Correlation of retinal and choroidal microvascular impairment in systemic sclerosis

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Abstract

Purpose

To investigate the correlation between retinal and choroidal microperfusion in patients with systemic sclerosis (SSc) using optical coherence tomography angiography (OCTA).

Methods

In this cross-sectional study SSc patients without clinical evidence of ocular involvement and healthy, age- and sex-matched volunteers were recruited. Participants underwent specific rheumatological and ophthalmological examinations, including optical coherence tomography (OCT) and OCTA. Retinal and choroidal thicknesses as well as perfusion of the retina and the choroidal sublayers were evaluated.

Results

A total of 15 SSc patients (30 eyes) with a median disease duration of 60 months and 15 matched, healthy controls (30 eyes) were recruited. OCT data revealed a significantly lower macular volume, as well as Sattler’s layer and Haller’s layer thickness in SSc patients compared to controls. In OCTA analysis, the perfusion of both retinal plexus as well as Sattler’s and Haller’s layer were significantly reduced in the SSc group. Patients with a disease duration of more than 60 months showed a statistically significant positive correlation between retinal and choroidal malperfusion, while those with a shorter disease duration did not.

Conclusion

OCTA analysis confirmed impairment of retinal and choroidal microperfusion in SSc patients, supporting the hypothesis of wide spreading vascular injury. In early stages, either the retinal or the choroidal perfusion seems to be involved, while later on, vascular impairment affects both tissues alike. Both, retinal and choroidal examinations should be considered as soon as the diagnosis of SSc is made, to avoid missing out on early alterations.

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Most cited references 37

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A Threshold Selection Method from Gray-Level Histograms

Nobuyuki Otsu (1979)

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Systemic sclerosis.

Yannick Allanore, Robert W Simms, Oliver Distler … (2015)

Systemic sclerosis is a complex autoimmune disease characterized by a chronic and frequently progressive course and by extensive patient-to-patient variability. Like other autoimmune diseases, systemic sclerosis occurs more frequently in women, with a peak of onset in the fifth decade of life. The exact cause of systemic sclerosis remains elusive but is likely to involve environmental factors in a genetically primed individual. Pathogenesis is dominated by vascular changes; evidence of autoimmunity with distinct autoantibodies and activation of both innate and adaptive immunity; and fibrosis of the skin and visceral organs that results in irreversible scarring and organ failure. Intractable progression of vascular and fibrotic organ damage accounts for the chronic morbidity and high mortality. Early and accurate diagnosis and classification might improve patient outcomes. Screening strategies facilitate timely recognition of life-threatening complications and initiation of targeted therapies to halt their progression. Effective treatments of organ-based complications are now within reach. Discovery of biomarkers - including autoantibodies that identify patient subsets at high risk for particular disease complications or rapid progression - is a research priority. Understanding the key pathogenetic pathways, cell types and mediators underlying disease manifestations opens the door for the development of targeted therapies with true disease-modifying potential. For an illustrated summary of this Primer, visit: http://go.nature.com/lchkcA.

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Retinal thickness decreases with age: an OCT study.

Duane J. Funk, B Alamouti (2003)

In three dimensional optic disc tomography a reference plane is required to calculate optic disc rim or cup values. The position of the reference plane often depends on the retinal thickness at the temporal disc margin. Originally it was assumed that the retinal thickness at the temporal disc margin is independent of age. The aim of the study was to check this hypothesis using optical coherence tomography, and additionally to determine the reproducibility of OCT measurements in this area. 100 eyes of 100 healthy volunteers were included in this study. Three OCT scans were performed on each eye. The scans were aligned vertically and placed at the temporal edge of the optic disc. For each eye, the thickness of the whole retina as well as the thickness of the retinal nerve fibre layer were calculated together with their coefficients of variation. Thereafter retinal thickness and nerve fibre layer thickness were correlated with age. The mean retinal thickness was 249 (SD 22) micro m. The mean nerve fibre layer thickness was 109 (22) micro m. The mean coefficients of variation were 3.5% (total retinal thickness) and 8.0% (nerve fibre layer thickness). Both the total retinal thickness and the nerve fibre layer thickness were significantly correlated with age (total retina: y = 269.5 - 0.53 x x; R(2) = 0.133; p = 0.0002, nerve fibre layer: y = 126.8 - 0.44 x x; R(2) = 0.094; p<0.0019. Using OCT scans the total retinal thickness can be calculated with high reproducibility (coefficient of variation = 3.5%). The reproducibility of nerve fibre layer thickness measurements is clearly lower (coefficient of variation = 8.0%). Both the total retinal thickness and the nerve fibre layer thickness significantly decrease with age. This influence of the age related decrease in RNFL/retinal thickness on the reference plane, however, is negligible.

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Author and article information

Contributors

Felix Rommel:

ORCID: http://orcid.org/0000-0003-2584-0607

felixrommel@gmail.com , felix.rommel@uksh.de

David Prangel: prangel.david@gmail.com

Michelle Prasuhn: michelle.prasuhn@uksh.de

Salvatore Grisanti: salvatore.grisanti@uksh.de

Mahdy Ranjbar: eye.research101@gmail.com

Journal

Journal ID (nlm-ta): Orphanet J Rare Dis

Journal ID (iso-abbrev): Orphanet J Rare Dis

Title: Orphanet Journal of Rare Diseases

Publisher: BioMed Central (London )

ISSN (Electronic): 1750-1172

Publication date (Electronic): 13 January 2021

Publication date PMC-release: 13 January 2021

Publication date Collection: 2021

Volume: 16

Electronic Location Identifier: 27

Affiliations

[1 ]GRID grid.4562.5, ISNI 0000 0001 0057 2672, Department of Ophthalmology, , University of Lübeck, ; Ratzeburger Allee 160, 23562 Lübeck, Germany

[2 ]GRID grid.4562.5, ISNI 0000 0001 0057 2672, Laboratory for Angiogenesis and Ocular Cell Transplantation, , University of Lübeck, ; Ratzeburger Allee 160, 23562 Lübeck, Germany

Author information

Felix Rommel http://orcid.org/0000-0003-2584-0607

Article

Publisher ID: 1649

DOI: 10.1186/s13023-020-01649-5

PMC ID: 7807887

PubMed ID: 33441156

SO-VID: b52a88a9-7767-49cb-a34a-e8bc3ef62780

License:

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

History

Date received : 23 March 2020

Date accepted : 17 December 2020

Funding

Funded by: Projekt DEAL

Open Access :

Open Access funding enabled and organized by Projekt DEAL.

Custom metadata

ScienceOpen disciplines: Infectious disease & Microbiology

Keywords: optical coherence tomography angiography,systemic sclerosis,retina,choroid,biomarker

Data availability:

ScienceOpen disciplines: Infectious disease & Microbiology

Keywords: optical coherence tomography angiography, systemic sclerosis, retina, choroid, biomarker

Correlation of retinal and choroidal microvascular impairment in systemic sclerosis

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Most cited references 37

A Threshold Selection Method from Gray-Level Histograms

Systemic sclerosis.

Retinal thickness decreases with age: an OCT study.

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