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array:24 [ "pii" => "S2387020616300663" "issn" => "23870206" "doi" => "10.1016/j.medcle.2016.04.041" "estado" => "S300" "fechaPublicacion" => "2016-01-01" "aid" => "3333" "copyright" => "Elsevier España, S.L.U.. All rights reserved" "copyrightAnyo" => "2015" "documento" => "article" "crossmark" => 1 "subdocumento" => "fla" "cita" => "Med Clin. 2016;146:1-7" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 1 "PDF" => 1 ] "Traduccion" => array:1 [ "es" => array:19 [ "pii" => "S002577531500322X" "issn" => "00257753" "doi" => "10.1016/j.medcli.2015.04.029" "estado" => "S300" "fechaPublicacion" => "2016-01-01" "aid" => "3333" "copyright" => "Elsevier España, S.L.U." "documento" => "article" "crossmark" => 1 "subdocumento" => "fla" "cita" => "Med Clin. 2016;146:1-7" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 43 "formatos" => array:2 [ "HTML" => 11 "PDF" => 32 ] ] "es" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Original</span>" "titulo" => "Cribado de hipertensión pulmonar en una cohorte española de pacientes con esclerosis sistémica" "tienePdf" => "es" "tieneTextoCompleto" => "es" "tieneResumen" => array:2 [ 0 => "es" 1 => "en" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "1" "paginaFinal" => "7" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Screening of pulmonary hypertension in a Spanish cohort of patients with systemic sclerosis" ] ] "contieneResumen" => array:2 [ "es" => true "en" => true ] "contieneTextoCompleto" => array:1 [ "es" => true ] "contienePdf" => array:1 [ "es" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figura 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1576 "Ancho" => 2139 "Tamanyo" => 267615 ] ] "descripcion" => array:1 [ "es" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Sistemática de cribado de hipertensión pulmonar en pacientes con esclerodermia.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Francisco José García Hernández, María Jesús Castillo Palma, Enrique Montero Mateos, Rocío González León, José Eduardo López Haldón, Julio Sánchez Román" "autores" => array:6 [ 0 => array:2 [ "nombre" => "Francisco José" "apellidos" => "García Hernández" ] 1 => array:2 [ "nombre" => "María Jesús" "apellidos" => "Castillo Palma" ] 2 => array:2 [ "nombre" => "Enrique" "apellidos" => "Montero Mateos" ] 3 => array:2 [ "nombre" => "Rocío" "apellidos" => "González León" ] 4 => array:2 [ "nombre" => "José Eduardo" "apellidos" => "López Haldón" ] 5 => array:2 [ "nombre" => "Julio" "apellidos" => "Sánchez Román" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2387020616300663" "doi" => "10.1016/j.medcle.2016.04.041" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020616300663?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S002577531500322X?idApp=UINPBA00004N" "url" => "/00257753/0000014600000001/v1_201512260043/S002577531500322X/v1_201512260043/es/main.assets" ] ] "itemSiguiente" => array:19 [ "pii" => "S2387020616300602" "issn" => "23870206" "doi" => "10.1016/j.medcle.2016.04.035" "estado" => "S300" "fechaPublicacion" => "2016-01-01" "aid" => "3285" "copyright" => "Elsevier España, S.L.U." "documento" => "article" "crossmark" => 1 "subdocumento" => "fla" "cita" => "Med Clin. 2016;146:8-15" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 2 "PDF" => 2 ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Original article</span>" "titulo" => "Prognostic implications of extra-hepatic clinical manifestations, autoimmunity and microscopic nail capillaroscopy in patients with primary biliary cirrhosis" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "8" "paginaFinal" => "15" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Implicación pronóstica de las manifestaciones clínicas extrahepáticas, autoinmunidad y capilaroscopia ungueal microscópica en pacientes con cirrosis biliar primaria" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "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" => 3137 "Ancho" => 2943 "Tamanyo" => 312690 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Study protocol of patients with primary biliary cirrhosis. ANA: antinuclear antibodies; RA: rheumatoid arthritis; PBC: primary biliary cirrhosis; PBC-SAD: patients with PBC and associated SAD; PBC-no SAD: PBC patients without associated SAD; CCP: cyclic citrullinated anti-peptide antibody; SAD: systemic autoimmune disease; ENA: extractable nuclear antibody; SSc: systemic sclerosis; lSSc: SSc with limited scleroderma; lSSc-RA: SSc associated with limited scleroderma and rheumatoid arthritis; lSSc-SjS: SSc associated with limited scleroderma and Sjogren syndrome; RF: rheumatoid factor; pre-SSc: pre-scleroderma; SjS: Sjögren's syndrome; TSH: thyrotropin.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Begoña Marí-Alfonso, María José Amengual-Guedan, Mercè Vergara-Gómez, Carmen Pilar Simeón-Aznar, Vicente Fonollosa-Plà, Esther Jove-Buxeda, Juan Oliva-Morera, Carles Tolosa-Vilella" "autores" => array:8 [ 0 => array:2 [ "nombre" => "Begoña" "apellidos" => "Marí-Alfonso" ] 1 => array:2 [ "nombre" => "María José" "apellidos" => "Amengual-Guedan" ] 2 => array:2 [ "nombre" => "Mercè" "apellidos" => "Vergara-Gómez" ] 3 => array:2 [ "nombre" => "Carmen Pilar" "apellidos" => "Simeón-Aznar" ] 4 => array:2 [ "nombre" => "Vicente" "apellidos" => "Fonollosa-Plà" ] 5 => array:2 [ "nombre" => "Esther" "apellidos" => "Jove-Buxeda" ] 6 => array:2 [ "nombre" => "Juan" "apellidos" => "Oliva-Morera" ] 7 => array:2 [ "nombre" => "Carles" "apellidos" => "Tolosa-Vilella" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0025775315001918" "doi" => "10.1016/j.medcli.2015.02.026" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775315001918?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020616300602?idApp=UINPBA00004N" "url" => "/23870206/0000014600000001/v3_201605230108/S2387020616300602/v3_201605230108/en/main.assets" ] "asociados" => array:1 [ 0 => array:19 [ "pii" => "S2387020616300882" "issn" => "23870206" "doi" => "10.1016/j.medcle.2016.04.047" "estado" => "S300" "fechaPublicacion" => "2016-01-01" "aid" => "3365" "copyright" => "Elsevier España, S.L.U." "documento" => "article" "crossmark" => 1 "subdocumento" => "sco" "cita" => "Med Clin. 2016;146:21-3" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "en" => array:10 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Editorial article</span>" "titulo" => "Pulmonary hypertension in scleroderma" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "21" "paginaFinal" => "23" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Hipertensión pulmonar en la esclerodermia" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Vicent Fonollosa-Pla, Carmen Pilar Simeón-Aznar" "autores" => array:2 [ 0 => array:2 [ "nombre" => "Vicent" "apellidos" => "Fonollosa-Pla" ] 1 => array:2 [ "nombre" => "Carmen Pilar" "apellidos" => "Simeón-Aznar" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0025775315004224" "doi" => "10.1016/j.medcli.2015.07.002" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775315004224?