Altered ocular microvasculature in patients with systemic sclerosis and very early disease of systemic sclerosis using optical coherence tomography angiography

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Abstract

The vascular hypothesis of systemic sclerosis (SSc) would predict microvascular alterations should also affect anatomical regions like ocular microvasculature. The objective of this study was to evaluate retinal and choriocapillary vessel density (VD) in patients with definite SSc and very early disease of systemic sclerosis (VEDOSS) using optical coherence tomography angiography (OCTA). 22 eyes of 22 patients and 22 eyes of 22 healthy subjects were included in this study. Patients were classified into patients with definite SSc and patients with VEDOSS. VD data of the superficial OCT angiogram (OCTA-SCP), deep OCT angiogram (OCTA-DCP) and choriocapillaris (OCTA-CC) were analysed. VD in the OCTA-SCP and OCTA-CC was lower in patients with SSc (p < 0.05). In VEDOSS patients, VD in the OCTA-CC was still reduced compared to controls (p < 0.05). Correlation analysis revealed a positive correlation between nailfold capillaroscopy and VD of OCTA-CC (Spearman correlation coefficient (rSp) 0.456, p < 0.05) and a negative correlation between skin score and VD of OCTA-SCP (p < 0.05). Ocular perfusion seems to be impaired in patients with SSc and even VEDOSS. VD correlated with disease severity. OCTA could be a new useful diagnostic and predictive parameter for monitoring patients with different stages of the disease.

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2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative.

Frank J.A. van den Hoogen, Dinesh Khanna, Jaap Fransen … (2013)

The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc. Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by 1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and 2) validating against the combined view of a group of experts on a set of cases with or without SSc. It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, 7 additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc. The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease. Copyright © 2013 by the American College of Rheumatology.

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Optical coherence tomography angiography

Richard Spaide, James Fujimoto, Nadia K. Waheed … (2017)

Optical coherence tomography (OCT) was one of the biggest advances in ophthalmic imaging. Building on that platform, OCT angiography (OCTA) provides depth resolved images of blood flow in the retina and choroid with levels of detail far exceeding that obtained with older forms of imaging. This new modality is challenging because of the need for new equipment and processing techniques, current limitations of imaging capability, and rapid advancements in both imaging and in our understanding of the imaging and applicable pathophysiology of the retina and choroid. These factors lead to a steep learning curve, even for those with a working understanding dye-based ocular angiography. All for a method of imaging that is a little more than 10 years old. This review begins with a historical account of the development of OCTA, and the methods used in OCTA, including signal processing, image generation, and display techniques. This forms the basis to understand what OCTA images show as well as how image artifacts arise. The anatomy and imaging of specific vascular layers of the eye are reviewed. The integration of OCTA in multimodal imaging in the evaluation of retinal vascular occlusive diseases, diabetic retinopathy, uveitis, inherited diseases, age-related macular degeneration, and disorders of the optic nerve is presented. OCTA is an exciting, disruptive technology. Its use is rapidly expanding in clinical practice as well as for research into the pathophysiology of diseases of the posterior pole.

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Update of EULAR recommendations for the treatment of systemic sclerosis

Otylia Kowal-Bielecka, Jaap Fransen, Jerome Avouac … (2017)

The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc.

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Author and article information

Contributors

Larissa Lahme: larissa.lahme@ukmuenster.de

Journal

Journal ID (nlm-ta): Sci Rep

Journal ID (iso-abbrev): Sci Rep

Title: Scientific Reports

Publisher: Nature Publishing Group UK (London )

ISSN (Electronic): 2045-2322

Publication date (Electronic): 29 June 2022

Publication date PMC-release: 29 June 2022

Publication date Collection: 2022

Volume: 12

Electronic Location Identifier: 10990

Affiliations

[1 ]GRID grid.16149.3b, ISNI 0000 0004 0551 4246, Department of Ophthalmology, , University of Muenster Medical Center, ; Domagkstraße 15, Muenster, Germany

[2 ]GRID grid.10253.35, ISNI 0000 0004 1936 9756, Department of Ophthalmology, Klinikum Fulda, , University of Marburg, ; Campus Fulda, Fulda, Germany

[3 ]GRID grid.5949.1, ISNI 0000 0001 2172 9288, Department of Dermatology, , University of Muenster, ; Muenster, Germany

Article

Publisher ID: 14377

DOI: 10.1038/s41598-022-14377-6

PMC ID: 9243093

PubMed ID: 35768479

SO-VID: 84cea265-6cef-4def-b3bc-eaff41f24abb

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Open Access This 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/.

History

Date received : 25 January 2022

Date accepted : 6 June 2022

Funding

Funded by: Westfälische Wilhelms-Universität Münster (1056)

Open Access :

Open Access funding enabled and organized by Projekt DEAL.

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ScienceOpen disciplines: Uncategorized

Keywords: rheumatology,eye diseases

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ScienceOpen disciplines: Uncategorized

Keywords: rheumatology, eye diseases

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Altered ocular microvasculature in patients with systemic sclerosis and very early disease of systemic sclerosis using optical coherence tomography angiography

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Most cited references 31

2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative.

Optical coherence tomography angiography

Update of EULAR recommendations for the treatment of systemic sclerosis

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