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

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

doi:10.1016/j.semarthrit.2018.12.007

Cited by 30 publications

(22 citation statements)

References 65 publications

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“…The demographic data including disease duration (date of first Raynaud's and non-Raynaud's symptoms); serological (anti-Scl-70, anti-centromere and antinuclear antibody), clinical [diffuse cutaneous SSc (dsSSc), and limited cutaneous SSc (lcSSc)] subsets according to LeRoy et al [1]; and skin scoring according to the definition by LeRoy et al in the MRSS (Modified Rodnan Score) [18] classified into 5 groups -normal, mild (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14), moderate (15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29), severe (30-39) and end stage (40+) -were gathered. Pulmonary involvement was assessed by high resolution CT scan (HRCT) at the first screening or after detecting low diffusing capacity of lung for carbon mono xide (DLCO) < 80% or low forced expiratory volume (FEV 1 ) < 75% in the pulmonary function test (PFT).…”

Section: Methodsmentioning

confidence: 99%

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Prevalence of retinal changes in patients with systemic sclerosis: the association between retinal vascular changes and nailfold capillaroscopic findings

Shenavandeh

Afarid²,

Hasanaghaei

et al. 2021

Reumatologia

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Background Systemic sclerosis (SSc) is a connective tissue disease affecting the skin and multiple organs. Most of the evidence on ocular involvement comes from small studies. We evaluated the retinal vascular changes in patients with SSc and its associations with types, disease duration, skin score, and nailfold capillaroscopy changes. Material and methods We evaluated the demographic, clinical and nailfold capillaroscopy data of 52 patients referring to the clinic of scleroderma with SSc according to the 2013 ACR/EULAR SSc criteria. In addition, full ophthalmic examination in 52 patients and fundus photography in 40 patients were done in the ophthalmology clinic. Results There were 52 (49 women and 3 men, 17 diffuse and 35 limited) patients with SSc with mean disease duration of 8 ±5 years and mean age of 40 ±9 years. Retinal changes in the ophthalmologic examination were seen in 30.7% as increased vascular tortuosity. None of the patients had cotton wool spot, hemorrhage or hard exudate. Forty patients underwent fundus photography and 22.5% of them had vascular tortuosity. Only the presence of hemorrhage in the nailfolds was correlated with retinal tortuosity, and the other characteristics of nailfold capillaroscopy did not have any association with retinal changes. Conclusions Retinal vascular changes were seen in about one third of our patients. There was no correlation between nailfold capillaroscopy, disease duration, type and skin score of SSc patients and retinal vascular changes. These findings suggest that the mechanisms or the quality of changes in the retinal vessels and nailfold vasculature may be different.

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Section: Methodsmentioning

confidence: 99%

“…In SSc, small vessels are mostly affected and, in some studies, cotton-wool spots in the retina, frequent atrophic lesions of the retinal pigment epithelium within the posterior pole of the retina, dilatation of the venous vessels, intra-retinal extravasation, hard exudates, and macular edema have been reported [10,11,[14][15][16].…”

Section: Introductionmentioning

confidence: 99%

Prevalence of retinal changes in patients with systemic sclerosis: the association between retinal vascular changes and nailfold capillaroscopic findings

Shenavandeh

Afarid²,

Hasanaghaei

et al. 2021

Reumatologia

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“…In the posterior segment, the most frequent finding was the alteration of the retinal pigment epithelium 3 (8.8%). Case-control studies are inconclusive about the changes observed in retina, unlike the choroidal vasculature appears to be more affected than the retinal microcirculation [20].…”

Section: Discussionmentioning

confidence: 99%

Ophthalmic Findings in Systemic Sclerosis and Its Association with Dermal Fibrosis