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020616300882?idApp=UINPBA00004N" "url" => "/23870206/0000014600000001/v3_201605230108/S2387020616300882/v3_201605230108/en/main.assets" ] ] "en" => array:20 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Original article</span>" "titulo" => "Screening of pulmonary hypertension in a Spanish cohort of patients with systemic sclerosis" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "1" "paginaFinal" => "7" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Francisco José García Hernández, María Jesús Castillo Palma, Enrique Montero Mateos, Rocío González León, José Eduardo López Haldón, Julio Sánchez Román" "autores" => array:6 [ 0 => array:4 [ "nombre" => "Francisco José" "apellidos" => "García Hernández" "email" => array:1 [ 0 => "pacolageno@gmail.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" => "María Jesús" "apellidos" => "Castillo Palma" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Enrique" "apellidos" => "Montero Mateos" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "Rocío" "apellidos" => "González León" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "José Eduardo" "apellidos" => "López Haldón" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 5 => array:3 [ "nombre" => "Julio" "apellidos" => "Sánchez Román" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servivio de Medicina Interna, Hospital Universitario Virgen del Rocío, Sevilla, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Cardiología, Hospital Universitario Virgen del Rocío, Sevilla, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Cribado de hipertensión pulmonar en una cohorte española de pacientes con esclerosis sistémica" ] ] "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" => 1572 "Ancho" => 2136 "Tamanyo" => 255315 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Systematic screening of pulmonary hypertension in patients with scleroderma.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Pulmonary hypertension (PH) is characterized by a value of the mean pulmonary artery pressure (PAP) (mPAP) measured by right heart catheterization (RHC)<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>25<span class="elsevierStyleHsp" style=""></span>mmHg at rest, secondary to an obstacle located at any point of the pulmonary vascular circuit. Pulmonary arterial hypertension (PAH), a PH variant secondary to proliferation-obstruction of the pulmonary arterioles and characterized by an increase in PAP in the absence of involvement of the left side of the heart or pulmonary parenchymal or thromboembolic disease, is a serious complication and relatively common in patients with systemic sclerosis (SSc).<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">1</span></a> Initial studies found that the progression of these patients and their response to vasodilator treatment was worse than that observed in individuals with idiopathic PAH (IPAH). Later it was shown that the prognosis improved significantly if the diagnosis of PAH was established at an early stage (ideally, during functional class [FC] <span class="elsevierStyleSmallCaps">i</span>). In order to establish a diagnosis and appropriate treatment as early as possible, the clinical practice guidelines recommend performing periodic screening by Transthoracic Doppler echocardiography (TDE) in patients with SSc.<a class="elsevierStyleCrossRefs" href="#bib0170"><span class="elsevierStyleSup">2,3</span></a> A system defining the risk criteria for the development of PH was recently established (PHAROS registry)<a class="elsevierStyleCrossRefs" href="#bib0180"><span class="elsevierStyleSup">4,5</span></a> (systolic PAP [sPAP]<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>40<span class="elsevierStyleHsp" style=""></span>mmHg, diffusion of carbon monoxide [DLCO]<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>55% of the predicted value or a forced vital capacity [FVC]/DLCO ratio<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>1.6), which gets patients diagnosed in the early functional stages with survivals of 75% at 3 years. Following these guidelines, a screening program for early detection of PAH in a cohort of patients with SSc was designed and developed, which happened to be the first work published, in this sense, among Spanish population.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Patients and method</span><p id="par0010" class="elsevierStylePara elsevierViewall">In June 2003, a PAH screening program in a cohort of patients with controlled SSc was started in a unit which specializes in keeping both processes under control in the Virgen del Rocio hospital in Seville. Data collection was closed on June 30, 2014. In addition to the patients evaluated in this program, the patients studied before the start of the screening program for suspected PAH were also considered. For the diagnosis of SSc, the <span class="elsevierStyleItalic">American Rheumatism Association</span> 1980<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">6</span></a> criteria were used, initially. Depending on the type of skin involvement, the disease was classified as diffuse SSc (dcSSc), with skin sclerosis distal and proximal to elbow and knee, and limited SSc (lcSSc), with cutaneous sclerosis limited proximally by elbows and knees. It can also affect face and neck. Patients without skin involvement but with vascular impairment (defined by Raynaud's phenomenon or anomalies in nailfold capillaroscopy) and positivity for SSc specific autoantibodies (anticentromere [ACA] or antitopoisomerase <span class="elsevierStyleSmallCaps">i</span> [ATA-I]) were considered affected by “sine scleroderma SSc” (ssSSc) if there was evidence of established visceral condition characteristic of SSc (gastrointestinal hypomotility, interstitial lung disease [ILD], PAH, scleroderma renal crisis or heart disease), or “pre-scleroderma” (pre-SSc) in case of suffering from Raynaud's phenomenon with SSc specific antibodies and/or capillaroscopic anomalies.<a class="elsevierStyleCrossRefs" href="#bib0195"><span class="elsevierStyleSup">7,8</span></a> Determining autoantibodies both antinuclear (ANA) as well as specific to SSc (ACA y ATA-I) was performed by immunofluorescence using HEp-2 as substrate (ANA and ACA) and by immunodiffusion or counterimmunoelectrophoresis (ATA-I). Patients were systematically assessed each year in the case of remaining asymptomatic, but that review was brought forward if the symptoms appeared. Systematic screening is summarized in <a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>. In each review, the presence of PAH symptoms or signs (dyspnoea, angina, syncope, and right heart failure) was evaluated. A TDE, a chest X-ray and respiratory function tests (RFTs) with determination of FVC and DLCO were performed in all patients yearly. More recently, the protocol included the <span class="elsevierStyleItalic">N-terminal pro-brain natriuretic peptide</span> (NT-proBNP) determination. It was considered that the sPAP, evaluated by TDE, was high if its value (resulting from the formula sPAP<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>4V<span class="elsevierStyleSup">2</span><span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>DBP; V being the maximum speed of tricuspid regurgitation [TRV] and DBP the mean right atrial pressure, evaluated according to the echocardiographer's interpretation) was higher than 35<span class="elsevierStyleHsp" style=""></span>mmHg.