{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T23:03:13Z","timestamp":1792623793899,"version":"4.5.0"},"reference-count":194,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2024,9,18]],"date-time":"2024-09-18T00:00:00Z","timestamp":1726617600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Cystic Fibrosis Foundation (CFF)","award":["CHATTE22F0"],"award-info":[{"award-number":["CHATTE22F0"]}]},{"name":"Cystic Fibrosis Foundation (CFF)","award":["R01HL157414-01"],"award-info":[{"award-number":["R01HL157414-01"]}]},{"name":"National Institute of Health\/National Heart, Lung, and Blood Institute (NIH\/NHLBI)","award":["CHATTE22F0"],"award-info":[{"award-number":["CHATTE22F0"]}]},{"name":"National Institute of Health\/National Heart, Lung, and Blood Institute (NIH\/NHLBI)","award":["R01HL157414-01"],"award-info":[{"award-number":["R01HL157414-01"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JoF"],"abstract":"<jats:p>Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity disease caused by Aspergillus fumigatus (Af), prevalent in persons with cystic fibrosis (CF) or asthma. In ABPA, Af proteases drive a T-helper cell-2 (Th2)-mediated allergic immune response leading to inflammation that contributes to permanent lung damage. Corticosteroids and antifungals are the mainstays of therapies for ABPA. However, their long-term use has negative sequelae. The treatment of patients with CF (pwCF) has been revolutionized by the efficacy of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy. Pharmacological improvement in CFTR function with highly effective elexacaftor\/tezacaftor\/ivacaftor (ETI) provides unprecedented improvements in lung function and other clinical outcomes of pwCF. The mechanism behind the improvement in patient outcomes is a continued topic of investigation as our understanding of the role of CFTR function evolves. As ETI therapy gains traction in CF management, understanding its potential impact on ABPA, especially on the allergic immune response pathways and Af infection becomes increasingly crucial for optimizing patient outcomes. This literature review aims to examine the extent of these findings and expand our understanding of the already published research focusing on the intersection between ABPA therapeutic approaches in CF and the rapid impact of the evolving CFTR modulator landscape. While our literature search yielded limited reports specifically focusing on the role of CFTR modulator therapy on CF-ABPA, findings from epidemiologic and retrospective studies suggest the potential for CFTR modulator therapies to positively influence pulmonary outcomes by addressing the underlying pathophysiology of CF-ABPA, especially by decreasing inflammatory response and Af colonization. Thus, this review highlights the promising scope of CFTR modulator therapy in decreasing the overall prevalence and incidence of CF-ABPA.<\/jats:p>","DOI":"10.3390\/jof10090656","type":"journal-article","created":{"date-parts":[[2024,9,18]],"date-time":"2024-09-18T09:49:19Z","timestamp":1726652959000},"page":"656","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Allergic Bronchopulmonary Aspergillosis (ABPA) in the Era of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators"],"prefix":"10.3390","volume":"10","author":[{"given":"Paulami","family":"Chatterjee","sequence":"first","affiliation":[{"name":"Division of Pulmonary, Allergy and Critical Care Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6141-1562","authenticated-orcid":false,"given":"Carson Tyler","family":"Moss","sequence":"additional","affiliation":[{"name":"Department of Medicine, Stanford University School of Medicine, Stanford, CA 94304, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-7793-1088","authenticated-orcid":false,"given":"Sarah","family":"Omar","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Allergy and Critical Care Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-9281-4864","authenticated-orcid":false,"given":"Ekroop","family":"Dhillon","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Allergy and Critical Care Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-9625-168X","authenticated-orcid":false,"given":"Carlos Daniel","family":"Hernandez Borges","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Alta Bates Summit Medical Center, Sutter Health, Oakland, CA 94609, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5744-3628","authenticated-orcid":false,"given":"Alan C.","family":"Tang","sequence":"additional","affiliation":[{"name":"Department of Medicine, Keck School of Medicine, Los Angeles, CA 90089, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A.","family":"Stevens","sequence":"additional","affiliation":[{"name":"Division of Infectious Diseases and Geographic Medicine, Stanford University Medical School, Stanford, CA 94305, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joe L.","family":"Hsu","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Allergy and Critical Care Medicine, Stanford University School of Medicine, Stanford, CA 94305, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,9,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1055\/s-0039-1694021","article-title":"Cystic Fibrosis: Pathophysiology of Lung Disease","volume":"40","author":"Bergeron","year":"2019","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1126\/science.1703660","article-title":"Cystic fibrosis transmembrane conductance regulator: Nucleotide binding to a synthetic peptide","volume":"251","author":"Thomas","year":"1991","journal-title":"Science"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"S3","DOI":"10.1152\/physrev.1999.79.1.S3","article-title":"Physiological basis of cystic fibrosis: A historical perspective","volume":"79","author":"Quinton","year":"1999","journal-title":"Physiol. Rev."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1001\/archpedi.1938.01980140114013","article-title":"Cystic fibrosis of the pancreas and its relation to celiac disease: A clinical and pathologic study","volume":"56","author":"Andersen","year":"1938","journal-title":"Am. J. Dis. Child."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1016\/j.jcf.2022.01.009","article-title":"Worldwide rates of diagnosis and effective treatment for cystic fibrosis","volume":"21","author":"Guo","year":"2022","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1891","DOI":"10.1016\/S0140-6736(09)60327-5","article-title":"Cystic fibrosis","volume":"373","author":"Freedman","year":"2009","journal-title":"Lancet"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1016\/j.pcl.2016.04.001","article-title":"Background and Epidemiology","volume":"63","author":"Sanders","year":"2016","journal-title":"Pediatr. Clin. N. Am."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"2330","DOI":"10.1001\/jama.2021.5086","article-title":"Tackling the Misconception That Cystic Fibrosis Is a \u201cWhite People\u2019s Disease\u201d","volume":"325","author":"Rubin","year":"2021","journal-title":"JAMA"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1002\/humu.10041","article-title":"Cystic fibrosis: A worldwide analysis of CFTR mutations--correlation with incidence data and application to screening","volume":"19","author":"Bobadilla","year":"2002","journal-title":"Hum. Mutat."