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      Ocular manifestations in patients with systemic sclerosis

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          Abstract

          Systemic sclerosis (SSc) is a rare, chronic autoimmune disease with unknown etiology. Its prominent features are fibrosis, vasculopathy and impaired immune response. Disease can also affect eyes leading to various findings in ophthalmological examination.

          The objective of this study was to determine the prevalence and type of ocular involvement in patients with SSc. A systematic literature review was conducted using electronic databases. A combination of following keywords was used: “systemic sclerosis” and ophthalmology-related search terms, including the keywords “eye”, “ocular” and “ophthalmic”.

          In conclusion, eyelid and conjunctival abnormalities and dry eye disease are among the most common ocular manifestations of SSc. Their diversity is connected to complexity of the disease.

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          Systemic sclerosis.

          Christopher P. Denton, Dinesh Khanna (2017)
          Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Additionally, treatments for specific complications have emerged and a growing evidence base supports the use of immune suppression for the treatment of skin and lung fibrosis. Some manifestations of the disease, such as scleroderma renal crisis, pulmonary arterial hypertension, digital ulceration, and gastro-oesophageal reflux, are now treatable. However, the burden of non-lethal complications associated with systemic sclerosis is substantial and is likely to become more of a challenge. Here, we review the clinical features of systemic sclerosis and describe the best practice approaches for its management. Furthermore, we identify future areas for development.
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            Fibrosis--a lethal component of systemic sclerosis.

            Yuen Yee Ho, David Lagares, Andrew Tager … (2014)
            Fibrosis is a pathological process characterized by excessive accumulation of connective tissue components in an organ or tissue. Fibrosis is produced by deregulated wound healing in response to chronic tissue injury or chronic inflammation, the hallmarks of rheumatic diseases. Progressive fibrosis, which distorts tissue architecture and results in progressive loss of organ function, is now recognized to be one of the major causes of morbidity and mortality in individuals with one of the most lethal rheumatic disease, systemic sclerosis (SSc). In this Review, we discuss the pathological role of fibrosis in SSc. We discuss the involvement of endothelium and pericyte activation, aberrant immune responses, endoplasmic reticulum stress and chronic tissue injury in the initiation of fibrosis in SSc. We then discuss fibroblast activation and myofibroblast differentiation that occurs in response to these initiating processes and is responsible for excessive accumulation of extracellular matrix. Finally, we discuss the chemical and mechanical signals that drive fibroblast activation and myofibroblast differentiation, which could serve as targets for new therapies for fibrosis in SSc.
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              Ocular immune privilege: therapeutic opportunities from an experiment of nature.

              J Wayne Streilein (2003)
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                Author and article information

                Journal
                Journal ID (nlm-ta): Reumatologia
                Journal ID (iso-abbrev): Reumatologia
                Journal ID (publisher-id): RU
                Title: Reumatologia
                Publisher: Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie
                ISSN (Print): 0034-6233
                ISSN (Electronic): 2084-9834
                Publication date (Electronic): 23 December 2020
                Publication date (Print): 2020
                Volume: 58
                Issue: 6
                Pages: 401-406
                Affiliations
                [1 ]Department of Ophthalmology, Professor K. Gibinski University Clinical Center of the Medical University of Silesia, Katowice, Poland
                [2 ]Department of Ophthalmology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Katowice, Poland
                [3 ]Department of Internal Medicine, Rheumatology and Clinical Immunology, Medical University of Silesia, Katowice, Poland
                Author notes
                Address for correspondence: Małgorzata Kozikowska, Department of Ophthalmology, Professor K. Gibinski University Clinical Center of the Medical University of Silesia, 35 Ceglana St., 40-514 Katowice, Poland. e-mail: kozikowskamalg@ 123456gmail.com
                Article
                Publisher ID: 42774
                DOI: 10.5114/reum.2020.102004
                PMC ID: 7792544
                PubMed ID: 33456083
                SO-VID: 928ef900-d690-43d7-b3ae-3a18be44c2fe
                Copyright © Copyright: © 2020 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License, allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.

                History
                Date received : 15 June 2020
                Date accepted : 24 November 2020
                Categories
                Subject: Review Paper

                Keywords: systemic sclerosis,dry eye disease,ocular manifestations,eyelid changes
                Data availability:
                Keywords: systemic sclerosis, dry eye disease, ocular manifestations, eyelid changes

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