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"autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Gabriel Horta-Baas, Imelda Vergara-Sánchez, Maria del Socorro Romero-Figueroa" "autores" => array:3 [ 0 => array:4 [ "nombre" => "Gabriel" "apellidos" => "Horta-Baas" "email" => array:1 [ 0 => "gabho@hotmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Imelda" "apellidos" => "Vergara-Sánchez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "Maria del Socorro" "apellidos" => "Romero-Figueroa" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Reumatología, Hospital General Regional 220, Instituto Mexicano del Seguro Social, Toluca, Estado de México, Mexico" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Neurología Pediátrica, Hospital General Regional 1, Instituto Mexicano del Seguro Social, Mérida, Yucatán, Mexico" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Coordinación de Investigación en Salud, Delegación Estado de México Poniente, Instituto Mexicano del Seguro Social, Toluca, Estado de México, Mexico" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Vasculopatía del sistema nervioso central en esclerosis sistémica limitada" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1082 "Ancho" => 999 "Tamanyo" => 174254 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Angio-resonance of the brain showing a narrowing of the middle cerebral artery (arrow) and of the anterior cerebral artery in its precommunicating segment (*).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We read with interest the review “Large vessel vasculopathy in systemic sclerosis” by Tejera Segura and Ferraz-Amaro<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> and would like to contribute an additional case of vasculopathy of the central nervous system.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 60-year-old woman with a history of asymmetric Raynaud's syndrome, hand oedema and joint pain for seven years. It started with sudden headaches, disorientation and impaired cognition. The electroencephalogram recorded data of frontal lobe epilepsy and the angio-resonance a narrowing of the middle cerebral artery and anterior cerebral artery in its precommunicating segment (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The transthoracic echocardiogram showed a structurally healthy heart, with no evidence of pulmonary hypertension; the carotid ultrasound did not detect atherosclerosis. Laboratory studies showed: positive centromeric patterns of antinuclear antibodies 1:160, positive anti-centromere antibodies; anti-Scl-70, anti-Ro, pANCA, cANCA and negative rheumatoid factor; the C reactive protein and erythrocyte sedimentation rate were reported within normal limits. Arterial hypertension or renal crisis data was not documented. Diagnosed with systemic sclerosis (SS), limited cutaneous systemic sclerosis (lcSSc) and vascular disease of the central nervous system (CNS), treatment was initiated with 0.5<span class="elsevierStyleHsp" style=""></span>mg/kg/day prednisone, 1.5<span class="elsevierStyleHsp" style=""></span>g/day mycophenolic acid, 100<span class="elsevierStyleHsp" style=""></span>mg/day acetylsalicylic acid salicylic, 200<span class="elsevierStyleHsp" style=""></span>mg/day hydroxychloroquine and 200<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>/day magnesium valproate, which resulted in a remission of all symptoms within 5 days of starting treatment.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">SS is a multisystemic disease characterised by deposition of collagen in the dermis and internal organs, vascular hyperreactivity, endothelial dysfunction, microvascular disorders and angiogenesis, and in the activation of cellular and humoral immunity.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2–4</span></a> Typical vascular abnormality is endothelial injury with mononuclear cell infiltration, platelet activation and intimal hyperplasia; it is considered a microvascular with perivascular inflammation, leading to the narrowing of the vessels without evidence of vasculitis.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,5</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Although vascular endothelial injury mainly affects microcirculation,<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> recent studies show that there is a condition of the medium and large calibre vessels, especially in the ulnar arteries in patients with lcSSc, and in involvement with anti-centromere antibodies.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3,5</span></a> Unlike other connective tissue diseases, it is believed that SS rarely affects the CNS. This can be explained by lack of connective tissue in the brain, the peculiar histology of cerebral arteries (absence of the external elastic lamina, the scarcity of the media and adventitia), and the immunological particularity of the brain given the almost total absence of lymphocytes in normal conditions.<a class="elsevierStyleCrossRefs" href="#bib0080"><span class="elsevierStyleSup">6,7</span></a> However, cases of CNS vascular disease have been documented through studies of angiography or angio-resonance,<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,4,8</span></a> and autopsy studies have described calcifications of the brain arteries, suggesting that SS could induce primary brain vascular changes.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">When there is CNS involvement in the scleroderma, it usually manifests as linear scleroderma en coup de saber, complications of heart-lung or kidney disease, coexistence of a systemic vasculitis or an overlap syndrome and, to a lesser extent, by the pathological process of the SS.<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">9,10</span></a> The presence of cerebral vascular disease has been associated with pulmonary arterial hypertension and scleroderma renal crisis.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">There is a report of five cases of vasculitis associated with SS, four with diffuse SS, and in one case with lcSSc; the cases were women aged between 24 and 67, the diagnosis of CNS vasculitis was the result of cerebral angiography. In two of the five cases a biopsy was carried out of the leptomeninges without finding vasculitis.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> Treatment consisted of immunosuppressants (methylprednisolone, cyclophosphamide and cyclosporin A); there was a good response in three cases.<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">8–10</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">In our case we could not identify any other cause or comorbidity that could explain the vasculopathy. The absence of other systemic autoimmune diseases suggests that the affliction of the CNS could be a complication per se of the lcSSc itself. Angiographic findings in SS may be the result of a reversible vasospasm of the CNS, a vascular disease of the CNS and, less frequently, because of vasculitis of the CNS. Such that every day there are more data supporting the fact that these disorders in the CNS may be a primary event of SS.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Horta-Baas G, Vergara-Sánchez I, Romero-Figueroa MS. Vasculopatía del sistema nervioso central en esclerosis sistémica limitada. Med Clin (Barc). 2016;146:282–283.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1082 "Ancho" => 999 "Tamanyo" => 174254 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Angio-resonance of the brain showing a narrowing of the middle cerebral artery (arrow) and of the anterior cerebral artery in its precommunicating segment (*).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:3 [ "comentario" => "pii: S0025-7753(15)00036-6 [Epub ahead of print]" "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Vasculopathy of large vessels in systemic sclerosis [Article in Spanish]" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "B. 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