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1038\/clpt.2012.183","article-title":"Longitudinal cystic fibrosis care","volume":"93","author":"Antunovic","year":"2013","journal-title":"Clin. Pharmacol. Ther."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1007\/s11046-021-00541-5","article-title":"CF Fungal Disease in the Age of CFTR Modulators","volume":"186","author":"Bercusson","year":"2021","journal-title":"Mycopathologia"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1091\/mbc.e14-04-0935","article-title":"From CFTR biology toward combinatorial pharmacotherapy: Expanded classification of cystic fibrosis mutations","volume":"27","author":"Veit","year":"2016","journal-title":"Mol. Biol. Cell"},{"key":"ref_13","first-page":"CD010966","article-title":"Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del)","volume":"12","author":"Southern","year":"2020","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1542\/pir.35.5.194","article-title":"Cystic fibrosis","volume":"35","author":"Paranjape","year":"2014","journal-title":"Pediatr. Rev."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"18825","DOI":"10.1073\/pnas.0904709106","article-title":"Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770","volume":"106","author":"Hadida","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"233","DOI":"10.7326\/M13-0636","article-title":"Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: Survival analysis of the Cystic Fibrosis Foundation patient registry","volume":"161","author":"MacKenzie","year":"2014","journal-title":"Ann. Intern. Med."},{"key":"ref_17","unstructured":"FDA (2023). TRIKAFTA\u00ae (Elexacaftor, Tezacaftor, and Ivacaftor), FDA."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"701","DOI":"10.1146\/annurev.biochem.75.103004.142532","article-title":"CFTR function and prospects for therapy","volume":"77","author":"Riordan","year":"2008","journal-title":"Annu. Rev. Biochem."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"850","DOI":"10.1111\/cea.12141","article-title":"Allergic bronchopulmonary aspergillosis: Review of literature and proposal of new diagnostic and classification criteria","volume":"43","author":"Agarwal","year":"2013","journal-title":"Clin. Exp. Allergy"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"P3","DOI":"10.1164\/rccm.1906P3","article-title":"What is allergic bronchopulmonary aspergillosis (ABPA)?","volume":"190","year":"2014","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"S183","DOI":"10.1080\/13693780802189938","article-title":"Immunological mechanisms behind the cystic fibrosis-ABPA link","volume":"47","author":"Hartl","year":"2009","journal-title":"Med. Mycol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"S203","DOI":"10.1080\/13693780500052255","article-title":"Pathophysiology and immunology of allergic bronchopulmonary aspergillosis","volume":"43","author":"Moss","year":"2005","journal-title":"Med. Mycol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1385\/CRIAI:23:1:087","article-title":"Allergic bronchopulmonary aspergillosis","volume":"23","author":"Moss","year":"2002","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"S225","DOI":"10.1086\/376525","article-title":"Allergic bronchopulmonary aspergillosis in cystic fibrosis\u2014State of the art: Cystic Fibrosis Foundation Consensus Conference","volume":"37","author":"Stevens","year":"2003","journal-title":"Clin. Infect. Dis. Off. Publ. Infect. Dis. Soc. Am."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1001","DOI":"10.1067\/mai.2003.1395","article-title":"Association of polymorphisms in the collagen region of SP-A2 with increased levels of total IgE antibodies and eosinophilia in patients with allergic bronchopulmonary aspergillosis","volume":"111","author":"Saxena","year":"2003","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1004","DOI":"10.1111\/j.1398-9995.2005.00887.x","article-title":"Allergic bronchopulmonary aspergillosis","volume":"60","author":"Tonnel","year":"2005","journal-title":"Allergy"},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Tracy, M.C., Okorie, C.U.A., Foley, E.A., and Moss, R.B. (2016). Allergic Bronchopulmonary Aspergillosis. J. Fungi, 2.","DOI":"10.3390\/jof2020017"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1128\/CMR.12.2.310","article-title":"Aspergillus fumigatus and aspergillosis","volume":"12","year":"1999","journal-title":"Clin. Microbiol. Rev."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1378\/chest.08-2586","article-title":"Allergic bronchopulmonary aspergillosis","volume":"135","author":"Agarwal","year":"2009","journal-title":"Chest"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1513\/pats.200908-086AL","article-title":"Allergic bronchopulmonary aspergillosis","volume":"7","author":"Patterson","year":"2010","journal-title":"Proc. Am. Thorac. Soc."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1186\/s13223-023-00822-2","article-title":"The impact of CFTR modulator triple therapy on type 2 inflammatory response in patients with cystic fibrosis","volume":"19","author":"Mehta","year":"2023","journal-title":"Allergy Asthma Clin. Immunol. Off. J. Can. Soc. Allergy Clin. Immunol."},{"key":"ref_32","doi-asserted-by":"crossref","unstructured":"Harvey, C., Weldon, S., Elborn, S., Downey, D.G., and Taggart, C. (2022). The Effect of CFTR Modulators on Airway Infection in Cystic Fibrosis. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23073513"},{"key":"ref_33","first-page":"863","article-title":"Aspergillosis and atopy in cystic fibrosis","volume":"120","author":"Nelson","year":"1979","journal-title":"Am. Rev. Respir. Dis."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"5186","DOI":"10.4049\/jimmunol.177.8.5186","article-title":"Aspergillus fumigatus generates an enhanced Th2-biased immune response in mice with defective cystic fibrosis transmembrane conductance regulator","volume":"177","author":"Allard","year":"2006","journal-title":"J. Immunol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"S32","DOI":"10.1016\/S1569-1993(23)00994-3","article-title":"Evaluating iron-induced Aspergillus proteases: A central effector for Th-2 immune response pathway in allergic bronchopulmonary aspergillosis in people with cystic fibrosis","volume":"22","author":"Chatterjee","year":"2023","journal-title":"J. Cyst. Fibros."