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Hermoso-Fernández, Norberto Ortego-Centeno, José L. Callejas-Rubio" "autores" => array:3 [ 0 => array:4 [ "nombre" => "Francisco M." "apellidos" => "Hermoso-Fernández" "email" => array:1 [ 0 => "framaherfer@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Norberto" "apellidos" => "Ortego-Centeno" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "José L." "apellidos" => "Callejas-Rubio" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Departamento de Oftalmología, Hospital Universitario San Cecilio, Granada, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Departamento de Medicina Interna, Hospital Universitario San Cecilio, Granada, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Vasculitis retiniana bilateral asociada a esclerosis sistémica sine escleroderma" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 516 "Ancho" => 755 "Tamanyo" => 40669 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Left eye: arrow A, sheathed veins leading to vascular occlusions; arrow B, telangiectasias; arrow C, haemorrhages.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Systemic sclerosis is a rare connective tissue disorder characterized by abnormal proliferation of fibroblasts leading to deposition of extracellular matrix in the skin, blood vessels, or viscera. This results in rigidity of connective tissue structures in the skin or in the body's organs.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> On the other hand, retinal vasculitis is an inflammation of the retinal blood vessels that progresses from vessel closure to ischemia and subsequent retinal neovascularization. It is characterized by intermittent sheathing of affected eyes and aneurysms, showing vascular staining on fluorescein angiography. Systemic sclerosis has been linked to numerous ocular conditions but, to the best of our knowledge, this is the first reported case of a combination of systemic sclerosis and bilateral retinal vasculitis.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a 40-year-old woman who came to our hospital for blurred vision since that morning. On ophthalmologic examination, her best-corrected visual acuity (Snellen chart) was 20/20 in both eyes. Nothing unusual was noticed on slit-lamp examinations. Fundus evaluation showed retinal occlusion of the inferior temporal branch vein, retinal neovascularisation, venous tortuosity and vitreous haemorrhage in the left eye, as well as superior temporal arteritis in the right eye. Retinography showed transient sheathed veins and some occluded, surrounded by a large number of retinal haemorrhages in both eyes (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Fluorescein angiography showed extensive areas of non-perfused retina with telangiectasias and staining of retinal vessel walls.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">The study of infectious causes for uveitis was negative (tuberculosis, syphilis, Lyme disease). The general work-up pointed to vasculitis of immunological origin: Raynaud’s phenomenon, pathological capillaroscopy, positive ANAS 1/640, so argon laser photocoagulation was applied to the ischaemic retina of both eyes. There was a lack of response to cyclosporine and azathioprine, with persistent vascular sheathing and repeated vitreous haemorrhage. Her persistent vitreous haemorrhage in the right eye was treated with vitrectomy. Systemic treatment consisted of glucocorticoids and adalimumab for 2 years, resulting in clinical stability.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The diagnosis emerged later when she reported gastro-oesophageal reflux and heartburn. Oesophageal manometry was performed, which showed dysmotility with absence of movement and a positive result for anticentromere antibodies. It was classified as gastrointestinal vasculitis, which remitted with corticosteroid boluses. A diagnosis of systemic sclerosis <span class="elsevierStyleItalic">sine</span> scleroderma was made.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The general treatment was discontinued six months later due to inactivity of the vasculitis, clearance of vascular sheathing and absence of retinal haemorrhages.</p><p id="par0030" class="elsevierStylePara elsevierViewall">The patient has undergone monthly follow-up for a period of 2 years and the clinical features of the treated lesions have remained unchanged during this time.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Systemic sclerosis is characterized by significant vascular involvement that is not limited to the peripheral microcirculation of the skin, but has also been observed in the heart, lungs, kidneys, gastrointestinal tract and eyes.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Depending on disease onset we can identify 3 types: diffuse cutaneous systemic sclerosis, which is associated with anti-topoisomerase antibodies while limited cutaneous scleroderma and <span class="elsevierStyleItalic">sine</span> scleroderma is mainly associated with anti-centromere<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> antibody.</p><p id="par0040" class="elsevierStylePara elsevierViewall">Histological studies in patients with systemic sclerosis have shown that the choroidal vessels are severely affected and show endothelial cell damage, basement membrane thickening and absence of pericytes. The persistence of such widespread endothelial dysfunction in systemic vasculitis seems to have long-term consequences, leading to the acceleration of premature ischaemic disease.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">We can conclude that systemic sclerosis <span class="elsevierStyleItalic">sine</span> scleroderma can occur with retinal vasculitis. Retinal vasculitis may precede the development of systemic sclerosis and the involvement of internal organs, including cardiac involvement, by several years, which represents the most relevant clinical characteristic with a high rate of morbidity and mortality<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> and, therefore, a positive Raynaud's phenomenon or a pathological capillaroscopy with anti-centromere antibodies should alert us to look for possible systemic and organ involvement of this disease.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 516 "Ancho" => 755 "Tamanyo" => 40669 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Left eye: arrow A, sheathed veins leading to vascular occlusions; arrow B, telangiectasias; arrow C, haemorrhages.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Gender and ocular manifestations of connective tissue diseases and systemic vasculitides. Sen HN, editor" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "M.M. Choudhary" 1 => "R.A. Hajj-Ali" 2 => "C.Y. Lowder" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1155/2014/403042" "Revista" => array:4 [ "tituloSerie" => "J Ophthalmol [Internet]" "fecha" => "2014" "volumen" => "2014" "itemHostRev" => array:3 [ "pii" => "S0016508509013778" "estado" => "S300" "issn" => "00165085" ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Choroidal impairment and macular thinning in patients with systemic sclerosis: the acute study" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "F. Ingegnoli" 1 => "R. Gualtierotti" 2 => "L. Pierro" 3 => "C. Del Turco" 4 => "E. Miserocchi" 5 => "T. 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Zanatta" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1093/rheumatology/keab738" "Revista" => array:2 [ "tituloSerie" => "Rheumatology [Internet]" "fecha" => "2021" ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000015900000006/v1_202209200648/S2387020622004132/v1_202209200648/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000015900000006/v1_202209200648/S2387020622004132/v1_202209200648/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020622004132?idApp=UINPBA00004N" ]
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Vol. 159. Issue 6.
Pages e39-e40 (September 2022)
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Vol. 159. Issue 6.
Pages e39-e40 (September 2022)
Letter to the Editor
Bilateral retinal vasculitis in association with systemic sclerosis sine scleroderma
Vasculitis retiniana bilateral asociada a esclerosis sistémica sine escleroderma
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