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Current Allergy and Asthma Reports

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01-02-2021 | Cystic Fibrosis | Asthma (V Ortega, Section Editor)

Asthma in Cystic Fibrosis: Definitions and Implications of This Overlap Syndrome

Authors: Chad R. Marion, Manuel Izquierdo, Holly C. Hanes, Christopher Barrios

Published in: Current Allergy and Asthma Reports | Issue 2/2021

Abstract

Purpose of Review

Cystic fibrosis (CF) is a multisystem, autosomal recessive disease that leads to progressive loss of lung function. Respiratory symptoms for both CF and asthma include cough, wheezing, and dyspnea. There is debate within the CF community on how to best define and distinguish CF-asthma overlap syndrome (CFAOS) from asthma-like features, though CFAOS is well-recognized. We aim to review the epidemiology, diagnosis, and treatment of asthma in CF and explore areas where further research is needed.

Recent Findings

There has been considerable improvement in the understanding and treatment of asthma over the past two decades leading to novel therapies such as biologic agents that target the airway inflammation in asthmatics based on their asthma phenotype. These therapies are being studied in CFAOS and are promising treatments.

Summary

This review provides a comprehensive overview of the definition, epidemiology, diagnosis, and current treatment of CFAOS.

Farrell PM, White TB, Ren CL, Hempstead SE, Accurso F, Derichs N, et al. Diagnosis of cystic fibrosis: consensus guidelines from the Cystic Fibrosis Foundation. J Pediatr. 2017;181S:S4–S15.e1. https://doi.org/10.1016/j.jpeds.2016.09.064.CrossRefPubMed

Willis-Owen SAG, Cookson WOC, Moffatt MF. The genetics and genomics of asthma. Annu Rev Genomics Hum Genet. 2018;19:223–46. https://doi.org/10.1146/annurev-genom-083117-021651.CrossRefPubMed

Shrine N, Portelli MA, John C, Soler Artigas M, Bennett N, Hall R, et al. Moderate-to-severe asthma in individuals of European ancestry: a genome-wide association study. Lancet Respir Med. 2019;7(1):20–34. https://doi.org/10.1016/S2213-2600(18)30389-8.CrossRefPubMedPubMedCentral

Global Initiative for Asthma. Global Strategy for Asthma Management and Prevention, 2020. Available from: www.ginasthma.org. (2020). Accessed.

Ren CL, Konstan MW, Rosenfeld M, Pasta DJ, Millar SJ, Morgan WJ, et al. Early childhood wheezing is associated with lower lung function in cystic fibrosis. Pediatr Pulmonol. 2014;49(8):745–50. https://doi.org/10.1002/ppul.22894.CrossRefPubMed

Morgan WJ, Butler SM, Johnson CA, Colin AA, FitzSimmons SC, Geller DE, et al. Epidemiologic study of cystic fibrosis: design and implementation of a prospective, multicenter, observational study of patients with cystic fibrosis in the U.S. and Canada. Pediatr Pulmonol. 1999;28(4):231–41. https://doi.org/10.1002/(sici)1099-0496(199910)28:4<231::aid-ppul1>3.0.co;2-2.CrossRefPubMed

Mogayzel PJ, Naureckas ET, Robinson KA, Mueller G, Hadjiliadis D, Hoag JB, et al. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013;187(7):680–9. https://doi.org/10.1164/rccm.201207-1160oe.CrossRefPubMed

Kent BD, Lane SJ, van Beek EJ, Dodd JD, Costello RW, Tiddens HA. Asthma and cystic fibrosis: a tangled web. Pediatr Pulmonol. 2014;49(3):205–13. https://doi.org/10.1002/ppul.22934.CrossRefPubMed

Balfour-Lynn IM, Elborn JS. “CF asthma”: what is it and what do we do about it? Thorax. 2002;57(8):742–8. https://doi.org/10.1136/thorax.57.8.742.CrossRefPubMedPubMedCentral

10.

Brand PL. Bronchodilators in cystic fibrosis. J R Soc Med. 2000;93(Suppl 38):37–9.PubMedPubMedCentral

11.

• Adam RJ, Hisert KB, Dodd JD, Grogan B, Launspach JL, Barnes JK, et al. Acute administration of ivacaftor to people with cystic fibrosis and a G551D-CFTR mutation reveals smooth muscle abnormalities. JCI Insight. 2016;1(4):e86183. https://doi.org/10.1172/jci.insight.86183Comment: Study showing potentiation of CFTR improved airway distensibility and vascular tone.CrossRefPubMedPubMedCentral

12.