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1046\/j.1365-2249.1996.d01-826.x","article-title":"Reduced IL-10 secretion by CD4+ T lymphocytes expressing mutant cystic fibrosis transmembrane conductance regulator (CFTR)","volume":"106","author":"Moss","year":"1996","journal-title":"Clin. Exp. Immunol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1038\/labinvest.3700379","article-title":"Enhanced IgE allergic response to Aspergillus fumigatus in CFTR-\/- mice","volume":"86","author":"Braag","year":"2006","journal-title":"Lab. Investig. A J. Tech. Methods Pathol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1165\/rcmb.2010-0224OC","article-title":"Lack of cystic fibrosis transmembrane conductance regulator in CD3+ lymphocytes leads to aberrant cytokine secretion and hyperinflammatory adaptive immune responses","volume":"44","author":"Mueller","year":"2011","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1165\/rcmb.2011-0399RT","article-title":"Immune responses in cystic fibrosis: Are they intrinsically defective?","volume":"46","author":"Ratner","year":"2012","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1934","DOI":"10.1038\/s41423-020-0499-3","article-title":"CFTR is a negative regulator of \u03b3\u03b4 T cell IFN-\u03b3 production and antitumor immunity","volume":"18","author":"Duan","year":"2021","journal-title":"Cell. Mol. Immunol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"e00696","DOI":"10.1002\/rcr2.696","article-title":"Mepolizumab use in cystic fibrosis-associated allergic bronchopulmonary aspergillosis","volume":"9","author":"Boyle","year":"2021","journal-title":"Respirol. Case Rep."},{"key":"ref_42","first-page":"88","article-title":"Management of patients with cystic fibrosis and allergic bronchopulmonary aspergillosis using anti-immunoglobulin e therapy (omalizumab)","volume":"17","author":"Elmallah","year":"2012","journal-title":"J. Pediatr. Pharmacol. Ther. JPPT Off. J. PPAG"},{"key":"ref_43","doi-asserted-by":"crossref","unstructured":"Shankar, J., Thakur, R., Clemons, K.V., and Stevens, D.A. (2024). Interplay of Cytokines and Chemokines in Aspergillosis. J. Fungi, 10.","DOI":"10.3390\/jof10040251"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"e161593","DOI":"10.1172\/JCI161593","article-title":"Antigen specificity and cross-reactivity drive functionally diverse anti-Aspergillus fumigatus T cell responses in cystic fibrosis","volume":"133","author":"Schwarz","year":"2023","journal-title":"J. Clin. Investig."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"e190","DOI":"10.2741\/990","article-title":"Immunopathogenesis of allergic bronchopulmonary aspergillosis and airway remodeling","volume":"8","author":"Kauffman","year":"2003","journal-title":"Front. Biosci. A J. Virtual Libr."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1183\/09031936.06.00074705","article-title":"The link between fungi and severe asthma: A summary of the evidence","volume":"27","author":"Denning","year":"2006","journal-title":"Eur. Respir. J."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"5904","DOI":"10.4049\/jimmunol.169.10.5904","article-title":"A protease-activated pathway underlying Th cell type 2 activation and allergic lung disease","volume":"169","author":"Kheradmand","year":"2002","journal-title":"J. Immunol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1185","DOI":"10.1067\/mai.2000.106210","article-title":"Protease-dependent activation of epithelial cells by fungal allergens leads to morphologic changes and cytokine production","volume":"105","author":"Kauffman","year":"2000","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/S1569-1993(02)00033-4","article-title":"Immunopathogenesis of allergic bronchopulmonary aspergillosis in cystic fibrosis","volume":"1","author":"Knutsen","year":"2002","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_50","doi-asserted-by":"crossref","unstructured":"Druey, K.M., McCullough, M., and Krishnan, R. (2020). Aspergillus fumigatus Protease Alkaline Protease 1 (Alp1): A New Therapeutic Target for Fungal Asthma. J. Fungi, 6.","DOI":"10.3390\/jof6020088"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"368","DOI":"10.4049\/immunohorizons.1900046","article-title":"Aspergillus fumigatus-Secreted Alkaline Protease 1 Mediates Airways Hyperresponsiveness in Severe Asthma","volume":"3","author":"Redes","year":"2019","journal-title":"ImmunoHorizons"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1111\/cea.12354","article-title":"Pattern recognition pathways leading to a Th2 cytokine bias in allergic bronchopulmonary aspergillosis patients","volume":"45","author":"Becker","year":"2015","journal-title":"Clin. Exp. Allergy J. Br. Soc. Allergy Clin. Immunol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1016\/j.alit.2015.04.004","article-title":"ILC2s and fungal allergy","volume":"64","author":"Kita","year":"2015","journal-title":"Allergol. Int. Off. J. Jpn. Soc. Allergol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1038\/nri2939","article-title":"Immunity to fungal infections","volume":"11","author":"Romani","year":"2011","journal-title":"Nat. Rev. Immunol."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1183\/09031936.00173514","article-title":"CXCR4+ granulocytes reflect fungal cystic fibrosis lung disease","volume":"46","author":"Carevic","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"496","DOI":"10.1136\/thx.49.5.496","article-title":"Eosinophilic activation in cystic fibrosis","volume":"49","author":"Koller","year":"1994","journal-title":"Thorax"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1164\/ajrccm.152.2.7633718","article-title":"Increased degranulation of eosinophil and neutrophil granulocytes in cystic fibrosis","volume":"152","author":"Koller","year":"1995","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/j.mycmed.2018.02.001","article-title":"Evaluation of murine lung epithelial cells (TC-1 JHU-1) line to develop Th2-promoting cytokines IL-25\/IL-33\/TSLP and genes Tlr2\/Tlr4 in response to Aspergillus fumigatus","volume":"28","author":"Khosravi","year":"2018","journal-title":"J. Mycol. Med."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"614","DOI":"10.1016\/j.chom.2020.02.006","article-title":"Club Cell TRPV4 Serves as a Damage Sensor Driving Lung Allergic Inflammation","volume":"27","author":"Wiesner","year":"2020","journal-title":"Cell Host Microbe"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"3669","DOI":"10.2174\/13816128113199990349","article-title":"Immune response, diagnosis and treatment of allergic bronchopulmonary aspergillosis in cystic fibrosis lung disease","volume":"19","author":"Eickmeier","year":"2013","journal-title":"Curr. Pharm. Des."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1487","DOI":"10.1183\/09031936.00139513","article-title":"Treatment options in severe fungal asthma and allergic bronchopulmonary aspergillosis","volume":"43","author":"Moss","year":"2014","journal-title":"Eur. Respir. J."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"529","DOI":"10.4103\/ijmr.IJMR_1187_19","article-title":"Allergic bronchopulmonary aspergillosis","volume":"151","author":"Agarwal","year":"2020","journal-title":"Indian J. Med. Res."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1261","DOI":"10.1016\/j.jaci.2020.08.029","article-title":"New clinical diagnostic criteria for allergic bronchopulmonary aspergillosis\/mycosis and its validation","volume":"147","author":"Asano","year":"2021","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.molimm.2012.09.011","article-title":"Mechanisms of immune dysfunction and bacterial persistence in non-cystic fibrosis bronchiectasis","volume":"55","author":"Chalmers","year":"2013","journal-title":"Mol. Immunol."