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      Conjunctival Chemosis and Annular Ciliochoroidal Detachments Detected by Anterior-Segment Optical Coherence Tomography in a Case of Systemic Lupus Erythematosus

      case-report

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          Abstract

          A 61-year-old Japanese woman presented to our hospital for treatment of systemic serositis associated with systemic lupus erythematosus (SLE). At the initial ophthalmologic examination, her best-corrected visual acuity was 1.2 and 0.6 in her right and left eyes, respectively. Slit-lamp examination showed marked chemosis in both eyes (OU). Swept source-based, anterior-segment optical coherence tomography (AS-OCT) clearly showed conjunctival elevations corresponding to the chemosis in all scan directions OU. In some scans, hyporeflective spaces with luminal structures corresponding to dilated lymphatic channels and nonluminal structures corresponding to interstitial fluid accumulation were seen clearly under the conjunctival epithelium and/or in the parenchyma. In all scan directions, the supraciliary space was seen clearly, suggesting the presence of an annular ciliochoroidal detachment. Fundus examinations showed retinal edema temporal to the optic nerve head and subfoveal serous retinal detachments OU. Ocular effusions resolved by 2 weeks after the start of steroid pulse therapy, and pleural effusions and ascites resolved and pericardial effusion decreased by 2 months. AS-OCT can be useful for understanding the mechanism(s) of the less common anterior-segment ocular manifestations of SLE.

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          Most cited references12

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          Systemic lupus erythematosus and ocular involvement: an overview.

          Rosanna Dammacco (2018)
          Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology and with remarkably heterogeneous clinical features. Virtually any organ system can be affected, including the eye. SLE-related eye involvement can be diagnosed in approximately one-third of the patients and is usually indicative of disease activity. An early diagnosis and the adoption of suitable therapeutic measures are necessary to prevent sight-threatening consequences, especially in patients with juvenile SLE. Periocular lesions, such as eyelid involvement and orbital inflammation, are relatively rare and, in case of orbital masses, may require a biopsy control. Keratoconjunctivitis sicca or secondary Sjögren's syndrome is the most frequent ophthalmic manifestation of SLE. According to its variable severity, lubricating tear drops may be sufficient in mild cases, whereas cyclosporine-A ophthalmic solution, glucocorticoids (GCs), methotrexate, and/or other immunosuppressive drugs may be required in the more severe cases. Partial occlusion of the lacrimal punctum by thermal cautery is rarely applied. Although uncommon, episcleritis and scleritis can sometimes be detected as an initial finding of SLE and reveal themselves as moderate to intense ocular pain, redness, blurred vision, and lacrimation. Unilateral or more often bilateral retinopathy is responsible for visual loss of variable severity and is ascribed to vasculitis of the retinal capillaries and arterioles. In addition to the combined treatment suitable for all patients with active SLE, intravitreal bevacizumab should be considered in cases of severe vaso-occlusive retinopathy and laser photocoagulation in cases of neovascularization. Purtscher-like retinopathy is likely ascribable to the formation of microemboli that results in retinal vascular occlusion and microvascular infarcts. Choroidal disease is characterized by monolateral or bilateral blurred vision. Because of the choroidal effusion, retinal detachment and secondary angle-closure glaucoma may occur. Ischemic optic neuropathy is characterized by acute-onset and progressive binocular visual impairment as a consequence of occlusion of the small vessels of the optic nerves due to immune complex vasculitis. Intravenous GC boluses followed by oral GCs and/or, in case of recurrence, intravenous cyclophosphamide and/or rituximab are commonly employed. Neovascularization can be treated by intravitreal bevacizumab and progression of retinal ischemic areas by retinal laser photocoagulation. Ocular adverse events (AE) have been described following the long-term administration of one or more of the drugs presently used for the treatment of SLE patients. Posterior subcapsular cataracts and secondary open-angle glaucoma are common AE of the prolonged GC administration. The long-term administration of hydroxychloroquine (HCQ) sulfate is well known to be associated with AE, such as vortex keratopathy and in particular the often irreversible and sight-threatening maculopathy. Length of administration > 5 years, > 1000 g total HCQ consumption, > 6.5 mg/kg daily dosing, coexistence of renal disease, and preexisting maculopathy are all considered risk factors for HCQ-induced retinopathy. Ocular AE of additional immunosuppressive and biological agents are still poorly known, given the worldwide more limited experience with their long-term use. A thorough ophthalmological control is strongly recommended at closer intervals for all SLE patients, in step with the total length of exposure to the drugs and the cumulative dose administered.
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            In vivo crystalline lens measurements with novel swept-source optical coherent tomography: an investigation on variability of measurement