<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">2</span></a> Depending on the clinical and echocardiographic data (sPAP<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>50<span class="elsevierStyleHsp" style=""></span>mmHg, sPAP<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>35<span class="elsevierStyleHsp" style=""></span>mmHg in the presence of unexplained dyspnoea or a FVC/DLCO ratio<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>1.8, or, more recently, a value of NT-proBNP<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>200<span class="elsevierStyleHsp" style=""></span>pg/ml) it was decided to continue monitoring (when the conditions mentioned were met) or initiate a standardized study to establish or rule out a definitive diagnosis of PAH (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The assessment of potential ILD was carried out by chest radiography, RFTs (FVC<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>70% of predicted value) and high resolution computed tomography (HRCT), if considered appropriate; the assessment of venous thromboembolism was performed by pulmonary scintigraphy and/or angio-CT of the thorax; other tests (such as polysomnography) if the medical history recommended it. Finally, we conducted a RHC to establish or exclude the diagnosis of PH with certainty, excluding only those patients in whom it was considered that the PH was undoubtedly associated with left heart failure (clinical and radiologic assessment and indicative TDE data, such as ejection fraction of the left ventricle [LVEF]<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>50% as an expression of systolic dysfunction, mitral or aortic valvular dysfunction of at least moderate grade or significant parameters of diastolic dysfunction according to the echocardiographer) or with advanced pulmonary fibrosis (significant changes in HRCT with FVC<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>70% and FVC/DLCO ratio<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>1.8). The existence of PH was considered by RHC when the mPAP at rest was ≥25<span class="elsevierStyleHsp" style=""></span>mmHg, distinguishing between precapillary PH when pulmonary capillary pressure (PCP) was ≤15<span class="elsevierStyleHsp" style=""></span>mmHg, and when the postcapillary PCP was >15<span class="elsevierStyleHsp" style=""></span>mmHg. PAH was considered confirmed when the requirements for precapillary PH were met in the RHC, with FVC<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>70% or DLCO value disproportionately low in relation to that one's value (FVC/DLCO<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>1.8). PAH was considered potential when patients had a sPAP value >35<span class="elsevierStyleHsp" style=""></span>mmHg and ≤50<span class="elsevierStyleHsp" style=""></span>mmHg in the TDE, being asymptomatic and without evidence of significant respiratory or cardiac dysfunction. In patients with exertional dyspnoea not justified by any other circumstance in which the sPAP at rest was normal, a stress TDE (lateral position modifiable cycle ergometer, model Variobike 2000<span class="elsevierStyleSup">®</span>, Schiller) was performed with protocol by stages every 3<span class="elsevierStyleHsp" style=""></span>min, starting with a power of 25<span class="elsevierStyleHsp" style=""></span>W, increased by 25<span class="elsevierStyleHsp" style=""></span>W at each stage. If in the course of the test, the 50<span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>mmHg were exceeded, with a ratio between the peak value of tricuspid regurgitation (reflecting pressure values) and velocity–time integral of the outflow tract of the right ventricle (TRV/<span class="elsevierStyleInf">VTI</span>, giving an indirect measure of cardiac output)<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>0.2 (ratio value that guides towards increase resistance and not to cardiac output as the cause of PH) exercise-induced PAH diagnosis was established.<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">9</span></a> The elevation of the PAP during exercise measured through TDE was confirmed by stress RHC.<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">10</span></a> All patients gave their informed consent in accordance with the regulations of the ethics committee. We used the SPSS<span class="elsevierStyleSup">®</span> v22.0 software for the statistical analysis of data. For quantitative variables (age, time to diagnosis or exclusion of PAH) the mean was calculated (standard deviation [SD]) and the median (range between percentiles with p25–p75 [Q1–Q3]). None of these variables adopted a normal distribution (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0.001), as shown by the Shapiro–Wilk test, therefore, comparisons were made (between median) using the nonparametric Mann–Whitney <span class="elsevierStyleItalic">U</span> test and not (between mean) by the <span class="elsevierStyleItalic">t</span> Student parametric test. When the calculated effectiveness was below 5, Fisher's <span class="elsevierStyleItalic">χ</span><span class="elsevierStyleSup">2</span> test with correction was used for qualitative variables. It was considered significant, with a confidence interval of 95%, a “2-tail” <span class="elsevierStyleItalic">p</span> value<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0.05.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Results</span><p id="par0015" class="elsevierStylePara elsevierViewall">The existence of PH was evaluated by TDE in 184 patients in a cohort of 232 with SSc (79.3%). 88.6% (163) were women. The mean age at baseline was 57.8 years (1315<span class="elsevierStyleHsp" style=""></span>SD), with a median of 58.5 years (Q1–Q3 48–69) and the disease progression mean time (considered from the first symptom compatible with SSc) was 12.63 years (10.98), with a median of 10 years (Q1–Q3 5–17.75). The disease variants were: lcSSc in 100 patients (54.3%), dcSSc in 60 (32.6%) and ssSSc/ps in 24 (13%). A high value of sPAP was detected (pre-set limit<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>35<span class="elsevierStyleHsp" style=""></span>mmHg) by TDE in 64 patients (34.78%). PAH was discarded during the diagnostic process prior to the RHC in 19 patients: 5 due to the set of clinical, radiographic and echocardiographic data of systolic (LVEF<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>50%) or diastolic (relaxation data abnormal) dysfunction of the left ventricle or left valvulopathy (in all significant cases), 4 severe ILD, with FVC<span class="elsevierStyleHsp" style=""></span>≤<span class="elsevierStyleHsp" style=""></span>45% and proportional reduction in the DLCO, and 9 after checking that the value of the sPAP was normal in subsequent TDE studies. Another patient died of acute respiratory failure. This patient had a sPAP value of 70<span class="elsevierStyleHsp" style=""></span>mmHg, measured by TDE. A study by lung scintigraphy showed a high probability of pulmonary embolism. A complete diagnostic evaluation was made according to the recommended algorithm (with RHC) in 31 of the 64 patients with elevated sPAP in TDE (48.4%). The diagnosis of chronic thromboembolic PH in one patient and PAH in 25 (13.58% of all evaluated; 39.06% of patients with elevated sPAP in TDE, 80.64% of those undergoing RHC) was confirmed. Two of them (1.1% of the total number) had exertional dyspnoea, with a sPAP (in TDE) in the normal range during rest, which increased significantly during exercise (45 and 75<span class="elsevierStyleHsp" style=""></span>mgHg, respectively), with a TRV/VTI ratio<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>0.2. MPAP in these 2 patients, estimated by RHC, at rest, was 21 and 24<span class="elsevierStyleHsp" style=""></span>mmHg, respectively <span class="elsevierStyleItalic">(borderline)</span>, and increased to 38<span class="elsevierStyleHsp" style=""></span>mmHg during exercise. It was considered that both had exercised-induced PAH.