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1378\/chest.116.3.639","article-title":"Allergic bronchopulmonary aspergillosis in cystic fibrosis: Reported prevalence, regional distribution, and patient characteristics. Scientific Advisory Group, Investigators, and Coordinators of the Epidemiologic Study of Cystic Fibrosis","volume":"116","author":"Geller","year":"1999","journal-title":"Chest"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1067\/mai.2002.130179","article-title":"Allergic bronchopulmonary aspergillosis","volume":"110","author":"Greenberger","year":"2002","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1080\/17476348.2016.1249853","article-title":"Developments in the diagnosis and treatment of allergic bronchopulmonary aspergillosis","volume":"10","author":"Agarwal","year":"2016","journal-title":"Expert Rev. Respir. Med."},{"key":"ref_68","unstructured":"Sisodia, J., and Bajaj, T. (2024). Allergic Bronchopulmonary Aspergillosis. StatPearl, StatPearls Publishing LLC."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"S219","DOI":"10.1093\/mmy\/myy076","article-title":"Pathophysiological aspects of Aspergillus colonization in disease","volume":"57","author":"Gago","year":"2019","journal-title":"Med. Mycol."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"1787","DOI":"10.1002\/ppul.24465","article-title":"An investigation into biomarkers for the diagnosis of ABPA and aspergillus disease in cystic fibrosis","volume":"54","author":"Keown","year":"2019","journal-title":"Pediatr. Pulmonol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"361","DOI":"10.3109\/13693786.2012.738312","article-title":"Global burden of allergic bronchopulmonary aspergillosis with asthma and its complication chronic pulmonary aspergillosis in adults","volume":"51","author":"Denning","year":"2013","journal-title":"Med. Mycol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"1765","DOI":"10.1111\/cea.12595","article-title":"Prevalence of Aspergillus sensitization and allergic bronchopulmonary aspergillosis in cystic fibrosis: Systematic review and meta-analysis","volume":"45","author":"Maturu","year":"2015","journal-title":"Clin. Exp. Allergy J. Br. Soc. Allergy Clin. Immunol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1034\/j.1399-3003.2000.016003464.x","article-title":"Allergic bronchopulmonary aspergillosis in cystic fibrosis. A European epidemiological study. Epidemiologic Registry of Cystic Fibrosis","volume":"16","author":"Mastella","year":"2000","journal-title":"Eur. Respir. J."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"74","DOI":"10.4103\/atm.ATM_231_16","article-title":"Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis","volume":"12","author":"Janahi","year":"2017","journal-title":"Ann. Thorac. Med."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"499","DOI":"10.4103\/lungindia.lungindia_216_18","article-title":"Childhood allergic bronchopulmonary aspergillosis","volume":"35","author":"Jat","year":"2018","journal-title":"Lung India Off. Organ Indian Chest Soc."},{"key":"ref_76","unstructured":"CFF (2014). Patient Registry Annual Data Report, CFF."},{"key":"ref_77","unstructured":"CFF (2022). Patient Registry Annual Data Report, CFF."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1056\/NEJMoa1105185","article-title":"A CFTR potentiator in patients with cystic fibrosis and the G551D mutation","volume":"365","author":"Ramsey","year":"2011","journal-title":"N. Engl. J. Med."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1186\/s12955-015-0293-6","article-title":"Effect of ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation: Patient-reported outcomes in the STRIVE randomized, controlled trial","volume":"13","author":"Quittner","year":"2015","journal-title":"Health Qual. Life Outcomes"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1186\/s12955-016-0465-z","article-title":"Impact of pulmonary exacerbations and lung function on generic health-related quality of life in patients with cystic fibrosis","volume":"14","author":"Solem","year":"2016","journal-title":"Health Qual. Life Outcomes"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/j.jcf.2017.06.002","article-title":"Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor","volume":"17","author":"Flume","year":"2018","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/S2213-2600(15)00545-7","article-title":"Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): An open-label, single-arm study","volume":"4","author":"Davies","year":"2016","journal-title":"Lancet. Respir. Med."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1002\/ppul.23659","article-title":"In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies","volume":"52","author":"McGarry","year":"2017","journal-title":"Pediatr. Pulmonol."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"2037","DOI":"10.1042\/CS20170995","article-title":"Improvement in exercise duration, lung function and well-being in G551D-cystic fibrosis patients: A double-blind, placebo-controlled, randomized, cross-over study with ivacaftor treatment","volume":"131","author":"Edgeworth","year":"2017","journal-title":"Clin. Sci."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1016\/S2213-2600(18)30202-9","article-title":"Ivacaftor treatment of cystic fibrosis in children aged 12 to <24 months and with a CFTR gating mutation (ARRIVAL): A phase 3 single-arm study","volume":"6","author":"Rosenfeld","year":"2018","journal-title":"Lancet Respir. Med."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1016\/j.jcf.2019.03.009","article-title":"An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2\u20135 years (KLIMB)","volume":"18","author":"Rosenfeld","year":"2019","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/j.jpeds.2018.05.018","article-title":"Energy Balance and Mechanisms of Weight Gain with Ivacaftor Treatment of Cystic Fibrosis Gating Mutations","volume":"201","author":"Stallings","year":"2018","journal-title":"J. Pediatr."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1016\/S2213-2600(14)70132-8","article-title":"A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: A phase 2 randomised controlled trial","volume":"2","author":"Boyle","year":"2014","journal-title":"Lancet Respir. Med."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"2024","DOI":"10.1056\/NEJMoa1709847","article-title":"Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis","volume":"377","author":"Rowe","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1056\/NEJMoa1409547","article-title":"Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR","volume":"373","author":"Wainwright","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1016\/S2213-2600(16)30121-7","article-title":"for the VX-809 TRAFFIC and TRANSPORT study groups. Efficacy and safety of lumacaftor\/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: A pooled analysis","volume":"4","author":"Elborn","year":"2016","journal-title":"Lancet. Respir. Med."