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The Many Radiological Presentations of Pulmonary Nodules in Type IV Ehlers-Danlos Syndrome
Las muchas caras radiológicas de los nódulos pulmonares en el síndrome de Ehlers-Danlos tipo iv
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B) Imagen coronal de la TC de tórax (ventana de parénquima pulmonar) realizada en 2014 en la que visualiza la aparición de 2 nódulos en pulmón derecho: uno sólido (flecha) y otro subsólido de atenuación predominante en vidrio deslustrado con un pequeño centro sólido (asterisco). C) Imagen axial de la TC de tórax (ventana de parénquima pulmonar) realizada en 2016 en la que se demuestra la aparición de 2 nódulos pulmonares cavitados en vértice pulmonar derecho (flechas). Nótese que el nódulo más lateral presenta una cavitación excéntrica con morfología en semiluna. D) Imagen axial de la TC de tórax (ventana de mediastino) realizada en 2017 en la que se observa la aparición de una calcificación lineal (flecha) en el interior de un nódulo sólido en el lóbulo medio.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Luis Gorospe Sarasúa, Gemma María Muñoz-Molina, Paola Arrieta" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Luis" "apellidos" => "Gorospe Sarasúa" ] 1 => array:2 [ "nombre" => "Gemma María" "apellidos" => "Muñoz-Molina" ] 2 => array:2 [ "nombre" => "Paola" "apellidos" => "Arrieta" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S1579212918303379" "doi" => "10.1016/j.arbr.2018.10.006" "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/S1579212918303379?idApp=UINPBA00003Z" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289618302102?idApp=UINPBA00003Z" "url" => "/03002896/0000005400000012/v1_201812020626/S0300289618302102/v1_201812020626/es/main.assets" ] ] "itemSiguiente" => array:19 [ "pii" => "S1579212918303367" "issn" => "15792129" "doi" => "10.1016/j.arbr.2018.10.005" "estado" => "S300" "fechaPublicacion" => "2018-12-01" "aid" => "1931" "copyright" => "SEPAR" "documento" => "article" "crossmark" => 1 "subdocumento" => "sco" "cita" => "Arch Bronconeumol. 2018;54:627" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 343 "formatos" => array:3 [ "EPUB" => 70 "HTML" => 162 "PDF" => 111 ] ] "en" => array:11 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Clinical Image</span>" "titulo" => "High Resolution Computed Tomography and Fiberoptic Bronchoscopy in the Study of Severe Asthma" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:1 [ "paginaInicial" => "627" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "La tomografía computarizada de alta resolución y la fibrobroncoscopia en el estudio del asma grave" ] ] "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" => 1455 "Ancho" => 1583 "Tamanyo" => 403875 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Chest HRCT, axial slice: image of calcification in the righter lower lobe. (B) Chest HRCT, coronal slice. (C) Bronchoscopy image of foreign body. (D) Pathology image consistent with bone tissue.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "David Clofent, Irene Sansano, Iñigo Ojanguren" "autores" => array:3 [ 0 => array:2 [ "nombre" => "David" "apellidos" => "Clofent" ] 1 => array:2 [ "nombre" => "Irene" "apellidos" => "Sansano" ] 2 => array:2 [ "nombre" => "Iñigo" "apellidos" => "Ojanguren" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0300289618302084" "doi" => "10.1016/j.arbres.2018.05.018" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289618302084?idApp=UINPBA00003Z" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1579212918303367?idApp=UINPBA00003Z" "url" => "/15792129/0000005400000012/v1_201812020625/S1579212918303367/v1_201812020625/en/main.assets" ] "itemAnterior" => array:18 [ "pii" => "S1579212918303355" "issn" => "15792129" "doi" => "10.1016/j.arbr.2018.10.004" "estado" => "S300" "fechaPublicacion" => "2018-12-01" "aid" => "1929" "documento" => "article" "crossmark" => 1 "subdocumento" => "sco" "cita" => "Arch Bronconeumol. 2018;54:625" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 296 "formatos" => array:3 [ "EPUB" => 58 "HTML" => 152 "PDF" => 86 ] ] "en" => array:11 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Clinical Image</span>" "titulo" => "Radiology, Bronchoscopy and Microbiology in Bronchopulmonary Aspergillosis" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:1 [ "paginaInicial" => "625" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Radiología, broncoscopia y microbiología de la aspergilosis bronco-pulmonar" ] ] "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" => 1293 "Ancho" => 1583 "Tamanyo" => 242150 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(a and b) Coronal and axial computed tomography images, showing high density material in the mediastinum (corresponding to the mucous plug) in almost all of the bronchial tree, with partial occupation starting at the entrance to the main bronchus, complete occupation of the intermediate bronchus, and complete occlusion of the lower and middle lobe bronchi (black arrows), causing partial atelectasis of the parenchyma dependent on these lobar bronchi (white arrow). Bilateral varicose bronchiectasis were also observed in the parenchymal images. (c) Bronchoscopic image of the mucous plug protruding from the entrance to the right bronchus just before aspiration. (d) Characteristic white, cottony <span class="elsevierStyleItalic">Aspergillus terreus</span> colonies isolated in Sabouraud chloramphenicol agar, that develop into cinnamon or coffee-colored powder or granules.