serum antibody for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) were recorded. Overall, 32 studies with 1458 patients were included in the pooled analysis. Around half of the patients had conjunctivitis. The five most common systemic manifestations were fever (96.4%), gastrointestinal s/s (76.7%), shock (61.5%), rash (57.1%), and neurological s/s (36.8%). Almost one-third presented complete KD and about half had exposure history to COVID-19 cases. The positivity of the serology (82.2%) was higher than that of the nasopharyngeal RT-PCR (37.0%). MIS-C associated with COVID-19 leads to several features similar to KD. Epidemiological and laboratory findings suggest that post-infective immune dysregulation may play a predominant role. Further studies are crucial to elucidate the underlying pathogenesis. Full article

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11 pages, 264 KiB

Open AccessReview

Optical Coherence Tomography Angiography as a New Tool for Evaluation of the Subclinical Retinal Involvement in Patients with Systemic Lupus Erythematosus—A Review

by Małgorzata Mimier-Janczak, Dorota Kaczmarek, Dawid Janczak and Radosław Kaczmarek

J. Clin. Med. 2021, 10(13), 2887; https://doi.org/10.3390/jcm10132887 - 29 Jun 2021

Cited by 9 | Viewed by 1699

Abstract

Knowing the proven relationship between lupus retinopathy and systemic changes and disease activity, it is crucial to find the possibility of early diagnosis of retinal changes at a subclinical level in order to provide faster medical intervention and protect the patient from irreversible [...] Read more.

Knowing the proven relationship between lupus retinopathy and systemic changes and disease activity, it is crucial to find the possibility of early diagnosis of retinal changes at a subclinical level in order to provide faster medical intervention and protect the patient from irreversible changes in the eye and other organs. The aim of this review is an analysis of studies investigating early pathological changes in retinal vascularization obtained by optical coherence tomography angiography (OCTA) and their relationship to the systemic lupus erythematosus (SLE). A literature search was performed to identify all relevant articles, regarding detection of subclinical retinal changes using OCTA in systemic lupus erythematosus listed in PubMed database. Seven out of seven papers found showed a decrease in superficial capillary plexus in ocular asymptomatic patients diagnosed with SLE. A decrease in retinal vessel density measured by OCTA may be a good marker of SLE activity and poor prognosis. OCTA in a safe manner can give clinicians a new perspective on processes of vessel remodeling and answer the question of how SLE might impact the eye from a structural point of view. Adding OCTA to the standard diagnostic process of SLE patients, may detect systemic changes early and prevent further visual deterioration by stopping progression of lupus retinopathy. Full article

12 pages, 602 KiB

Open AccessArticle

Globotrioasylsphingosine Levels and Optical Coherence Tomography Angiography in Fabry Disease Patients

by Maximilian Robert Justus Wiest, Mario Damiano Toro, Albina Nowak, Joel Baur, Katrin Fasler, Timothy Hamann, Mayss Al-Sheikh and Sandrine Anne Zweifel

J. Clin. Med. 2021, 10(5), 1093; https://doi.org/10.3390/jcm10051093 - 05 Mar 2021

Cited by 19 | Viewed by 1820

Abstract

Background: To date, there are no studies associating the dried blood spot (DBS) levels of globotrioasylsphingosine (lysoGb3) with quantitative optical coherence tomography angiography (OCTA) parameters in Fabry disease (FD) patients. Here, we aimed to investigate the association between OCTA vessel density (VD), vessel [...] Read more.

Background: To date, there are no studies associating the dried blood spot (DBS) levels of globotrioasylsphingosine (lysoGb3) with quantitative optical coherence tomography angiography (OCTA) parameters in Fabry disease (FD) patients. Here, we aimed to investigate the association between OCTA vessel density (VD), vessel length density (VLD) with DBS lysoGb3. Methods: A retrospective, single center analysis of all consecutive FD patients enrolled at the Department of Ophthalmology of the University Hospital of Zurich from 1 December 2017 to 9 September 2020. An association between VD and VLD detected by OCTA and lysoGb3 was investigated using a linear mixed model. Results: A total of 57 FD patients (23 male, 34 female; 109 eyes) were included. Forty-one patients suffered from the classic phenotype and 16 from the later-onset phenotype. LysoGb3 inversely correlated with VD and VLD in both the superficial (VD: p = 0.034; VLD: p = 0.02) and deep capillary plexus (VD: p = 0.017; VLD: p = 0.018) in the overall FD cohort. Conclusions: Our study shows an association between lysoGb3 and OCTA VD and VLD. This supports the hypothesis that quantitative OCTA parameters might be useful as diagnostic biomarkers for evaluating systemic involvement in FD, and possibly other diseases. Full article

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