            Takuhei Shoji, Naoko KATO, Sho Ishikawa … (2017)
            Objective To evaluate the reproducibility of in vivo crystalline lens measurements obtained by novel commercially available swept-source (SS) optical coherence tomography (OCT) specifically designed for anterior segment imaging. Methods and analysis One eye from each of 30 healthy subjects was randomly selected using the CASIA2 (Tomey, Nagoya, Japan) in two separate visits within a week. Each eye was imaged twice. After image scanning, the anterior and posterior lens curvatures and lens thickness were calculated automatically by the CASIA2 built-in program at 0 dioptre (D) (static), −1 D, −3 D and −5 D accommodative stress. The intraobserver and intervisit reproducibility coefficient (RC) and intraclass correlation coefficient (ICC) were calculated. Results The intraobserver and intervisit RCs ranged from 0.824 to 1.254 mm and 0.789 to 0.911 mm for anterior lens curvature, from 0.276 to 0.299 mm and 0.221 to 0.270 mm for posterior lens curvature and from 0.065 to 0.094 mm and 0.054 to 0.132 mm for lens thickness, respectively. The intraobserver and intervisit ICCs ranged from 0.831 to 0.865 and 0.828 to 0.914 for anterior lens curvature, from 0.832 to 0.898 and 0.840 to 0.933 for posterior lens curvature and from 0.980 to 0.992 and 0.942 to 0.995 for lens thickness. High ICC values were observed for each measurement regardless of accommodative stress. RCs in younger subjects tended to be larger than those in older subjects. Conclusions This novel anterior segment SS-OCT instrument produced reliable in vivo crystalline lens measurement with good repeatability and reproducibility regardless of accommodation stress.
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              Reproducibility and Agreement of Anterior Segment Parameter Measurements Obtained Using the CASIA2 and Spectralis OCT2 Optical Coherence Tomography Devices.

              Benjamin Y. Xu, Derek D Mai, Rafaella C. Penteado … (2017)
              To assess the reproducibility and agreement of measurement values obtained from the Tomey CASIA2 and Heidelberg Spectralis OCT2 anterior segment optical coherence tomographic devices.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Case Rep Ophthalmol
                Journal ID (iso-abbrev): Case Rep Ophthalmol
                Journal ID (publisher-id): COP
                Title: Case Reports in Ophthalmology
                Publisher: S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.com )
                ISSN (Electronic): 1663-2699
                Publication date Collection: Jan-Apr 2021
                Publication date (Electronic): 12 April 2021
                Publication date PMC-release: 12 April 2021
                Volume: 12
                Issue: 1
                Pages: 154-158
                Affiliations
                [1] aDepartment of Ophthalmology, Shimane University Faculty of Medicine, Izumo, Japan
                [2] bDepartment of Rheumatology, Shimane University Faculty of Medicine, Izumo, Japan
                Author notes
                Article
                Publisher ID: cop-0012-0154
                DOI: 10.1159/000514527
                PMC ID: 8077491
                SO-VID: f3e39ffc-4235-42b3-b616-0b4fa5403f7a
                Copyright © Copyright © 2021 by S. Karger AG, Basel
                License:

                This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.

                History
                Date received : 29 December 2020
                Date accepted : 17 January 2021
                Date: 2021
                Page count
                Figures: 2, References: 12, Pages: 5
                Categories
                Subject: Case Report

                Keywords: systemic lupus erythematosus,lymphangiectasia,ciliary detachment,choroidal effusion,anterior-segment optical coherence tomography
                Data availability:
                Keywords: systemic lupus erythematosus, lymphangiectasia, ciliary detachment, choroidal effusion, anterior-segment optical coherence tomography

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