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Francisco M. Páez Codeso, María Pilar Bermúdez Ruiz, Antonio Dorado Galindo" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Francisco M." 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(B) Chest CT coronal image of the scan of the (pulmonary parenchymal window) from 2014, showing the appearance of 2 nodules in the right lung: one solid (arrow) and other subsolid with mainly ground glass attenuation and a small, solid core (asterisk). (C) Chest CT axial image (pulmonary parenchymal window) from 2016 showing the appearance of 2 cavitary lung nodules in the right pulmonary apex (arrows). Note that the most lateral nodule has non-centered cavitation with half-moon morphology. (D) Chest CT axial image (mediastinal window) from 2017 in which linear calcification (arrow) has appeared within a solid nodule in the middle lobe.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We report the case of a 30-year-old patient, diagnosed in 2011 with vascular Ehlers-Danlos syndrome (also called Ehlers-Danlos syndrome type IV), who had presented several episodes of bilateral pneumothorax in the previous 2 years. Although the most significant complications presented by this patient were associated with the formation and spontaneous rupture of aneurysms in multiple arteries (carotid, renal, splenic, vertebral, etc.), since 2013 a wide variety of pulmonary nodules had also appeared on incidental radiological images, including solid nodules (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A), subsolid nodules (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B), cavitary nodules (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>C), and nodules containing calcifications (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>D).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Ehlers-Danlos syndrome is a rare autosomal dominant connective tissue disorder, in which a COL3A1 gene mutation causes abnormal type III procollagen synthesis, increasing the likelihood of spontaneous rupture of arteries and vital organs. Very few references are available on the pulmonary radiological manifestations of this rare syndrome, but they seem to be triggered by the spontaneous formation of lung lacerations (caused by systemic tissue fragility), which may progress to bruising, cavitary nodules, fibrous nodules, and finally nodules with metaplastic ossification.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">1,2</span></a> In the absence of clinical evidence of infection (as in our case), it is reasonable to attribute the etiology of the pulmonary nodules to inherent tissue fragility causing the spontaneous formation of parenchymal lacerations, and to progressive secondary changes in the lung tissue, as described by some authors.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">2</span></a></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: Gorospe Sarasúa L, Muñoz-Molina GM, Arrieta P. Las muchas caras radiológicas de los nódulos pulmonares en el síndrome de Ehlers-Danlos tipo <span class="elsevierStyleSmallCaps">iv.</span> Arch Bronconeumol. 2018;54:626.</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" => 426 "Ancho" => 475 "Tamanyo" => 53476 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Chest CT axial image (pulmonary parenchymal window) from 2013, showing a solid nodule in the right lower lobe (arrow). (B) Chest CT coronal image of the scan of the (pulmonary parenchymal window) from 2014, showing the appearance of 2 nodules in the right lung: one solid (arrow) and other subsolid with mainly ground glass attenuation and a small, solid core (asterisk). (C) Chest CT axial image (pulmonary parenchymal window) from 2016 showing the appearance of 2 cavitary lung nodules in the right pulmonary apex (arrows). Note that the most lateral nodule has non-centered cavitation with half-moon morphology. (D) Chest CT axial image (mediastinal window) from 2017 in which linear calcification (arrow) has appeared within a solid nodule in the middle lobe.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:2 [ 0 => array:3 [ "identificador" => "bib0015" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Respiratory complications of Ehlers-Danlos syndrome type IV" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "K. Hatake" 1 => "Y. Morimura" 2 => "R. Kudo" 3 => "W. Kawashima" 4 => "S. Kasuda" 5 => "H. Kuniyasu" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Leg Med (Tokyo)" "fecha" => "2013" "volumen" => "15" "paginaInicial" => "23" "paginaFinal" => "27" ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0020" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pleuropulmonary pathology of vascular Ehlers-Danlos syndrome: spontaneous laceration, haematoma and fibrous nodules" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "Y. Kawabata" 1 => "A. Watanabe" 2 => "S. Yamaguchi" 3 => "M. Aoshima" 4 => "A. Shiraki" 5 => "A. Hatamochi" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Histopathology" "fecha" => "2010" "volumen" => "56" "paginaInicial" => "944" "paginaFinal" => "950" ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/15792129/0000005400000012/v1_201812020625/S1579212918303379/v1_201812020625/en/main.assets" "Apartado" => array:4 [ "identificador" => "21342" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Clinical Image" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15792129/0000005400000012/v1_201812020625/S1579212918303379/v1_201812020625/en/main.pdf?idApp=UINPBA00003Z&text.app=https://archbronconeumol.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1579212918303379?idApp=UINPBA00003Z" ]
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