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1016\/j.jcf.2018.12.004","article-title":"Measuring recovery in health-related quality of life during and after pulmonary exacerbations in patients with cystic fibrosis","volume":"18","author":"Flume","year":"2019","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1016\/j.jcf.2018.07.011","article-title":"Lumacaftor\/Ivacaftor reduces pulmonary exacerbations in patients irrespective of initial changes in FEV(1)","volume":"18","author":"McColley","year":"2019","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"912","DOI":"10.1164\/rccm.201608-1754OC","article-title":"Lumacaftor\/Ivacaftor in Patients Aged 6-11 Years with Cystic Fibrosis and Homozygous for F508del-CFTR","volume":"195","author":"Milla","year":"2017","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/S2213-2600(16)30427-1","article-title":"Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): A phase 3, extension study","volume":"5","author":"Konstan","year":"2017","journal-title":"Lancet. Respir. Med."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1016\/j.jcf.2017.09.012","article-title":"Lumacaftor\/ivacaftor in patients with cystic fibrosis and advanced lung disease homozygous for F508del-CFTR","volume":"17","author":"Jain","year":"2018","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1016\/S2213-2600(17)30215-1","article-title":"Efficacy and safety of lumacaftor and ivacaftor in patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR: A randomised, placebo-controlled phase 3 trial","volume":"5","author":"Ratjen","year":"2017","journal-title":"Lancet. Respir. Med."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1016\/S2213-2600(18)30460-0","article-title":"Safety, pharmacokinetics, and pharmacodynamics of lumacaftor and ivacaftor combination therapy in children aged 2\u20135 years with cystic fibrosis homozygous for F508del-CFTR: An open-label phase 3 study","volume":"7","author":"McNamara","year":"2019","journal-title":"Lancet. Respir. Med."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"1433","DOI":"10.1164\/rccm.201710-1983OC","article-title":"Effects of Lumacaftor-Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis","volume":"197","author":"Graeber","year":"2018","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1164\/rccm.201808-1436LE","article-title":"Might Brushed Nasal Cells Be a Surrogate for CFTR Modulator Clinical Response?","volume":"199","author":"Pranke","year":"2019","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"2013","DOI":"10.1056\/NEJMoa1709846","article-title":"Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del","volume":"377","author":"Munck","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1164\/rccm.201704-0717OC","article-title":"on behalf of the VX11-661-101 Study Group. Tezacaftor\/Ivacaftor in Subjects with Cystic Fibrosis and F508del\/F508del-CFTR or F508del\/G551D-CFTR","volume":"197","author":"Donaldson","year":"2018","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1016\/j.jcf.2019.06.009","article-title":"A phase 3 study of tezacaftor in combination with ivacaftor in children aged 6 through 11 years with cystic fibrosis","volume":"18","author":"Walker","year":"2019","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"1612","DOI":"10.1056\/NEJMoa1807120","article-title":"VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles","volume":"379","author":"Keating","year":"2018","journal-title":"N. Engl. J. Med."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele","volume":"381","author":"Middleton","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"1940","DOI":"10.1016\/S0140-6736(19)32597-8","article-title":"Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: A double-blind, randomised, phase 3 trial","volume":"394","author":"Heijerman","year":"2019","journal-title":"Lancet"},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"00758-2020","DOI":"10.1183\/23120541.00758-2020","article-title":"Would it have happened without Christmas? Thoracic emphysema and allergic bronchopulmonary aspergillosis in a juvenile cystic fibrosis patient with the G551D mutation receiving ivacaftor","volume":"7","author":"Mainz","year":"2021","journal-title":"ERJ Open Res."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1136\/thx.7.4.317","article-title":"Broncho-pulmonary aspergillosis; a review and a report of eight new cases","volume":"7","author":"Hinson","year":"1952","journal-title":"Thorax"},{"key":"ref_109","first-page":"e4550","article-title":"Diagnosing Allergic Bronchopulmonary Aspergillosis: A Review","volume":"11","author":"Patel","year":"2019","journal-title":"Cureus"},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.ccm.2021.12.002","article-title":"Allergic Bronchopulmonary Aspergillosis","volume":"43","author":"Agarwal","year":"2022","journal-title":"Clin. Chest Med."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"20406223211047003","DOI":"10.1177\/20406223211047003","article-title":"Review of current and future therapeutics in ABPA","volume":"12","author":"Chan","year":"2021","journal-title":"Ther. Adv. Chronic Dis."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"405","DOI":"10.7326\/0003-4819-86-4-405","article-title":"Clinical and immunologic criteria for the diagnosis of allergic bronchopulmonary aspergillosis","volume":"86","author":"Rosenberg","year":"1977","journal-title":"Ann. Intern. Med."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"286","DOI":"10.7326\/0003-4819-96-3-286","article-title":"Allergic bronchopulmonary aspergillosis: Staging as an aid to management","volume":"96","author":"Patterson","year":"1982","journal-title":"Ann. Intern. Med."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.anai.2013.04.014","article-title":"When to suspect and work up allergic bronchopulmonary aspergillosis","volume":"111","author":"Greenberger","year":"2013","journal-title":"Ann. Allergy Asthma Immunol."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1016\/j.jaci.2011.12.970","article-title":"Fungi and allergic lower respiratory tract diseases","volume":"129","author":"Knutsen","year":"2012","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1016\/j.jaip.2020.08.043","article-title":"Which Are the Optimal Criteria for the Diagnosis of Allergic Bronchopulmonary Aspergillosis? A Latent Class Analysis","volume":"9","author":"Saxena","year":"2021","journal-title":"J. Allergy Clin. Immunol. Pr."},{"key":"ref_117","doi-asserted-by":"crossref","unstructured":"Agarwal, R., Saxena, P., Muthu, V., Sehgal, I.S., Dhooria, S., Prasad, K.T., Aggarwal, A.N., and Chakrabarti, A. (2022). Evaluation of Simpler Criteria for Diagnosing Allergic Bronchopulmonary Aspergillosis Complicating Asthma. Front. Cell. Infect. Microbiol., 12.","DOI":"10.3389\/fcimb.2022.861866"},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"930","DOI":"10.1016\/S0025-6196(11)62114-9","article-title":"Diagnosis and treatment of allergic bronchopulmonary aspergillosis","volume":"76","author":"Vlahakis","year":"2001","journal-title":"Mayo Clin. Proc."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"286","DOI":"10.4103\/0970-2113.99122","article-title":"Allergic bronchopulmonary aspergillosis without asthma complicating pulmonary tuberculosis","volume":"29","author":"Gupta","year":"2012","journal-title":"Lung India Off. Organ Indian Chest Soc."