Case Report
Cystic Fibrosis–Asthma Overlap Syndrome. Combination of Cystic Fibrosis Transmembrane Conductance Regulator Modulators and Type 2 Targeted Biologic Treatment for Asthma
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ECG on the right performed after 2 months shows normalized leads. (b) Coronary angiogram on the left reveals the arteries without coronary disease. Left: right coronary artery. Right: left coronary artery. (c) Left ventriculography in systolic frame shows apical segment akinesis with a mild depressed LVEF (46%). (d) Echocardiography performed 2 months later shows a preserved contractibility with normalization of the LVEF (60%).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Albert Rafecas-Codern, Paula Pujal-Montaña, Virginia Pajares" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Albert" "apellidos" => "Rafecas-Codern" ] 1 => array:2 [ "nombre" => "Paula" "apellidos" => "Pujal-Montaña" ] 2 => array:2 [ "nombre" => "Virginia" "apellidos" => "Pajares" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289623001527?idApp=UINPBA00003Z" "url" => "/03002896/0000005900000008/v3_202311210631/S0300289623001527/v3_202311210631/en/main.assets" ] "itemAnterior" => array:18 [ "pii" => "S0300289623001242" "issn" => "03002896" "doi" => "10.1016/j.arbres.2023.04.001" "estado" => "S300" "fechaPublicacion" => "2023-08-01" "aid" => "3304" "copyright" => "SEPAR" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Arch Bronconeumol. 2023;59:510-1" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Case Report</span>" "titulo" => "Aortobronchopulmonary Fistula—An Unusual Cause of Hemoptysis" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "510" "paginaFinal" => "511" ] ] "contieneResumen" => array:1 [ "en" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 1 "multimedia" => array:5 [ "identificador" => "fig0010" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => false "mostrarDisplay" => true "figura" => array:1 [ 0 => array:4 [ "imagen" => "fx1.jpeg" "Alto" => 767 "Ancho" => 1333 "Tamanyo" => 114722 ] ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Laura Castellanos López, Elisa Martínez Besteiro, Adrián Martínez Vergara" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Laura" "apellidos" => "Castellanos López" ] 1 => array:2 [ "nombre" => "Elisa" "apellidos" => "Martínez Besteiro" ] 2 => array:2 [ "nombre" => "Adrián" "apellidos" => "Martínez Vergara" ] ] ] ] "resumen" => array:1 [ 0 => array:3 [ "titulo" => "Graphical abstract" "clase" => "graphical" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall"><elsevierMultimedia ident="fig0010"></elsevierMultimedia></p></span>" ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289623001242?idApp=UINPBA00003Z" "url" => "/03002896/0000005900000008/v3_202311210631/S0300289623001242/v3_202311210631/en/main.assets" ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Case Report</span>" "titulo" => "Cystic Fibrosis–Asthma Overlap Syndrome. Combination of Cystic Fibrosis Transmembrane Conductance Regulator Modulators and Type 2 Targeted Biologic Treatment for Asthma" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "512" "paginaFinal" => "513" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Miguel Jiménez-Gómez, Rocío Magdalena Díaz Campos, Layla Diab Cáceres" "autores" => array:3 [ 0 => array:4 [ "nombre" => "Miguel" "apellidos" => "Jiménez-Gómez" "email" => array:1 [ 0 => "migueljimenezgomez@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" => "Rocío Magdalena" "apellidos" => "Díaz Campos" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "Layla" "apellidos" => "Diab Cáceres" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Pulmonology Department, 12th of October University Hospital, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Pulmonology Department, Asthma Unit, 12th of October University Hospital, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Pulmonology Department, Cystic Fibrosis Unit, 12th of October University Hospital, Madrid, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "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" => 1426 "Ancho" => 2925 "Tamanyo" => 321960 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Treatment, lung function and respiratory exacerbations over time. Lung function is represented as predicted volume exhaled during the first second (FEV1 percentage). Exacerbations which required antibiotics are represented as blue bars, and those requiring corticosteroids are represented as dashed green bars. ETI: elexacaftor/tezacaftor/ivacaftor.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Cystic fibrosis (CF) and asthma share similar symptoms and lung function (such as airflow obstruction and hyperresponsiveness). There is debate on how to best define and distinguish asthma-like features and CF-asthma overlap syndrome (CFAOS).<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">2–4</span></a> Management of both has dramatically changed over the past years.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a> Highly effective CFTR modulators, especially elexacaftor/tezacaftor/ivacaftor (ETI), are transforming CF by targeting the root cause itself.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">1</span></a> Furthermore, asthma biologic therapies (such as anti-interleukin [IL]-5 and anti-immunoglobulin [Ig] E monoclonal antibodies [Mab]) decrease exacerbations and improve lung function in severe asthma patients not associated with CF<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a> (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a non-smoking woman diagnosed with CF (F508del/R117H) at the age of 21 (2009), studied for repeated respiratory infections, airflow obstruction and central bronchiectasis. She developed a severe respiratory involvement with bilateral cystic bronchiectasis predominantly in upper lobes with chronic bronchial infection by <span class="elsevierStyleItalic">Staphylococcus aureus</span>. Bronchodilators and airway clearance techniques were started. While bronchorrhea improved, she referred persistent wheezing and dyspnoea related to temperature changes, nocturnal cough and seasonal rhino-conjunctivitis. The skin prick test was positive to olive tree, <span class="elsevierStyleItalic">Alternaria tenuis</span> and cat epithelium. Lung function showed a moderate airflow obstruction with high variability and a high exhaled fraction of nitric oxide (FE<span class="elsevierStyleInf">NO</span>) of 55–103<span class="elsevierStyleHsp" style=""></span>ppb. A persistent peripheral eosinophilia of 800–2300<span class="elsevierStyleHsp" style=""></span>cells/μL (normal 0–500<span class="elsevierStyleHsp" style=""></span>cells/μL) was observed, with persistent total IgE of 197–287<span class="elsevierStyleHsp" style=""></span>KU/L (normal<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>120<span class="elsevierStyleHsp" style=""></span>KU/L). After ruling out allergic bronchopulmonary aspergillosis, diagnosis of bronchial asthma was made. High doses of inhaled corticosteroids and long-acting bronchodilators (beta 2 agonist and anticholinergic) were initiated.