Stanbrook MB, Corey M, Tullis DE. The repeatability of forced expiratory volume measurements in adults with cystic fibrosis. Chest. 2004;125(1):150–5. https://doi.org/10.1378/chest.125.1.150.CrossRefPubMed

13.

Legge JS. Peak-expiratory-flow meters and asthma self-management. Lancet. 1996;347(9017):1709–10. https://doi.org/10.1016/s0140-6736(96)90802-8.CrossRefPubMed

14.

Tiddens HA, Donaldson SH, Rosenfeld M, Pare PD. Cystic fibrosis lung disease starts in the small airways: can we treat it more effectively? Pediatr Pulmonol. 2010;45(2):107–17. https://doi.org/10.1002/ppul.21154.CrossRefPubMed

15.

Eggleston PA, Rosenstein BJ, Stackhouse CM, Mellits ED, Baumgardner RA. A controlled trial of long-term bronchodilator therapy in cystic fibrosis. Chest. 1991;99(5):1088–92. https://doi.org/10.1378/chest.99.5.1088.CrossRefPubMed

16.

Conway SP, Watson A. Nebulised bronchodilators, corticosteroids, and rhDNase in adult patients with cystic fibrosis. Thorax. 1997;52(Suppl 2):S64–8. https://doi.org/10.1136/thx.52.2008.s64.CrossRefPubMedPubMedCentral

17.

Levine H, Cohen-Cymberknoh M, Klein N, Hoshen M, Mussaffi H, Stafler P, et al. Reversible airway obstruction in cystic fibrosis: common, but not associated with characteristics of asthma. J Cyst Fibros. 2016;15(5):652–9. https://doi.org/10.1016/j.jcf.2016.01.003.CrossRefPubMed

18.

• Cook DP, Rector MV, Bouzek DC, Michalski AS, Gansemer ND, Reznikov LR, et al. Cystic Fibrosis transmembrane conductance regulator in sarcoplasmic reticulum of airway smooth muscle. Implications for airway contractility. Am J Respir Crit Care Med. 2016;193(4):417–26. https://doi.org/10.1164/rccm.201508-1562OCComment: Study using newborn pigs (wild type and CF) to evaluate the role of CFTR expressed in airway smooth muscle on airway dysfunction.CrossRefPubMedPubMedCentral

19.

Furlan LL, Ribeiro JD, Bertuzzo CS, Salomao Junior JB, Souza DRS, Marson FAL. Variants in the interleukin 8 gene and the response to inhaled bronchodilators in cystic fibrosis. J Pediatr. 2017;93(6):639–48. https://doi.org/10.1016/j.jped.2017.03.005.CrossRef

20.

van Haren EH, Lammers JW, Festen J, van Herwaarden CL. Bronchial vagal tone and responsiveness to histamine, exercise and bronchodilators in adult patients with cystic fibrosis. Eur Respir J. 1992;5(9):1083–8.PubMed

21.

Sanchez I, Powell RE, Pasterkamp H. Wheezing and airflow obstruction during methacholine challenge in children with cystic fibrosis and in normal children. Am Rev Respir Dis. 1993;147(3):705–9. https://doi.org/10.1164/ajrccm/147.3.705.CrossRefPubMed

22.

McCuaig S, Martin JG. How the airway smooth muscle in cystic fibrosis reacts in proinflammatory conditions: implications for airway hyper-responsiveness and asthma in cystic fibrosis. Lancet Respir Med. 2013;1(2):137–47. https://doi.org/10.1016/s2213-2600(12)70058-9.CrossRefPubMed

23.

• Matusovsky OS, Kachmar L, Ijpma G, Panariti A, Benedetti A, Martin JG, et al. Contractile properties of intrapulmonary airway smooth muscle in cystic fibrosis. Am J Respir Cell Mol Biol. 2019;60(4):434–44. https://doi.org/10.1165/rcmb.2018-0005OCComment: Study exploring the role of airway smooth muscle in airway dysfunction in CF.CrossRefPubMed

24.

Eggleston PA, Rosenstein BJ, Stackhouse CM, Alexander MF. Airway hyperreactivity in cystic fibrosis. Clinical correlates and possible effects on the course of the disease. Chest. 1988;94(2):360–5. https://doi.org/10.1378/chest.94.2.360.CrossRefPubMed

25.