},{"key":"ref_120","first-page":"e15498","article-title":"Allergic Bronchopulmonary Aspergillosis with or without Asthmatic Symptoms?","volume":"13","year":"2021","journal-title":"Cureus"},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"101392","DOI":"10.1016\/j.mycmed.2023.101392","article-title":"Allergic bronchopulmonary aspergillosis: A multidisciplinary review","volume":"33","author":"Roboubi","year":"2023","journal-title":"J. Mycol. Med."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"896","DOI":"10.1016\/j.jaip.2022.12.012","article-title":"Omalizumab in Allergic Bronchopulmonary Aspergillosis: A Systematic Review and Meta-Analysis","volume":"11","author":"Jin","year":"2023","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"420","DOI":"10.1002\/art.21984","article-title":"Population-based assessment of adverse events associated with long-term glucocorticoid use","volume":"55","author":"Curtis","year":"2006","journal-title":"Arthritis Rheum"},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"1828","DOI":"10.1016\/S0140-6736(03)14904-5","article-title":"Glucocorticoids and invasive fungal infections","volume":"362","author":"Lionakis","year":"2003","journal-title":"Lancet"},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1093\/cid\/ciw326","article-title":"Practice Guidelines for the Diagnosis and Management of Aspergillosis: 2016 Update by the Infectious Diseases Society of America","volume":"63","author":"Patterson","year":"2016","journal-title":"Clin. Infect. Dis. Off. Publ. Infect. Dis. Soc. Am."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1056\/NEJM200003163421102","article-title":"A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis","volume":"342","author":"Stevens","year":"2000","journal-title":"N. Engl. J. Med."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"682","DOI":"10.1055\/s-0031-1295716","article-title":"Allergic bronchopulmonary aspergillosis and related allergic syndromes","volume":"32","author":"Hogan","year":"2011","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_128","first-page":"423","article-title":"Voriconazole and posaconazole improve asthma severity in allergic bronchopulmonary aspergillosis and severe asthma with fungal sensitization","volume":"49","author":"Chishimba","year":"2012","journal-title":"J. Asthma Off. J. Assoc. Care Asthma"},{"key":"ref_129","first-page":"Cd002204","article-title":"Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis","volume":"9","author":"Francis","year":"2022","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"833","DOI":"10.1007\/s40265-019-01127-8","article-title":"Adverse Effects Associated with Long-Term Administration of Azole Antifungal Agents","volume":"79","author":"Benitez","year":"2019","journal-title":"Drugs"},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1007\/s12281-014-0183-0","article-title":"Cardiotoxicity Induced by Antifungal Drugs","volume":"8","author":"Nix","year":"2014","journal-title":"Curr. Fungal Infect. Rep."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1016\/j.rmed.2017.11.007","article-title":"Omalizumab treatment for allergic bronchopulmonary aspergillosis in young patients with cystic fibrosis","volume":"133","author":"Perisson","year":"2017","journal-title":"Respir. Med."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1016\/j.jcf.2019.07.011","article-title":"Omalizumab for asthma and allergic bronchopulmonary aspergillosis in adults with cystic fibrosis","volume":"19","author":"Koutsokera","year":"2020","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1097\/MCP.0000000000000951","article-title":"Eosinophilic respiratory disorders and the impact of biologics","volume":"29","author":"Bernstein","year":"2023","journal-title":"Curr. Opin. Pulm. Med."},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"319","DOI":"10.4168\/aair.2016.8.4.319","article-title":"Omalizumab Improves Quality of Life and Asthma Control in Chinese Patients with Moderate to Severe Asthma: A Randomized Phase III Study","volume":"8","author":"Li","year":"2016","journal-title":"Allergy Asthma Immunol. Res."},{"key":"ref_136","first-page":"Cd010288","article-title":"Anti-IgE therapy for allergic bronchopulmonary aspergillosis in people with cystic fibrosis","volume":"9","author":"Jat","year":"2021","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"100676","DOI":"10.1016\/j.waojou.2022.100676","article-title":"Current and emerging biologic therapies targeting eosinophilic disorders","volume":"15","author":"Pitlick","year":"2022","journal-title":"World Allergy Organ. J."},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1016\/j.jaip.2014.12.008","article-title":"Clinical efficacy and immunologic effects of omalizumab in allergic bronchopulmonary aspergillosis","volume":"3","author":"Voskamp","year":"2015","journal-title":"J Allergy Clin Immunol Pr."},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1186\/s13223-019-0397-3","article-title":"Use of mepolizumab in adult patients with cystic fibrosis and an eosinophilic phenotype: Case series","volume":"16","author":"Zhang","year":"2020","journal-title":"Allergy Asthma Clin. Immunol. Off. J. Can. Soc. Allergy Clin. Immunol."},{"key":"ref_140","first-page":"101964","article-title":"Successful treatment with dupilumab in mepolizumab-resistant allergic bronchopulmonary aspergillosis","volume":"47","author":"Kawasaki","year":"2024","journal-title":"Respir. Med. Case Rep."},{"key":"ref_141","doi-asserted-by":"crossref","unstructured":"Tomomatsu, K., Yasuba, H., Ishiguro, T., Imokawa, S., Hara, J., Soeda, S., Harada, N., Tsurikisawa, N., Oda, N., and Katoh, S. (2023). Real-world efficacy of anti-IL-5 treatment in patients with allergic bronchopulmonary aspergillosis. Sci. Rep., 13.","DOI":"10.1038\/s41598-023-32246-8"},{"key":"ref_142","doi-asserted-by":"crossref","unstructured":"Baldo, B.A. (2022). Immune- and Non-Immune-Mediated Adverse Effects of Monoclonal Antibody Therapy: A Survey of 110 Approved Antibodies. Antibodies, 11.","DOI":"10.3390\/antib11010017"},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"190068","DOI":"10.1183\/16000617.0068-2019","article-title":"CFTR targeted therapies: Recent advances in cystic fibrosis and possibilities in other diseases of the airways","volume":"29","author":"Patel","year":"2020","journal-title":"Eur. Respir. Rev. Off. J. Eur. Respir. Soc."},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1055\/s-0029-1238919","article-title":"A pipeline of therapies for cystic fibrosis","volume":"30","author":"Ashlock","year":"2009","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_145","doi-asserted-by":"crossref","unstructured":"Bengtson, C., Silswal, N., Baumlin, N., Yoshida, M., Dennis, J., Yerrathota, S., Kim, M., and Salathe, M. (2022). The CFTR Amplifier Nesolicaftor Rescues TGF-\u03b21 Inhibition of Modulator-Corrected F508del CFTR Function. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms231810956"},{"key":"ref_146","first-page":"Cd012040","article-title":"Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis","volume":"3","author":"Aslam","year":"2023","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"1193","DOI":"10.1164\/rccm.201910-1943SO","article-title":"Cystic Fibrosis: Emergence of Highly Effective Targeted Therapeutics and Potential Clinical Implications","volume":"201","author":"Mall","year":"2020","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/j.jcf.2022.05.007","article-title":"VOCAL: An observational study of ivacaftor for people with cystic fibrosis and selected non-G551D-CFTR gating mutations","volume":"22","author":"Simmonds","year":"2023","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1016\/j.jcf.2014.09.005","article-title":"Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation","volume":"13","author":"Munck","year":"2014","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"18843","DOI":"10.1073\/pnas.1105787108","article-title":"Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809","volume":"108","author":"Hadida","year":"2011","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"718","DOI":"10.1378\/chest.11-2672","article-title":"Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation","volume":"142","author":"Flume","year":"2012","journal-title":"Chest"},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1136\/thoraxjnl-2011-200393","article-title":"Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation","volume":"67","author":"Clancy","year":"2012","journal-title":"Thorax"},{"key":"ref_153","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1097\/MCP.0b013e32833f1d00","article-title":"Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis","volume":"16","author":"Sloane","year":"2010","journal-title":"Curr. Opin. Pulm. Med."},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"895","DOI":"10.1016\/j.jcf.2021.03.011","article-title":"Elexacaftor co-potentiates the activity of F508del and gating mutants of CFTR","volume":"20","author":"Veit","year":"2021","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_155","doi-asserted-by":"crossref","unstructured":"Bacalhau, M., Camargo, M., Magalh\u00e3es-Ghiotto, G.A.V., Drumond, S., Castelletti, C.H.M., and Lopes-Pacheco, M. (2023). Elexacaftor-Tezacaftor-Ivacaftor: A Life-Changing Triple Combination of CFTR Modulator Drugs for Cystic Fibrosis. Pharmaceuticals, 16.","DOI":"10.3390\/ph16030410"},{"key":"ref_156","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1055\/s-0042-1758851","article-title":"CFTR Modulators: Current Status and Evolving Knowledge","volume":"44","author":"Regard","year":"2023","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"2202029","DOI":"10.1183\/13993003.02029-2022","article-title":"Long-term safety and efficacy of elexacaftor\/tezacaftor\/ivacaftor in people with cystic fibrosis and at least one F508del allele: 144-week interim results from a 192-week open-label extension study","volume":"62","author":"Daines","year":"2023","journal-title":"Eur. Respir. J."},{"key":"ref_158","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1016\/j.jcf.2023.02.004","article-title":"Elexacaftor\/tezacaftor\/ivacaftor projected survival and long-term health outcomes in people with cystic fibrosis homozygous for F508del","volume":"22","author":"Lopez","year":"2023","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_159","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1164\/rccm.202108-1986OC","article-title":"Clinical Effectiveness of Elexacaftor\/Tezacaftor\/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial","volume":"205","author":"Nichols","year":"2022","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_160","doi-asserted-by":"crossref","first-page":"540","DOI":"10.1164\/rccm.202110-2249OC","article-title":"Effects of Elexacaftor\/Tezacaftor\/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles","volume":"205","author":"Graeber","year":"2022","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1186\/s12967-017-1193-9","article-title":"Cystic fibrosis: Current therapeutic targets and future approaches","volume":"15","author":"Rafeeq","year":"2017","journal-title":"J. Transl. Med."},{"key":"ref_162","doi-asserted-by":"crossref","unstructured":"Ambrogi, M., and Vezina, C.M. (2024). Roles of airway and intestinal epithelia in responding to pathogens and maintaining tissue homeostasis. Front. Cell. Infect. Microbiol., 14.","DOI":"10.3389\/fcimb.2024.1346087"},{"key":"ref_163","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1038\/s41591-021-01332-7","article-title":"Transcriptional analysis of cystic fibrosis airways at single-cell resolution reveals altered epithelial cell states and composition","volume":"27","author":"Carraro","year":"2021","journal-title":"Nat. Med."},{"key":"ref_164","doi-asserted-by":"crossref","first-page":"1419","DOI":"10.1164\/rccm.202004-0991OC","article-title":"Single-Cell Transcriptional Archetypes of Airway Inflammation in Cystic Fibrosis","volume":"202","author":"Schupp","year":"2020","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_165","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1016\/j.jcf.2018.03.010","article-title":"CFTR rescue with VX-809 and VX-770 favors the repair of primary airway epithelial cell cultures from patients with class II mutations in the presence of Pseudomonas aeruginosa exoproducts","volume":"17","author":"Adam","year":"2018","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_166","doi-asserted-by":"crossref","first-page":"1338","DOI":"10.1164\/rccm.202310-1836OC","article-title":"Pharmacological Improvement of CFTR Function Rescues Airway Epithelial Homeostasis and Host Defense in Children with Cystic Fibrosis","volume":"209","author":"Loske","year":"2024","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_167","doi-asserted-by":"crossref","first-page":"1275","DOI":"10.1164\/rccm.202008-3198OC","article-title":"Secretory Cells Dominate Airway CFTR Expression and Function in Human Airway Superficial Epithelia","volume":"203","author":"Okuda","year":"2021","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_168","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1038\/s41586-018-0394-6","article-title":"A single-cell atlas of the airway epithelium reveals the CFTR-rich pulmonary ionocyte","volume":"560","author":"Plasschaert","year":"2018","journal-title":"Nature"},{"key":"ref_169","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1007\/s00018-016-2391-y","article-title":"Role of CFTR in epithelial physiology","volume":"74","author":"Gray","year":"2017","journal-title":"Cell. Mol. Life Sci."},{"key":"ref_170","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1016\/j.ccm.2022.06.007","article-title":"The Impact of Highly Effective Modulator Therapy on Cystic Fibrosis Microbiology and Inflammation","volume":"43","author":"Caverly","year":"2022","journal-title":"Clin. Chest Med."},{"key":"ref_171","doi-asserted-by":"crossref","first-page":"L635","DOI":"10.1152\/ajplung.00198.2017","article-title":"Vx-809\/Vx-770 treatment reduces inflammatory response to Pseudomonas aeruginosa in primary differentiated cystic fibrosis bronchial epithelial cells","volume":"314","author":"Ruffin","year":"2018","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"ref_172","doi-asserted-by":"crossref","first-page":"1271","DOI":"10.1164\/rccm.202002-0369OC","article-title":"Ivacaftor Reverses Airway Mucus Abnormalities in a Rat Model Harboring a Humanized G551D-CFTR","volume":"202","author":"Birket","year":"2020","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_173","doi-asserted-by":"crossref","first-page":"eaau7531","DOI":"10.1126\/scitranslmed.aau7531","article-title":"In utero and postnatal VX-770 administration rescues multiorgan disease in a ferret model of cystic fibrosis","volume":"11","author":"Sun","year":"2019","journal-title":"Sci. Transl. Med."