{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,15]],"date-time":"2026-03-15T14:21:25Z","timestamp":1773584485844,"version":"3.50.1"},"reference-count":43,"publisher":"MDPI AG","issue":"15","license":[{"start":{"date-parts":[[2023,8,5]],"date-time":"2023-08-05T00:00:00Z","timestamp":1691193600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>This cross-sectional study aimed to compare optical coherence tomography angiography (OCT-A) findings in patients with primary Raynaud\u2019s phenomenon (PRP; n = 22), very early disease of systemic sclerosis (VEDOSS; n = 19), and systemic sclerosis (SSc; 25 patients with limited cutaneous SSc (lcSSc) and 13 patients with diffuse cutaneous SSc (dcSSc)). Whole, parafoveal, and perifoveal superficial capillary plexus (SCP) vessel densities (VDs), deep capillary plexus VDs, and whole, inside, and peripapillary VDs were significantly higher in the PRP group (p &lt; 0.001). In the lcSSc group, the FAZ perimeter was significantly higher than that in the VEDOSS group (p = 0.017). Retinal nerve fiber layer VDs were significantly lower in the lcSSc group than in the PRP and VEDOSS groups (p &lt; 0.001). The whole and peripapillary optic disc VDs of the VEDOSS group were significantly higher than in the lcSSc group (p &lt; 0.001). Whole SCP VDs (94.74% sensitivity, 100.00% specificity) and parafoveal SCP VDs (89.47% sensitivity, 100.00% specificity) showed the best performance in distinguishing patients with SSc from those with PRP. OCT-A seems to have potential diagnostic value in differentiating patients with PRP from patients with SSc and VEDOSS, and there is potential value in assessing prognostic roles, since findings from OCT-A images could be early indicators of retinal vascular injury long before overt SSc symptoms develop.<\/jats:p>","DOI":"10.3390\/diagnostics13152607","type":"journal-article","created":{"date-parts":[[2023,8,5]],"date-time":"2023-08-05T10:21:39Z","timestamp":1691230899000},"page":"2607","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Optical Coherence Tomography Angiography Is a Useful Tool for Distinguishing Primary Raynaud\u2019s Phenomenon from Systemic Sclerosis and\/or Very Early Disease of Systemic Sclerosis"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8882-0692","authenticated-orcid":false,"given":"Adem","family":"Erturk","sequence":"first","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Afyonkarahisar Health Sciences University, Afyonkarahisar 03030, Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0875-1517","authenticated-orcid":false,"given":"Ozgur","family":"Erogul","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Faculty of Medicine, Afyonkarahisar Health Sciences University, Afyonkarahisar 03030, Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Murat","family":"Kasikci","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Mugla Training and Research Hospital, Mugla 48000, Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,8,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1038\/s41433-019-0591-z","article-title":"Analysis of retinal and choroidal microvasculature in systemic sclerosis: An optical coherence tomography angiography study","volume":"34","year":"2020","journal-title":"Eye"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"15002","DOI":"10.1038\/nrdp.2015.2","article-title":"Systemic sclerosis","volume":"1","author":"Allanore","year":"2015","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1097\/BOR.0000000000000877","article-title":"Practical management of Raynaud\u2019s phenomenon\u2014A primer for practicing physicians","volume":"34","author":"Ramahi","year":"2022","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"10990","DOI":"10.1038\/s41598-022-14377-6","article-title":"Altered ocular microvasculature in patients with systemic sclerosis and very early disease of systemic sclerosis using optical coherence tomography angiography","volume":"12","author":"Mihailovic","year":"2022","journal-title":"Sci. Rep."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"3902","DOI":"10.1002\/art.24038","article-title":"Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud\u2019s phenomenon to systemic sclerosis: A twenty-year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis","volume":"58","author":"Koenig","year":"2008","journal-title":"Arthritis Rheum."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2087","DOI":"10.1136\/annrheumdis-2013-203716","article-title":"Preliminary analysis of the very early diagnosis of systemic sclerosis (VEDOSS) EUSTAR multicentre study: Evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis","volume":"73","author":"Minier","year":"2014","journal-title":"Ann. Rheum. Dis."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1136\/ard.2010.136929","article-title":"Preliminary criteria for the very early diagnosis of systemic sclerosis: Results of a Delphi Consensus Study from EULAR Scleroderma Trials and Research Group","volume":"70","author":"Avouac","year":"2011","journal-title":"Ann. Rheum. Dis."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1136\/ard.62.3.204","article-title":"Retinal findings in systemic sclerosis: A comparison with nailfold capillaroscopic patterns","volume":"62","author":"Ushiyama","year":"2003","journal-title":"Ann. Rheum. Dis."