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diagnosis of bronchial asthma was made&#46; High doses of inhaled corticosteroids and long-acting bronchodilators &#40;beta 2 agonist and anticholinergic&#41; were initiated&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">In 2017 she started ivacaftor and lung function improved&#46; However&#44; three years later &#40;2020&#41; she had a primoinfection by <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> treated with inhaled colistimethate sodium&#46; Although eradication was achieved&#44; after one year under treatment with good adherence to antibiotic and bronchodilators&#44; &#40;verified by electronic prescription and inhalers&#8217; adhesion test &#40;TAI&#41; score 50 of 50 points&#41;&#44; she remained with moderate pulmonary exacerbations&#44; lung function decrease and uncontrolled asthma &#40;asthma control test score 16 of 25 points&#41; requiring oral antibiotics and acute oral corticosteroids&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">When ETI was approved in our country &#40;2022&#41;&#44; we switched CTFR therapy to ETI&#46; Mepolizumab &#40;anti-IL5 Mab&#41; was also started because of uncontrolled T2 &#40;eosinophilic and allergic&#41; severe asthma&#44; but had to be stopped because of late bronchospasm&#46; Later&#44; benralizumab &#40;anti-IL5 receptor Mab&#41; was initiated with adequate tolerance&#46; After one year under treatment with ETI and benralizumab&#44; the patient had a clinical &#40;asymptomatic&#44; better exercise tolerance and less exacerbations&#41;&#44; functional &#40;resolution of airflow obstruction&#41;&#44; radiological &#40;bronchiectasis improvement&#41; and microbiological &#40;clearance of <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> and no other isolations&#41; improvement&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">There is no gold standard for diagnosis of CFAOS&#44; being helpful a suggestive clinical history&#44; presence of atopic disease&#44; airflow reversibility with high FE<span class="elsevierStyleInf">NO</span> and symptoms improvement with appropriate treatment of suspected asthma&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a> Treatment should be individualised&#46; To our knowledge&#44; this is the first case combining ETI and biological asthma treatment for CFAOS&#46; CFTR modulators&#44; taking into account their role on airway remodelling&#44; may play a key role on patients with CFAOS and asthma-overlapping features of CF&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">3</span></a></p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Authors&#8217; Contributions</span><p id="par0030" class="elsevierStylePara elsevierViewall">All authors contributed equally to this manuscript&#46;</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 con&#64258;ict of interests&#46;</p></span></span>"
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Case Report
Cystic Fibrosis–Asthma Overlap Syndrome. Combination of Cystic Fibrosis Transmembrane Conductance Regulator Modulators and Type 2 Targeted Biologic Treatment for Asthma
Miguel Jiménez-Gómeza,
Corresponding author
migueljimenezgomez@gmail.com

Corresponding author.
, Rocío Magdalena Díaz Camposb, Layla Diab Cáceresc
a Pulmonology Department, 12th of October University Hospital, Madrid, Spain
b Pulmonology Department, Asthma Unit, 12th of October University Hospital, Madrid, Spain
c Pulmonology Department, Cystic Fibrosis Unit, 12th of October University Hospital, Madrid, Spain
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E monoclonal antibodies &#91;Mab&#93;&#41; 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> &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a non-smoking woman diagnosed with CF &#40;F508del&#47;R117H&#41; at the age of 21 &#40;2009&#41;&#44; studied for repeated respiratory infections&#44; airflow obstruction and central bronchiectasis&#46; 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>&#46; Bronchodilators and airway clearance techniques were started&#46; While bronchorrhea improved&#44; she referred persistent wheezing and dyspnoea related to temperature changes&#44; nocturnal cough and seasonal rhino-conjunctivitis&#46; The skin prick test was positive to olive tree&#44; <span class="elsevierStyleItalic">Alternaria tenuis</span> and cat epithelium&#46; Lung function showed a moderate airflow obstruction with high variability and a high exhaled fraction of nitric oxide &#40;FE<span class="elsevierStyleInf">NO</span>&#41; of 55&#8211;103<span class="elsevierStyleHsp" style=""></span>ppb&#46; A persistent peripheral eosinophilia of 800&#8211;2300<span class="elsevierStyleHsp" style=""></span>cells&#47;&#956;L &#40;normal 0&#8211;500<span class="elsevierStyleHsp" style=""></span>cells&#47;&#956;L&#41; was observed&#44; with persistent total IgE of 197&#8211;287<span class="elsevierStyleHsp" style=""></span>KU&#47;L &#40;normal<span class="elsevierStyleHsp" style=""></span>&#60;<span class="elsevierStyleHsp" style=""></span>120<span class="elsevierStyleHsp" style=""></span>KU&#47;L&#41;&#46; After ruling out allergic bronchopulmonary aspergillosis&#44; diagnosis of bronchial asthma was made&#46; High doses of inhaled corticosteroids and long-acting bronchodilators &#40;beta 2 agonist and anticholinergic&#41; were initiated&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">In 2017 she started ivacaftor and lung function improved&#46; However&#44; three years later &#40;2020&#41; she had a primoinfection by <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> treated with inhaled colistimethate sodium&#46; Although eradication was achieved&#44; after one year under treatment with good adherence to antibiotic and bronchodilators&#44; &#40;verified by electronic prescription and inhalers&#8217; adhesion test &#40;TAI&#41; score 50 of 50 points&#41;&#44; she remained with moderate pulmonary exacerbations&#44; lung function decrease and uncontrolled asthma &#40;asthma control test score 16 of 25 points&#41; requiring oral antibiotics and acute oral corticosteroids&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">When ETI was approved in our country &#40;2022&#41;&#44; we switched CTFR therapy to ETI&#46; Mepolizumab &#40;anti-IL5 Mab&#41; was also started because of uncontrolled T2 &#40;eosinophilic and allergic&#41; severe asthma&#44; but had to be stopped because of late bronchospasm&#46; Later&#44; benralizumab &#40;anti-IL5 receptor Mab&#41; was initiated with adequate tolerance&#46; After one year under treatment with ETI and benralizumab&#44; the patient had a clinical &#40;asymptomatic&#44; better exercise tolerance and less exacerbations&#41;&#44; functional &#40;resolution of airflow obstruction&#41;&#44; radiological &#40;bronchiectasis improvement&#41; and microbiological &#40;clearance of <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> and no other isolations&#41; improvement&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">There is no gold standard for diagnosis of CFAOS&#44; being helpful a suggestive clinical history&#44; presence of atopic disease&#44; airflow reversibility with high FE<span class="elsevierStyleInf">NO</span> and symptoms improvement with appropriate treatment of suspected asthma&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a> Treatment should be individualised&#46; To our knowledge&#44; this is the first case combining ETI and biological asthma treatment for CFAOS&#46; CFTR modulators&#44; taking into account their role on airway remodelling&#44; may play a key role on patients with CFAOS and asthma-overlapping features of CF&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">3</span></a></p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Authors&#8217; Contributions</span><p id="par0030" class="elsevierStylePara elsevierViewall">All authors contributed equally to this manuscript&#46;</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 con&#64258;ict of interests&#46;</p></span></span>"
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ISSN: 03002896
Original language: English
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