Abstract

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.

Highlights

  • IntroductionSystemic sclerosis (SSc; scleroderma) is a complex multisystem autoimmune disease of unknown etiology, characterized by vasculopathy and tissue fibrosis of the skin and various internal organs [1,2].Diagnostics 2020, 10, 404; doi:10.3390/diagnostics10060404 www.mdpi.com/journal/diagnosticsGenetic 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].The clinical manifestations of the disease are the result of three distinct processes: innate and adaptive immune system abnormalities, leading to the production of autoantibodies and cell-mediated autoimmunity, vasculopathy of small vessels, and fibroblast dysfunction leading to the accumulation of excessive collagen and other matrix components in the skin, blood vessels, and internal organs [5,6,7].Dry eye syndrome (DES) is a dysfunction of the tears and the ocular surface; it is very common and complex, affecting 5–50% of patients, giving rise to symptoms of discomfort, visual disturbances, and tear instability due to an increase in tear osmolarity and the inflammation of the ocular surface [8,9].Due to increases in video terminal syndrome and office eye disease syndrome, the prevalence of dry eye syndrome (DES) rises annually

  • dry eye syndrome (DES) is associated with many connective tissue diseases, and it has been reported as a clinical manifestation in the course of systemic sclerosis (SSc) [19]

  • When the ocular immune system is excessively stimulated, and/or the immunoregulatory mechanisms are disrupted, the balance between the innate and adaptive phases is dysregulated. This results in an abnormal production of autoantibodies and cell-mediated autoimmunity response, which leads to a fibroblast dysfunction, ocular surface inflammation, and chronic DES [25]

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Summary

Introduction

Systemic sclerosis (SSc; scleroderma) is a complex multisystem autoimmune disease of unknown etiology, characterized by vasculopathy and tissue fibrosis of the skin and various internal organs [1,2].Diagnostics 2020, 10, 404; doi:10.3390/diagnostics10060404 www.mdpi.com/journal/diagnosticsGenetic 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].The clinical manifestations of the disease are the result of three distinct processes: innate and adaptive immune system abnormalities, leading to the production of autoantibodies and cell-mediated autoimmunity, vasculopathy of small vessels, and fibroblast dysfunction leading to the accumulation of excessive collagen and other matrix components in the skin, blood vessels, and internal organs [5,6,7].Dry eye syndrome (DES) is a dysfunction of the tears and the ocular surface; it is very common and complex, affecting 5–50% of patients, giving rise to symptoms of discomfort, visual disturbances, and tear instability due to an increase in tear osmolarity and the inflammation of the ocular surface [8,9].Due to increases in video terminal syndrome and office eye disease syndrome, the prevalence of DES rises annually. Dry eye syndrome (DES) is a dysfunction of the tears and the ocular surface; it is very common and complex, affecting 5–50% of patients, giving rise to symptoms of discomfort, visual disturbances, and tear instability due to an increase in tear osmolarity and the inflammation of the ocular surface [8,9]. In systemic sclerosis (SSc) patients, dry eye syndrome (DES) is the most frequent ocular feature. 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. 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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