},{"key":"ref_174","doi-asserted-by":"crossref","first-page":"L1093","DOI":"10.1152\/ajplung.00082.2021","article-title":"Ivacaftor partially corrects airway inflammation in a humanized G551D rat","volume":"320","author":"Green","year":"2021","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"ref_175","doi-asserted-by":"crossref","unstructured":"Mainz, J.G., Arnold, C., Wittstock, K., Hipler, U.C., Lehmann, T., Zagoya, C., Duckstein, F., Ellemunter, H., and Hentschel, J. (2021). Ivacaftor Reduces Inflammatory Mediators in Upper Airway Lining Fluid from Cystic Fibrosis Patients with a G551D Mutation: Serial Non-Invasive Home-Based Collection of Upper Airway Lining Fluid. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.642180"},{"key":"ref_176","doi-asserted-by":"crossref","first-page":"e150398","DOI":"10.1172\/JCI150398","article-title":"Inflammatory cytokines TNF-alpha and IL-17 enhance the efficacy of cystic fibrosis transmembrane conductance regulator modulators","volume":"131","author":"Rehman","year":"2021","journal-title":"J. Clin. Investig."},{"key":"ref_177","doi-asserted-by":"crossref","first-page":"1123","DOI":"10.1164\/rccm.201510-1922OC","article-title":"Mutations of Cystic Fibrosis Transmembrane Conductance Regulator Gene Cause a Monocyte-Selective Adhesion Deficiency","volume":"193","author":"Sorio","year":"2016","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_178","doi-asserted-by":"crossref","first-page":"L432","DOI":"10.1152\/ajplung.00461.2017","article-title":"Lumacaftor (VX-809) restores the ability of CF macrophages to phagocytose and kill Pseudomonas aeruginosa","volume":"314","author":"Barnaby","year":"2018","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"ref_179","doi-asserted-by":"crossref","unstructured":"Zhang, S., Shrestha, C.L., and Kopp, B.T. (2018). Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function. Sci. Rep., 8.","DOI":"10.1038\/s41598-018-35151-7"},{"key":"ref_180","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1136\/thoraxjnl-2017-210134","article-title":"Delayed neutrophil apoptosis enhances NET formation in cystic fibrosis","volume":"73","author":"Gray","year":"2018","journal-title":"Thorax"},{"key":"ref_181","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1164\/rccm.202106-1426OC","article-title":"Trikafta Rescues CFTR and Lowers Monocyte P2X7R-induced Inflammasome Activation in Cystic Fibrosis","volume":"205","author":"Casey","year":"2022","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_182","doi-asserted-by":"crossref","first-page":"e54556","DOI":"10.7554\/eLife.54556","article-title":"Different CFTR modulator combinations downregulate inflammation differently in cystic fibrosis","volume":"9","author":"Scambler","year":"2020","journal-title":"eLife"},{"key":"ref_183","doi-asserted-by":"crossref","first-page":"1169","DOI":"10.1016\/j.immuni.2017.11.010","article-title":"Cystic Fibrosis Transmembrane Conductance Regulator Attaches Tumor Suppressor PTEN to the Membrane and Promotes Anti Pseudomonas aeruginosa Immunity","volume":"47","author":"Riquelme","year":"2017","journal-title":"Immunity"},{"key":"ref_184","doi-asserted-by":"crossref","unstructured":"Eschenhagen, P.N., Bacher, P., Grehn, C., Mainz, J.G., Scheffold, A., and Schwarz, C. (2023). Proliferative activity of antigen-specific CD154+ T cells against bacterial and fungal respiratory pathogens in cystic fibrosis decreases after initiation of highly effective CFTR modulator therapy. Front. Pharmacol., 14.","DOI":"10.3389\/fphar.2023.1180826"},{"key":"ref_185","doi-asserted-by":"crossref","first-page":"S113","DOI":"10.1016\/S1569-1993(23)00531-3","article-title":"P156 Treatment with the CFTR modulator [HB1] elexacafor\/tezacaftor\/ivacaftor reduces immunological response to airway Aspergillus in people with cystic fibrosis","volume":"22","author":"Francis","year":"2023","journal-title":"J. Cyst. Fibros."},{"key":"ref_186","doi-asserted-by":"crossref","first-page":"S39","DOI":"10.1016\/S1569-1993(23)00298-9","article-title":"EPS1. 07 Impact of elexacaftor\/tezacaftor\/ivacaftor (ETI) therapy on Aspergillus fumigatus serology in adults with cystic fibrosis","volume":"22","author":"Curran","year":"2023","journal-title":"J. Cyst. Fibros."},{"key":"ref_187","doi-asserted-by":"crossref","unstructured":"Jones, J.T., Morelli, K.A., Vesely, E.M., Puerner, C.T.S., Pavuluri, C.K., Ross, B.S., van Rhijn, N., Bromley, M.J., and Cramer, R.A. (2023). The cystic fibrosis treatment Trikafta affects the growth, viability, and cell wall of Aspergillus fumigatus biofilms. mBio, 14.","DOI":"10.1128\/mbio.01516-23"},{"key":"ref_188","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1136\/thoraxjnl-2017-210394","article-title":"Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor","volume":"73","author":"Bessonova","year":"2018","journal-title":"Thorax"},{"key":"ref_189","doi-asserted-by":"crossref","first-page":"1084","DOI":"10.1016\/j.jcf.2022.09.007","article-title":"Advent of elexacaftor\/tezacaftor\/ivacaftor for cystic fibrosis treatment: What consequences on Aspergillus-related diseases? Preliminary insights","volume":"21","author":"Chesnay","year":"2022","journal-title":"J. Cyst. Fibros. Off. J. Eur. Cyst. Fibros. Soc."},{"key":"ref_190","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.1513\/AnnalsATS.201902-122OC","article-title":"Ivacaftor Is Associated with Reduced Lung Infection by Key Cystic Fibrosis Pathogens. A Cohort Study Using National Registry Data","volume":"16","author":"Frost","year":"2019","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_191","doi-asserted-by":"crossref","first-page":"S27","DOI":"10.1016\/S1569-1993(23)00986-4","article-title":"51 Home-collected sputum samples for Aspergillus fumigatus detection in adults with cystic fibrosis","volume":"22","author":"Hong","year":"2023","journal-title":"J. Cyst. Fibros."},{"key":"ref_192","doi-asserted-by":"crossref","first-page":"S9","DOI":"10.1016\/S1569-1993(23)00949-9","article-title":"14 Spontaneous changes to airway microbiology after initiation of elexacaftor-tezacaftor-ivacaftor in people with advanced cystic fibrosis lung disease","volume":"22","author":"Mullins","year":"2023","journal-title":"J. Cyst. Fibros."},{"key":"ref_193","doi-asserted-by":"crossref","first-page":"S34","DOI":"10.1016\/S1569-1993(23)00999-2","article-title":"64 Sputum density of Aspergillus fumigatus markedly declines after treatment with elexacaftor-tezacaftor-ivacaftor","volume":"22","author":"Morgan","year":"2023","journal-title":"J. Cyst. Fibros."},{"key":"ref_194","doi-asserted-by":"crossref","unstructured":"Currie, A.J., Main, E.T., Wilson, H.M., Armstrong-James, D., and Warris, A. (2020). CFTR Modulators Dampen Aspergillus-Induced Reactive Oxygen Species Production by Cystic Fibrosis Phagocytes. Front. Cell. Infect. 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