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1590\/S1807-59322011000300003","article-title":"Ocular findings in patients with systemic sclerosis","volume":"66","author":"Santhiago","year":"2011","journal-title":"Clinics"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1016\/0002-9394(72)91208-1","article-title":"The choroidopathy of progressive systemic sclerosis (scleroderma)","volume":"74","author":"Farkas","year":"1972","journal-title":"Am. J. Ophthalmol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1080\/09273948.2019.1657467","article-title":"A wide spectrum of ocular manifestations signify patients with systemic sclerosis","volume":"29","author":"Szucs","year":"2021","journal-title":"Ocul. Immunol. Inflamm."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"104166","DOI":"10.1016\/j.mvr.2021.104166","article-title":"Evaluation of the direct effects on retinal and choroidal microvascularity of systemic scleroderma","volume":"136","author":"Ayan","year":"2021","journal-title":"Microvasc. Res."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1016\/j.semarthrit.2018.12.007","article-title":"Ocular involvement in systemic sclerosis: A systematic literature review, it\u2019s not all scleroderma that meets the eye","volume":"49","author":"Kreps","year":"2019","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"864871","DOI":"10.1155\/2015\/864871","article-title":"Ocular Blood Flow Autoregulation Mechanisms and Methods","volume":"2015","author":"Luo","year":"2015","journal-title":"J. Ophthalmol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1136\/ard.36.2.152","article-title":"Involvement of the eye in SLE and scleroderma. A study using fluorescein angiography in addition to clinical ophthalmic assessment","volume":"36","author":"Grennan","year":"1977","journal-title":"Ann. Rheum. Dis."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1016\/j.joco.2018.02.001","article-title":"Impact of correct anatomical slab segmentation on foveal avascular zone measurements by optical coherence tomography angiography in healthy adults","volume":"30","author":"Rommel","year":"2018","journal-title":"J. Curr. Ophthalmol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1001\/jamaophthalmol.2014.3616","article-title":"Retinal vascular layers imaged by fluorescein angiography and optical coherence tomography angiography","volume":"133","author":"Spaide","year":"2015","journal-title":"JAMA Ophthalmol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.ajo.2017.11.011","article-title":"Reproducibility of vessel density, fractal dimension, and foveal avascular zone using 7 different optical coherence tomography angiography devices","volume":"186","author":"Corvi","year":"2018","journal-title":"Am. J. Ophthalmol."},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Mihailovic, N., Brand, C., Lahme, L., Schubert, F., Bormann, E., Eter, N., and Alnawaiseh, M. (2018). Repeatability, reproducibility and agreement of foveal avascular zone measurements using three different optical coherence tomography angiography devices. PLoS ONE, 13.","DOI":"10.1371\/journal.pone.0206045"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1107","DOI":"10.5114\/aoms.2013.39217","article-title":"Prevalence of ocular manifestations in systemic sclerosis patients","volume":"9","author":"Waszczykowska","year":"2013","journal-title":"Arch. Med. Sci."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/j.mvr.2014.08.008","article-title":"Choroidal impairment and macular thinning in patients with systemic sclerosis: The acute study","volume":"97","author":"Ingegnoli","year":"2015","journal-title":"Microvasc. Res."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1038\/eye.2016.216","article-title":"Structural and molecular changes in the aging choroid: Implications for age-related macular degeneration","volume":"31","author":"Chirco","year":"2017","journal-title":"Eye"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1136\/annrheumdis-2018-214235","article-title":"Evaluation of retinal microvascular density in patients affected by systemic lupus erythematosus: An optical coherence tomography angiography study","volume":"78","author":"Conigliaro","year":"2019","journal-title":"Ann. Rheum. Dis."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"5827","DOI":"10.1093\/rheumatology\/keab258","article-title":"Retinal microcirculation abnormalities in patients with systemic sclerosis: An explorative optical coherence tomography angiography study","volume":"60","author":"Carnevali","year":"2021","journal-title":"Rheumatology"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1136\/annrheumdis-2018-214541","article-title":"Evaluation of retinal microvascular perfusion in systemic sclerosis: A case-control study","volume":"78","author":"Rothe","year":"2019","journal-title":"Ann. Rheum. Dis."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"148727","DOI":"10.1155\/2013\/148727","article-title":"Clinical study. Idiopathic granulomatous mastitis: An autoimmune disease","volume":"2013","author":"Altintoprak","year":"2013","journal-title":"Sci. World J."