<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">11</span></a> Seven patients with PAH (28%) had some degree of ILD associated, but RFTs data supported a predominantly vascular involvement (FVC<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>70% or DLCO value disproportionately low in relation to that one's value [FVC/DLCO<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>1.8]). The diagnosis of the other 5 patients undergoing RHC was diastolic heart failure in 2 cases and absence of PH in the other 3. These data are summarized in <a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>. Finally, there are 14 patients with sPAP value of >35<span class="elsevierStyleHsp" style=""></span>≤<span class="elsevierStyleHsp" style=""></span>50<span class="elsevierStyleHsp" style=""></span>mmHg in TDE that remain asymptomatic and are in regular clinical and echocardiographic follow-up (considered “potentially affected” of PAH); 5 of them have some degree of associated ILD, with a FVC value of >70% in all of them and a disproportionately low DLCO value (FVC/DLCO<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>1.8) in only one.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Patients with potential or confirmed pulmonary arterial hypertension</span><p id="par0020" class="elsevierStylePara elsevierViewall">Let's consider the 39 patients with potential (the group of 14 patients previously defined as “potentially affected” of PAH) or confirmed (the group of 25 patients with confirmed PAH after concluding the standardized diagnostic study) PAH. They represent 21.2% of the patients studied: 23 with lcSSc (59%, corresponding to 23% of patients evaluated with limited forms) and 16 with dcSSc (41%, corresponding to 26.67% of patients evaluated with diffuse forms). Their age is significantly more advanced with respect to patients without PAH: median 66 (Q1–Q3 59–71) versus 56 years (Q1–Q3 46.5–68); <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.001. When subgroups are analyzed, it is observed that this difference occurs at the expense of lcSSc: median 69 (Q1–Q3 62–72) versus 55 (Q1–Q3 47–69.4) in unaffected (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.002), with no significant differences in the dcSSc group: 60.5 (Q1–Q3 53–63.5) versus 57 years (Q1–Q3 from 53.3 to 66.5), <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.53. By contrast, no significant association was found between the existence of potential or confirmed PAH and other variables such as sex (female in 34/39 [87.18%] vs 129/145 [88.97%]), the different autoantibodies (ACA 22/39 [57%] vs 70/145 [48%] and ATA- <span class="elsevierStyleSmallCaps">I</span> in 7/39 [18%] vs 21/145 [14.5%]) or the progression time to diagnosis or exclusion of PAH: 9 (Q1–Q3 5–24) versus 10 (Q1–Q3 5–16) in patients without PAH. Regarding the type of SSc, no significant differences were observed for the frequency of lcSSc (23/39 [59%] against 77/145 [53.1%]; <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.59) and dcSSc (16/39 [41%] vs 44/145 [30.3%]; <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span> 0.25) compared to patients without PAH. By contrast, no progression towards PAH was observed in any cases of ssSSc/ps (0 vs. 24/145 [16.55%]; <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0.001). The median time of disease progression to diagnosis of potential or confirmed PAH was 9 years (Q1–Q3 5–25) for patients with lcSSc, and 14 years (Q1–Q3 from 5.25 to 23.75) for those with dcSSc, which is non-significant difference (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.77).</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Patients with confirmed pulmonary arterial hypertension</span><p id="par0025" class="elsevierStylePara elsevierViewall">If we consider only the group of patients with definitive PAH (25 patients, 13.58% of all evaluated, including 2 with exercise-induced PAH), their age at the time of confirming or rejecting the diagnosis was significantly higher than in patients without PAH: median 67 (Q1–Q3 59–70.5) versus 56 years (Q1–Q3 from 46.5 to 68); <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.003. Again, this significance depended on the limited forms, with a median of 67.5 (Q1–Q3 from 61.3 to 71.8) versus 55 (Q1–Q3 47–69.5), <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.0025, and not on the diffuse forms, with a median of 62 (Q1–Q3 56–68.5) versus 57 years (Q1–Q3 53.3–66.55), <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.24. The median time regarding progression of the disease to diagnosis or exclusion was somewhat shorter in those affected: median 8 (Q1–Q3 from 4.5 to 23) compared to 10 years (Q1–Q3 5–16), although the difference did not reach statistical significance (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.73). No significant differences were observed in the median time of progression considering lcSSc (9 [Q1–Q3 from 5.3 to 25] vs 11 years [Q1–Q3 from 6.5 to 17] [<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.73]) and dcSSc (6 [Q1–Q3 from 3.5 to 21.5] versus 9.5 years [Q1–Q3 5–17.5] [<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.54] separately, or between the two (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.52). No significant association between the presence of PAH and sex (female in 20/25 [80%] vs 129/145 [88.9%], <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.17) was found, or differences in the frequency of the SSc type compared to the group without PAH (lcSSc in 16/25 [64%] vs 77/145 [53.1%], <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.39; dcSSc in 9/25 [36%] vs 44/145 [30.3%], <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.82), except for the ssSSc/ps variant, which, as noted, there was no case of PAH development. Regarding the variety of autoantibodies, a higher frequency of ACA (16/25 [64%] vs. 70/145 [48.3%] was observed; <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.19) and lower ATA-I (3/25 [12%] vs 21/145 [14.5%]; <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.5) in patients with PAH, but none of those differences reached statistical significance. The results are summarized in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia></span></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Discussion</span><p id="par0030" class="elsevierStylePara elsevierViewall">The development of PAH has a negative impact on life expectancy in patients with SSc.