Ocular involvement in systemic sclerosis: A systematic literature review, it's not all scleroderma that meets the eye

Elke O Kreps; Charlotte Carton; Maurizio Cutolo; Carlo Alberto Cutolo; Amber Vanhaecke; Bart P Leroy; Vanessa Smith

doi:10.1016/j.semarthrit.2018.12.007

Ocular involvement in systemic sclerosis: A systematic literature review, it's not all scleroderma that meets the eye

Semin Arthritis Rheum. 2019 Aug;49(1):119-125. doi: 10.1016/j.semarthrit.2018.12.007. Epub 2018 Dec 28.

Authors

Elke O Kreps¹, Charlotte Carton², Maurizio Cutolo³, Carlo Alberto Cutolo⁴, Amber Vanhaecke⁵, Bart P Leroy⁶, Vanessa Smith⁷

Affiliations

¹ Department of Ophthalmology, Ghent University Hospital, Corneel Heymanslaan 10, 9000 Ghent, Belgium. Electronic address: elke.kreps@ugent.be.
² Department of Internal Medicine, Ghent University, Corneel Heymanslaan 10, 9000 Ghent, Belgium. Electronic address: charlotte.carton@ugent.be.
³ Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Viale Benedetto XV, 616132 Genoa, Italy. Electronic address: mcutolo@unige.it.
⁴ Clinica Oculistica, DiNOGMI, University of Genoa, IRCCS Ospedale Policlinico San Martino, Viale Benedetto XV, 616132 Genoa, Italy. Electronic address: cacutolo@gmail.com.
⁵ Department of Internal Medicine, Ghent University, Corneel Heymanslaan 10, 9000 Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address: amber.vanhaecke@ugent.be.
⁶ Department of Ophthalmology & Center for Medical Genetics, Ghent University Hospital, Corneel Heymanslaan 10, 9000 Ghent, Belgium; Division of Ophthalmology & Center for Molecular & Cellular Therapeutics, Children's Hospital of Philadelphia, Philadelphia, PA, USA. Electronic address: bart.leroy@ugent.be.
⁷ Department of Internal Medicine, Ghent University, Corneel Heymanslaan 10, 9000 Ghent, Belgium; Department of Rheumatology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Corneel Heymanslaan 10, 9000 Ghent, Belgium. Electronic address: vanessa.smith@ugent.be.

PMID: 30660382
DOI: 10.1016/j.semarthrit.2018.12.007

Abstract

Objectives: Systemic sclerosis (SSc) is a rare and complex autoimmune disorder characterized by microvascular damage and progressive fibrosis which affects the skin and multiple other organs. Much of the published data concerning SSc and the eye consists of single case reports or small case studies. This systematic review aims to provide an overview of the current level of evidence for SSc-related ocular changes.

Materials and methods: A systematic literature review was conducted using 3 electronic databases, according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. A combination of following keywords was used: "Systemic Sclerosis" and ophthalmology-related search terms, including the keywords "Eye", "Ocular" and "Ophthalmic". All articles were screened by 2 independent reviewers at title, abstract and full text level. We solely included case-control studies that investigated specific ocular findings in SSc patients compared to healthy controls.

Results: Nine of 270 articles were retained. Dry eye symptoms are associated with SSc, whereas objective signs (Schirmer I testing) show conflicting results. There is insufficient evidence of SSc-related changes to the central corneal thickness. In terms of posterior segment involvement, choroidal vasculature appears to be affected to greater extent than the retinal microcirculation. However, the limited number of patients included in the studies renders it hazardous to draw overall conclusions.

Conclusions: There is a paucity of well-designed case-control studies investigating possible ocular involvement in SSc. Our systematic review demonstrates limited proven associations between SSc and ocular abnormalities, mainly in terms of dry eye symptoms and choroidal thickness. Future standardized prospective studies are needed to clarify the impact of the disease on the eye.

Keywords: Case-control studies; Eye; Nailfold videocapillaroscopy; Systematic literature review; Systemic sclerosis.

Publication types

Systematic Review

MeSH terms

Eye / pathology*
Eye Diseases / etiology*
Eye Diseases / pathology
Humans
Scleroderma, Systemic / complications*
Scleroderma, Systemic / pathology