Villegas¹,

Yáñez²

2019

OALib

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Objective: To determine frequency and type of ophthalmologic findings in Systemic Sclerosis (SSc) and to establish their association with the extent and severity of cutaneous fibrosis. Methods: Descriptive-transversal study of 34 patients, (January-December 2017). It included patients with limited cutaneous Systemic Sclerosis (lcSS) and diffuse cutaneous Systemic Sclerosis (dcSS). The severity of cutaneous fibrosis was measured using the modified Rodnan skin score (mRSS). The ophthalmologic examination consisted of the evaluation of the palpebral fibrosis and the ocular surface by Schirmer's test, tear rupture time (BUT), OSDI (Ocular Disease Surface Index) and SANDE (Symptom Assessment in Dry Eye) questionnaires, eye fundus and retinography. We use simple logistic regression (OR) to establish the association with p < 0.05. Results: 21 patients (61.7%) with dcSS and 13 patients (38.2%) with lcSS. Women 32 (94.1%). Mean disease time 67.4 months. The mRSS was mild in 10 (29.4%) and moderate-severe in 24 (70.6%). The main findings: palpebral fibrosis 30 (88.2%), keratoconjunctivitis sicca (KCS) 26 (76.5%), blepharophimosis 20 (58.8%) and enophthalmos 7 (20.6%) had alteration of the retinal pigment epithelium 3 (8.8%). Associated with the extension: blepharophimosis, enophthalmos and KCS predominated in dcSS with Schirmer test positive in 52.4%, mild dry eye highlighted in both groups. We found significant association between blepharophimosis and moderate-severe mRSS. Conclusion: Eyelids skin thickening and KCS were more prevalent in dcSS. Moderate-severe mRSS was associated with atrophic changes in the eyelids.

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“…Genetic and environmental factors determine the susceptibility, severity, and onset of this disease [1]. Its peak incidence is between 30 and 60 years of age with a female predilection (6:1 ratio), while a more severe expression of the disease, including internal organ-based complications and higher mortality, has been reported in men [3,4].…”

Section: Introductionmentioning

confidence: 99%

“…Additionally, changes in collagen content and distribution within the cornea, the transparent outer layer of the eye and the main refractive unit, may cause plausible pathologic changes to ocular function in SSc patients. Indeed, in SSc patients, among the most common reported ocular pathologies is eyelid stiffness, which is seen in more than 50% of cases and results from the deposition of type I collagen in the dermis [4]. Keratoconjunctivitis sicca is the second most common problem, seen in 50% of affected patients, followed by cases of conjunctivitis, episcleritis, anterior uveitis, and hypertensive retinopathy [16].…”

Section: Introductionmentioning

confidence: 99%

Dry Eye in Systemic Sclerosis Patients: Novel Methods to Monitor Disease Activity

et al. 2020

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Background: In systemic sclerosis (SSc) patients, dry eye syndrome (DES) is the most frequent ocular feature. The aim of this study was to investigate ocular DES-related SSc patients and to establish any correlation with the severity of the disease. Methods: Retrospectively, data from 60 patients with SSc underwent ophthalmic examination, where non-invasive film tear break-up time (NIF-TBUT), tear film lipid layer thickness (LLT), anesthetic-free Schirmer test I, tear osmolarity measurement (TearLab System), and modified Rodnan skin score (mRSS) data were collected. The visual analog scale (VAS) and Symptom Assessment in Dry Eye (SANDE) methods were utilized. The results were correlated with mRSS and the duration of SSc. Results: Severe DES occurred in 84% of cases, and was more severe in women. The eyelids were involved in 86.6%, secondary to meibomian gland disease (MGD). A direct correlation was found between the tear osmolarity (mean 328.51 ± 23.8 SD) and skin score (mRSS) (r = 0.79; p < 0.01). Significantly reduced NIF-TBUT, LLT, and Schirmer test I values were observed in the case of severe skin involvement. Conclusions: SSc patients show lipid tear dysfunction related to the severity and duration of the disease due to inflammation and the subsequent atrophy of the meibomian glands.

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

Cited by 30 publications

References 65 publications

Prevalence of retinal changes in patients with systemic sclerosis: the association between retinal vascular changes and nailfold capillaroscopic findings

Prevalence of retinal changes in patients with systemic sclerosis: the association between retinal vascular changes and nailfold capillaroscopic findings

Ophthalmic Findings in Systemic Sclerosis and Its Association with Dermal Fibrosis

Dry Eye in Systemic Sclerosis Patients: Novel Methods to Monitor Disease Activity

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