<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">1</span></a> The expected survival in them (and in general in the group of patients with PAH associated with systemic autoimmune diseases [SAD]) is worse than in those affected by IPAH.<a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">12</span></a> The efficacy of vasodilator therapy in both groups of patients is similarly controversial. Kuhn et al.,<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">13</span></a> found that patients with PAH and SSc had a lower survival than patients with IPAH in response to prolonged treatment with epoprostenol. Kawut et al.<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">14</span></a> also found decreased survival in a group of 22 patients with related to the PAH's spectrum compared with that of 33 patients with IPAH, with a similar treatment. In contrast, Mukerjee et al.,<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">15</span></a> observed that the cumulative survival of 148 patients with PAH associated with SSc did not differ from that observed in patients with IPAH when treated early and actively; the increased pressure of the right atrium was associated with increased mortality. Therefore, it is recommended to begin treatment as early as possible (ideally in FC I) to prevent right ventricular decompensation. Later, a favourable response was found with bosentan,<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">16</span></a> sitaxentan,<a class="elsevierStyleCrossRef" href="#bib0245"><span class="elsevierStyleSup">17</span></a> sildenafil<a class="elsevierStyleCrossRef" href="#bib0250"><span class="elsevierStyleSup">18</span></a> or treprostinil<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">19</span></a> in patients with PAH associated with SSc. Certain markers may predict the development of PAH in SSc patients, contributing to early diagnosis: limited SSc forms, postmenopause, some HLA markers HLA (B35, DRw6, DRw52), anti-U3-RNP and anti-B23 antibodies, plasmin-α-inhibitor complex or increased NT-proBNP, although they are not useful for detection in early stages.<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">20</span></a> The presence of dyspnoea is considered a good predictor of PAH in SSc patients.<a class="elsevierStyleCrossRef" href="#bib0265"><span class="elsevierStyleSup">21</span></a> In a previous assessment of patients belonging to this study series (unpublished data) the presence of dyspnoea was significantly associated with the risk of PH (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0.001). In our experience, the absence of dyspnoea allowed to define a subgroup of patients at low risk for PAH, with a negative predictive value of 94%: only one patient in our series, with definitive diagnosis of PAH, has remained asymptomatic (without dyspnoea) at all times, a fact that we consider very important to optimize the use of resources, but we must take into account that the presence of dyspnoea may be a “too late” marker.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The RHC is the undisputed method for the diagnosis of PAH. However, its use as a screening method has significant disadvantages (it is an invasive procedure, costly and not without risk). The TDE is a method for performing a first evaluation, the usefulness and good correlation with the RHC have been previously contrasted.<a class="elsevierStyleCrossRefs" href="#bib0270"><span class="elsevierStyleSup">22,23</span></a> However, suspicion of PH, established by TDE, in patients with SSc requires careful differential diagnosis and must be confirmed, inexcusably, by RHC, as in any other category of PH. Moreover, although we can establish TDE suspicion of PH, it is not synonymous with PAH. In patients with SAD, especially SSc, in addition to this variant (PAH; group 1 Nice) other forms of PH can be developed; veno-occlusive pulmonary disease (PVOD, group 1′); secondary to left heart disease (group 2); secondary to ILD (group 3); and chronic thromboembolic (group 4), the latter especially in patients who have antiphospholipid antibodies. The measurement of PCP can discriminate between postcapillary PH (PCP<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>15<span class="elsevierStyleHsp" style=""></span>mmHg), caused by anomalies of the “left heart” and precapillary PH (PCP<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>15<span class="elsevierStyleHsp" style=""></span>mmHg). In the latter case, it is necessary to supplement the RHC findings with other complementary tests: Chest CT, angio-CT, lung scintigraphy, RFTs, angiography, etc., to discriminate between the different PH variants already mentioned. It is especially important to distinguish between PAH and PH secondary to ILD (so prevalent in SSc) or left heart disease, as treatment with vasodilators (effective in PAH) in these other cases is, not only pointless, but harmful. The existence of “disproportionate” PH (a term we use to simplify, but has been better defined at the recent Nice meeting) in patients of groups 2 and 3 also raises complex issues of treatment.<a class="elsevierStyleCrossRefs" href="#bib0270"><span class="elsevierStyleSup">22,23</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The prevalence of PAH in SAD is very variable as reported in different papers (<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>). This is due, among other causes, to the misuse of terminology, considering PH and PAH equivalents. For SSc, the frequencies reported by Pope et al.,<a class="elsevierStyleCrossRef" href="#bib0280"><span class="elsevierStyleSup">24</span></a> (33%), MacGregor et al.,<a class="elsevierStyleCrossRef" href="#bib0285"><span class="elsevierStyleSup">25</span></a> (13%) or Wigley et al.,<a class="elsevierStyleCrossRef" href="#bib0290"><span class="elsevierStyleSup">26</span></a> (26.7%) are based solely on data provided by TDE, a method which, by itself, as mentioned above, is not only insufficient for diagnosing PAH,<a class="elsevierStyleCrossRef" href="#bib0295"><span class="elsevierStyleSup">27</span></a> but even for the confirmed diagnosis of PH; in addition, the last author included in his series both, SSc patients as well as mixed connective tissue disease patients.<a class="elsevierStyleCrossRef" href="#bib0290"><span class="elsevierStyleSup">26</span></a> Mukerjee et al.,<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">15</span></a> and Vonk et al.,<a class="elsevierStyleCrossRef" href="#bib0300"><span class="elsevierStyleSup">28</span></a> which include the assessment using RHC, report a frequency of 12% and 10% in their respective series, but considered the PAH finding as nothing more than pre-capillary PH, and in the Hachulla et al.