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"2737","DOI":"10.1002\/art.38098","article-title":"2013 classification criteria for systemic sclerosis: An American College of Rheumatology\/European League against Rheumatism collaborative initiative","volume":"65","author":"Khanna","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"iii14","DOI":"10.1093\/rheumatology\/kep108","article-title":"Skin disease: A cardinal feature of systemic sclerosis","volume":"48","author":"Krieg","year":"2009","journal-title":"Rheumatology"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"171","DOI":"10.2147\/OARRR.S285409","article-title":"Predicting the progression of very early systemic sclerosis: Current insights","volume":"14","author":"Bellocchi","year":"2022","journal-title":"Open Access Rheumatol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"S1789","DOI":"10.21037\/jtd.2019.08.54","article-title":"Evaluation and classification of pulmonary arterial hypertension","volume":"11","author":"Sahay","year":"2019","journal-title":"J. Thorac. Dis."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1953","DOI":"10.1002\/art.37988","article-title":"Review: Evidence that systemic sclerosis is a vascular disease","volume":"65","author":"Kahaleh","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1159\/000113889","article-title":"Choroidal sclerosis in localized scleroderma (morphea en plaque)","volume":"40","author":"Milenkovic","year":"2008","journal-title":"Ophthalmic Res."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1097\/ICB.0000000000000146","article-title":"Indocyanine green angiographic evidence of choroiditis in scleroderma","volume":"9","author":"Abdellatief","year":"2015","journal-title":"Retin. Cases Brief Rep."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1111\/ceo.12905","article-title":"Hypertensive eye disease: A review","volume":"45","author":"Symes","year":"2017","journal-title":"Clin. Exp. Ophthalmol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1364\/BOE.7.000911","article-title":"Comparison of amplitude-decorrelation, speckle-variance and phase-variance OCT angiography methods for imaging the human retina and choroid","volume":"7","author":"Gorczynska","year":"2016","journal-title":"Biomed. Opt. Express"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1093\/rheumatology\/keq355","article-title":"Registries in systemic sclerosis: A worldwide experience","volume":"50","author":"Galluccio","year":"2011","journal-title":"Rheumatology"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1590\/abd1806-4841.20153881","article-title":"Systemic lupus erythematosus and Raynaud\u2019s phenomenon","volume":"90","author":"Heimovski","year":"2015","journal-title":"An. Bras. Dermatol."},{"key":"ref_38","doi-asserted-by":"crossref","unstructured":"Pieklarz, B., Gi\u0144dzie\u0144ska-Sie\u015bkiewicz, E., Zawadzka, I., Bagrowska, M., Daniluk, J., Konopi\u0144ska, J., Kowal-Bielecka, O., and Dmuchowska, D.A. (2023). Purtscher-like Retinopathy in a Patient with Systemic Sclerosis: A Case Report and Narrative Review. Biomedicines, 11.","DOI":"10.3390\/biomedicines11030839"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/j.autrev.2018.09.011","article-title":"Take a look at the eyes in systemic lupus erythematosus: A novel point of view","volume":"18","author":"Conigliaro","year":"2019","journal-title":"Autoimmun. Rev."},{"key":"ref_40","doi-asserted-by":"crossref","unstructured":"Garcia-Soler, E., Martinez-Rubio, C., De Smet, M.D., Udaondo, P., and Salom-Alonso, D. (2023). Systemic lupus erythematosus purtscher like retinopathy: Optical coherence tomography angiography assessment implications. Eur. J. Ophthalmol., 11206721231171705.","DOI":"10.1177\/11206721231171705"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"12440","DOI":"10.1111\/micc.12440","article-title":"Microvascular involvement in systemic sclerosis and systemic lupus erythematosus","volume":"26","author":"Saygin","year":"2019","journal-title":"Microcirculation"},{"key":"ref_42","first-page":"e834","article-title":"Progression of patients with Raynaud\u2019s phenomenon to Systemic Sclerosis classified according to the 2013 ACR\/EULAR criteria: Five year analysis of the EUSTAR multicentre prospective study for very early diagnosis of systemic sclerosis (VEDOSS)","volume":"3","author":"Lepri","year":"2021","journal-title":"Lancet Rheumatol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"3686","DOI":"10.1093\/rheumatology\/keac006","article-title":"Predictors of progression to systemic sclerosis: Analysis of very early diagnosis of systemic sclerosis in a large single-centre cohort","volume":"61","author":"Siqueira","year":"2022","journal-title":"Rheumatology"}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/13\/15\/2607\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T20:26:36Z","timestamp":1760127996000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/13\/15\/2607"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,8,5]]},"references-count":43,"journal-issue":{"issue":"15","published-online":{"date-parts":[[2023,8]]}},"alternative-id":["diagnostics13152607"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics13152607","relation":{},"ISSN":["2075-4418"],"issn-type":[{"value":"2075-4418","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,8,5]]}}}