</p><p id="par0015" class="elsevierStylePara elsevierViewall">In 2017 she started ivacaftor and lung function improved. However, three years later (2020) she had a primoinfection by <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> treated with inhaled colistimethate sodium. Although eradication was achieved, after one year under treatment with good adherence to antibiotic and bronchodilators, (verified by electronic prescription and inhalers’ adhesion test (TAI) score 50 of 50 points), she remained with moderate pulmonary exacerbations, lung function decrease and uncontrolled asthma (asthma control test score 16 of 25 points) requiring oral antibiotics and acute oral corticosteroids.</p><p id="par0020" class="elsevierStylePara elsevierViewall">When ETI was approved in our country (2022), we switched CTFR therapy to ETI. Mepolizumab (anti-IL5 Mab) was also started because of uncontrolled T2 (eosinophilic and allergic) severe asthma, but had to be stopped because of late bronchospasm. Later, benralizumab (anti-IL5 receptor Mab) was initiated with adequate tolerance. After one year under treatment with ETI and benralizumab, the patient had a clinical (asymptomatic, better exercise tolerance and less exacerbations), functional (resolution of airflow obstruction), radiological (bronchiectasis improvement) and microbiological (clearance of <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> and no other isolations) improvement.</p><p id="par0025" class="elsevierStylePara elsevierViewall">There is no gold standard for diagnosis of CFAOS, being helpful a suggestive clinical history, presence of atopic disease, airflow reversibility with high FE<span class="elsevierStyleInf">NO</span> and symptoms improvement with appropriate treatment of suspected asthma.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a> Treatment should be individualised. To our knowledge, this is the first case combining ETI and biological asthma treatment for CFAOS. CFTR modulators, taking into account their role on airway remodelling, may play a key role on patients with CFAOS and asthma-overlapping features of CF.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">3</span></a></p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Authors’ Contributions</span><p id="par0030" class="elsevierStylePara elsevierViewall">All authors contributed equally to this manuscript.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflict of Interests</span><p id="par0035" class="elsevierStylePara elsevierViewall">The authors state that they have no conflict of interests.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Authors’ Contributions" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Conflict of Interests" ] 2 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "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" => 1426 "Ancho" => 2925 "Tamanyo" => 321960 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Treatment, lung function and respiratory exacerbations over time. Lung function is represented as predicted volume exhaled during the first second (FEV1 percentage). Exacerbations which required antibiotics are represented as blue bars, and those requiring corticosteroids are represented as dashed green bars. ETI: elexacaftor/tezacaftor/ivacaftor.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0025" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Cystic fibrosis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "M. Shteinberg" 1 => "I.J. Haq" 2 => "D. Polineni" 3 => "J.C. Davies" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/S0140-6736(20)32542-3" "Revista" => array:6 [ "tituloSerie" => "Lancet" "fecha" => "2021" "volumen" => "397" "numero" => "10290" "paginaInicial" => "2195" "paginaFinal" => "2211" ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0030" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Asthma in cystic fibrosis: definitions and implications of this overlap syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "C.R. Marion" 1 => "M. Izquierdo" 2 => "H.C. Hanes" 3 => "C. Barrios" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s11882-020-00985-7" "Revista" => array:3 [ "tituloSerie" => "Curr Allergy Asthma Rep" "fecha" => "2021" "volumen" => "21" ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0035" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "How the airway smooth muscle in cystic fibrosis reacts in proinflammatory conditions: implications for airway hyper-responsiveness and asthma in cystic fibrosis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "S. McCuaig" 1 => "J.G. Martin" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/S2213-2600(12)70058-9" "Revista" => array:5 [ "tituloSerie" => "Lancet Respir Med" "fecha" => "2013" "volumen" => "1" "paginaInicial" => "137" "paginaFinal" => "147" ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0040" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Reversible airway obstruction in cystic fibrosis: common, but not associated with characteristics of asthma" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "H. Levine" 1 => "M. Cohen-Cymberknoh" 2 => "N. Klein" 3 => "M. Hoshen" 4 => "H. Mussaffi" 5 => "P. Stafler" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jcf.2016.01.003" "Revista" => array:5 [ "tituloSerie" => "J Cyst Fibros" "fecha" => "2016" "volumen" => "15" "paginaInicial" => "652" "paginaFinal" => "659" ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/03002896/0000005900000008/v3_202311210631/S0300289623001266/v3_202311210631/en/main.assets" "Apartado" => array:4 [ "identificador" => "93562" "tipo" => "SECCION" "es" => array:2 [ "titulo" => "Case Reports" "idiomaDefecto" => true ] "idiomaDefecto" => "es" ] "PDF" => "https://static.elsevier.es/multimedia/03002896/0000005900000008/v3_202311210631/S0300289623001266/v3_202311210631/en/main.pdf?idApp=UINPBA00003Z&text.app=https://archbronconeumol.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289623001266?idApp=UINPBA00003Z" ]
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