<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">29</span></a> series (frequency of 8%) patients with ILD are excluded from the study. The results of a recent Spanish multicenter study<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">7</span></a> in which we have participated, are very interesting because they analyze the frequency of PH in the different variants of cutaneous involvement of a very large series of patients (916) with SSc: 13.7% in the dcSSc, 8.8% in lcSSc and 7.1% in the ssSSc, although the different categories of PH are not clearly separated in this study. Conversely, Avouac et al.,<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">30</span></a> published in 2010 a rigorous multicenter study that quantified the different variants of PH in 206 patients (from a range of 1,165) with SSc and suspected PH. Precapillary PH was found in 64 cases (5% of the total number), of which 42 were classified as suffering from PAH (3.6%). PH due to ILD was detected in 22 patients (1.8%); postcapillary HP (secondary to left heart disease) in 17 (2%), and associated with PVOD in one (0.08%).</p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0045" class="elsevierStylePara elsevierViewall">We have used a systematic method similar to that of Avouac et al.,<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">30</span></a> in the follow-up of 184 patients with SSc, in our series, subject to annual monitoring by TDE. Those in which PH suspicion was established, using Hachulla et al's echocardiographic criteria (TRV<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>3<span class="elsevierStyleHsp" style=""></span>m/s TRV<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>2,5–3<span class="elsevierStyleHsp" style=""></span>m/s with unexplained dyspnoea)<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">29</span></a> and including patients with unexplained dyspnoea and also FVC/DLCO ratio<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>1.8, underwent RHC. PH's presence by TDE was found in 64 patients (34.8% of the series); most of them (25; 39.06% of patients with PH by TDE and 13.6% of the series) with PAH (once other different PH causes were discarded, mainly ILD or left ventricular dysfunction). This frequency, which could reach 21% (if those considered “potentially” affected of PAH are included) is considerably higher than that reported by Avouac et al.,<a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">30</span></a> but in the DETECT study published recently, the frequency of PAH in these patients reached a 19%, very close to that of our series.<a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">31</span></a> The delimitation between vascular and parenchymal disease among patients with SSc (even after excluding patients clearly belonging to group 3) is not always clear. In most patients that can be considered affected by PAH we observe minor radiological or respiratory functional anomalies (or even intense), the latter ones characterized by an increase (>1.8) FVC/DLCO ratio or a disproportionate increase in mPAP (all of which suggests a PAH associated component).</p><p id="par0050" class="elsevierStylePara elsevierViewall">Apart from the already mentioned presence of dyspnoea, the only clinical-epidemiological data that characterized the subgroup with PAH in this study was older age (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.001), a fact that is only evident in the lcSSc variant (both, whether we include the “potential-confirmed” group or whether we limit ourselves to evaluate patients with confirmed PAH). There was no relation found regarding gender or “longer-shorter” progression time (although a shorter progression trend was observed in patients with PAH). ACA frequency was higher in patients with confirmed PAH (64 versus 48%) than in those without PAH, but the difference did not reach statistical significance (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span> 0.19). Although the lcSSc frequency was higher than dcSSc among patients who developed PAH, these differences are proportional to those observed in the whole series. No association of PAH was observed in any ssSSc/ps case, although there are documented cases of this association.<a class="elsevierStyleCrossRefs" href="#bib0195"><span class="elsevierStyleSup">7,32</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Our work has as main limitations the fact that the first stage of diagnosis is the assessment by TDE along with the data provided by the RFTs, basically, and not having done a RHC in the entire group of patients considered, so there may be an underestimation of the prevalence of PAH in them, although this approach is common to most studies (excluding DETECT,<a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">31</span></a> although it finds a similar incidence to that found by us).</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Conflict of interests</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest related to this work.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres640216" "titulo" => "Abstract" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Background and objective" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Patients and method" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusions" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec654269" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres640215" "titulo" => "Resumen" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Fundamento y objetivo" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Pacientes y método" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusiones" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec654270" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Patients and method" ] 6 => array:3 [ "identificador" => "sec0015" "titulo" => "Results" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0020" "titulo" => "Patients with potential or confirmed pulmonary arterial hypertension" ] 1 => array:2 [ "identificador" => "sec0025" "titulo" => "Patients with confirmed pulmonary arterial hypertension" ] ] ] 7 => array:2 [ "identificador" => "sec0030" "titulo" => "Discussion" ] 8 => array:2 [ "identificador" => "sec0035" "titulo" => "Conflict of interests" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2014-11-25" "fechaAceptado" => "2015-04-09" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec654269" "palabras" => array:3 [ 0 => "Systemic sclerosis" 1 => "Pulmonary hypertension" 2 => "Screening" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec654270" "palabras" => array:3 [ 0 => "Esclerosis sistémica" 1 => "Hipertensión pulmonar" 2 => "Cribado" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Background and objective</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Pulmonary arterial hypertension (PAH) is an important cause of morbimortality in systemic sclerosis (SSc). Evolution is worse than that of subjects with idiopathic PAH, but prognosis improves when PAH is diagnosed early. The aim of this research is to describe results of a screening program for diagnosis of pulmonary hypertension (PH) carried out in a cohort of Spanish patients with SSc.</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Patients and method</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">PH screening was performed by transthoracic Doppler echocardiography (TTDE) in 184 patients with SSc. Patients with systolic pulmonary arterial pressure estimated by TTDE<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>35<span class="elsevierStyleHsp" style=""></span>mmHg were evaluated per protocol to confirm diagnosis and type of PH.</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">PAH was diagnosed in 25 patients (13.6%). Patients with diffuse and limited SSc developed PAH in a similar degree, 9/60 (15%) vs. 16/100 (16%), with no cases among patients with SSc “sine scleroderma” or “pre-scleroderma” (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>.001). The only clinical or epidemiological data characterizing patients with PAH were older age (mean age 67 years for patients with PAH vs. 56 years for those without PAH, <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>.007), limited SSc, a trend towards shorter evolution of the underlying disease (median 8 years for patients with PAH vs. 10 years for those without PAH, <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>.73), and a higher frequency of positive anticentromere antibodies (16 patients [64%] with PAH vs. 70 (48.3%) without PAH, <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>.19).</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclusions</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Prevalence of PAH in SSc was high and supports the implementation of a regular screening program.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Background and objective" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Patients and method" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusions" ] ] ] "es" => array:3 [ "titulo" => "Resumen" "resumen" => "<span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Fundamento y objetivo</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">La hipertensión arterial pulmonar (HAP) es causa importante de morbimortalidad en la esclerosis sistémica (ES). Su evolución es peor que en la HAP idiopática, pero el pronóstico mejora si se diagnostica precozmente. El objetivo de este trabajo es describir el resultado de un programa de cribado para el diagnóstico de hipertensión pulmonar (HP) desarrollado en una cohorte de pacientes españoles con ES.</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Pacientes y método</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Se realizó cribado de HP mediante ecocardiografía-doppler transtorácica (EDTT) en 184 pacientes con ES. Los pacientes con valor de presión arterial pulmonar sistólica estimada por EDTT<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>35<span class="elsevierStyleHsp" style=""></span>mmHg se evaluaron de forma protocolizada para establecer o no el diagnóstico de certeza de HP y su tipo.</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Se diagnosticó HAP en 25 pacientes (13,6%). Los pacientes con ES difusa y limitada desarrollaron HAP en proporciones semejantes: 9 de 60 (15%) frente a 16 de 100 (16%). No se registraron casos entre pacientes con ES «sine esclerodermia» o «preesclerodermia» (p<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0,001). Los únicos datos clinicoepidemiológicos que caracterizaron a los pacientes con HAP fueron una edad más avanzada (edad media de 67 años para pacientes con HAP frente a 56 años sin HAP, p<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0,007), especialmente relacionada con la ES limitada, y una tendencia hacia un menor tiempo de evolución de la enfermedad de base (mediana de 8 años para pacientes con HAP frente a 10 años sin HAP, p<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0,73) y una mayor frecuencia de positividad para anticuerpos anticentrómero: 16 (64%) pacientes con HAP frente a 70 (48,3%) sin HAP (p<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0,19).</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclusiones</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">La prevalencia de HAP en ES resultó elevada y apoya la implantación de programas de cribado sistemático.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Fundamento y objetivo" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Pacientes y método" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusiones" ] ] ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0020">Please cite this article as: García Hernández FJ, Castillo Palma MJ, Montero Mateos E, González León R, López Haldón JE, Sánchez Román J. Cribado de hipertensión pulmonar en una cohorte española de pacientes con esclerosis sistémica. Med Clin (Barc). 2016;146:1–7.</p>" ] ] "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1572 "Ancho" => 2136 "Tamanyo" => 255315 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Systematic screening of pulmonary hypertension in patients with scleroderma.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 2139 "Ancho" => 2848 "Tamanyo" => 387156 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Results of systematic screening for pulmonary hypertension in the evaluated cohort of patients.</p>" ] ] 2 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:3 [ "leyenda" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">ACA, anticentromere antibodies; ATA-I, antitopoisomerase I antibodies; dcSSc, diffuse cutaneous systemic sclerosis; lcSSc, limited cutaneous systemic sclerosis; ssSSc/ps, “sine scleroderma”/“pre-scleroderma” systemic sclerosis; PAH, pulmonary arterial hypertension; NS, statistically not significant difference.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Whole group \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Without PAH \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Potential/confirmed PAH \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Confirmed PAH \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Patients, n (%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">184 (100) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">145 (78.8) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">39 (21.2) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">25 (13.6) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Sex (n, % women) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">163 (88.6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">129 (88.9) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">34 (87.1), <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>NS<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">20 (80), <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>NS<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Age in years, median (Q1–Q3) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">58.5 (48–69) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">56 (46.5–68) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">66 (59–71) <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.001<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a><span class="elsevierStyleSup">,</span><a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">b</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">67 (59–70.5), <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.003<a class="elsevierStyleCrossRefs" href="#tblfn0005"><span class="elsevierStyleSup">a,b</span></a> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">SSc variants, n (%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">lcSSc: 100 (54.3)<br>dcSSc: 60 (32.6)<br>ssSSc/ps: 24 (13) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">lcSSc: 77 (53.1)<br>dcSSc: 44 (30.3)<br>ssSSc/ps: 24 (16.5) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">lcSSc: 23 (59), <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>NS<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a><br>dcSSc: 16 (41), <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>NS<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a><br>ssSSc/ps: 0 (0) <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0.001<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">lcSSc: 16 (64), <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>NS<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a><br>dcSSc: 9 (36), <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>NS<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a><br>ssSSc/ps: 0 (0) <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0.001<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Years up to PAH diagnosis/exclusion, median (Q1–Q3) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">10 (5–17.8) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">10 (5–16) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">9 (5–24), <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>NS<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a><span class="elsevierStyleSup">,</span><a class="elsevierStyleCrossRef" href="#tblfn0015"><span class="elsevierStyleSup">c</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">8 (5–23), <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>NS<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a><span class="elsevierStyleSup">,</span><a class="elsevierStyleCrossRef" href="#tblfn0015"><span class="elsevierStyleSup">c</span></a> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Autoantibodies, % \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">ACA 50, ATA-I: 15.2 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">ACA 48.3, ATA-I 14.5 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">ACA 57, <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>NS<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a>, ATA-I 18, <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>NS<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">ACA 64, <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>NS<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a>, ATA-I 12, <span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>NS<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1053348.png" ] ] ] "notaPie" => array:3 [ 0 => array:3 [ "identificador" => "tblfn0005" "etiqueta" => "a" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Regarding group without pulmonary arterial hypertension.</p>" ] 1 => array:3 [ "identificador" => "tblfn0010" "etiqueta" => "b" "nota" => "<p class="elsevierStyleNotepara" id="npar0010">Statistical significance was retained only for limited cutaneous systemic sclerosis.</p>" ] 2 => array:3 [ "identificador" => "tblfn0015" "etiqueta" => "c" "nota" => "<p class="elsevierStyleNotepara" id="npar0015">No statistical significance for variants of systemic sclerosis.</p>" ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Summary of main results.</p>" ] ] 3 => array:8 [ "identificador" => "tbl0010" "etiqueta" => "Table 2" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at2" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0070" class="elsevierStyleSimplePara elsevierViewall">RHC, right heart catheterization; Echo, transthoracic echocardiography; EMTC, mixed connective tissue disease; ILD, interstitial lung disease; DS, differential study of other causes of pulmonary hypertension; PAH, pulmonary arterial hypertension; PH, pulmonary hypertension.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Authors, year \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Prevalence of PAH, % \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Actual determination \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Pope et al.,<a class="elsevierStyleCrossRef" href="#bib0280"><span class="elsevierStyleSup">24</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">33 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PH (Echo) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mukerjee et al.<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">15</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">12 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Precapillary PH (RHC) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">MacGregor et al.<a class="elsevierStyleCrossRef" href="#bib0285"><span class="elsevierStyleSup">25</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">13 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PH (Echo) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hachulla et al.<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">29</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">8 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PAH (RHC). But exclude patients with ILD \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Wigley et al.<a class="elsevierStyleCrossRef" href="#bib0290"><span class="elsevierStyleSup">26</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">26.7 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">PH (Echo). MCTD patients included \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Vonk et al.<a class="elsevierStyleCrossRef" href="#bib0300"><span class="elsevierStyleSup">28</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">10 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Precapillary PH (RHC) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">Avouac et al.</span><a class="elsevierStyleCrossRef" href="#bib0310"><span class="elsevierStyleSup">30</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top"><span class="elsevierStyleBold">3.6</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top"><span class="elsevierStyleBold">PAH (RHC; DS)</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">Coghlan et al.</span><a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">31</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top"><span class="elsevierStyleBold">19</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top"><span class="elsevierStyleBold">PAH (RHC; DS)</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">Present study</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top"><span class="elsevierStyleBold">13.6</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top"><span class="elsevierStyleBold">PAH (RHC; DS)</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1053347.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Studies on the prevalence of pulmonary hypertension in patients with systemic sclerosis.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:32 [ 0 => array:3 [ "identificador" => "bib0165" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "E.T. 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