Alothman GA, Ho B, Alsaadi MM, Ho SL, O’Drowsky L, Louca E, et al. Bronchial constriction and inhaled colistin in cystic fibrosis. Chest. 2005;127(2):522–9. https://doi.org/10.1378/chest.127.2.522.CrossRefPubMed

26.

Dwyer TJ, Alison JA, McKeough ZJ, Daviskas E, Bye PTP. Effects of exercise on respiratory flow and sputum properties in patients with cystic fibrosis. Chest. 2011;139(4):870–7. https://doi.org/10.1378/chest.10-1158.CrossRefPubMed

27.

Dwyer TJ, Zainuldin R, Daviskas E, Bye PT, Alison JA. Effects of treadmill exercise versus Flutter(R) on respiratory flow and sputum properties in adults with cystic fibrosis: a randomised, controlled, cross-over trial. BMC Pulmo Med. 2017;17(1):14. https://doi.org/10.1186/s12890-016-0360-8.CrossRef

28.

Kirkby SE, Hayes D Jr, Parsons JP, Wisely CE, Kopp B, McCoy KS, et al. Eucapnic voluntary hyperventilation to detect exercise-induced bronchoconstriction in cystic fibrosis. Lung. 2015;193(5):733–8. https://doi.org/10.1007/s00408-015-9745-3.CrossRefPubMed

29.

Tobin MJ, Maguire O, Reen D, Tempany E, Fitzgerald MX. Atopy and bronchial reactivity in older patients with cystic fibrosis. Thorax. 1980;35(11):807–13. https://doi.org/10.1136/thx.35.11.807.CrossRefPubMedPubMedCentral

30.

Becker JW, Burke W, McDonald G, Greenberger PA, Henderson WR, Aitken ML. Prevalence of allergic bronchopulmonary aspergillosis and atopy in adult patients with cystic fibrosis. Chest. 1996;109(6):1536–40. https://doi.org/10.1378/chest.109.6.1536.CrossRefPubMed

31.

Wilmott RW. The relationship between atopy and cystic fibrosis. Clin Rev Allergy. 1991;9(1–2):29–46. https://doi.org/10.1007/978-1-4612-0475-6_3.CrossRefPubMed

32.

Robroeks CM, Rosias PP, van Vliet D, Jobsis Q, Yntema JB, Brackel HJ, et al. Biomarkers in exhaled breath condensate indicate presence and severity of cystic fibrosis in children. Pediatr Allergy Immunol. 2008;19(7):652–9. https://doi.org/10.1111/j.1399-3038.2007.00693.x.CrossRefPubMed

33.

Korten I, Liechti M, Singer F, Hafen G, Rochat I, Anagnostopoulou P, et al. Lower exhaled nitric oxide in infants with cystic fibrosis compared to healthy controls. J Cyst Fibros. 2018;17(1):105–8. https://doi.org/10.1016/j.jcf.2017.05.005.CrossRefPubMed

34.

National Asthma Education and Prevention Program. Expert Panel Report 3 (EPR-3): guidelines for the diagnosis and management of asthma-summary report 2007. J Allergy Clin Immunol. 2007;120(5 Suppl):S94–138. https://doi.org/10.1016/j.jaci.2007.09.043.CrossRef

35.

Konstan MW, Butler SM, Schidlow DV, Morgan WJ, Julius JR, Johnson CA. Patterns of medical practice in cystic fibrosis: part II. Use of therapies. Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis. Pediatr Pulmonol. 1999;28(4):248–54. https://doi.org/10.1002/(sici)1099-0496(199910)28:4<248::aid-ppul3>3.0.co;2-n.CrossRefPubMed

36.

König P, Poehler J, Barbero GJ. A placebo-controlled, double-blind trial of the long-term effects of albuterol administration in patients with cystic fibrosis. Pediatr Pulmonol. 1998;25(1):32–6. https://doi.org/10.1002/(sici)1099-0496(199801)25:1<32::aid-ppul3>3.0.co;2-q.CrossRefPubMed

37.

Halfhide C, Evans HJ, Couriel J. Inhaled bronchodilators for cystic fibrosis. Cochrane Database Syst Rev. 2005;(4):Cd003428. https://doi.org/10.1002/14651858.CD003428.pub2.

38.

Durzo A. The GOAL study. Canadian family physician Medecin de famille canadien. 2006;52(2):187–9.

39.

Hordvik NL, Sammut PH, Judy CG, Colombo JL. Effects of standard and high doses of salmeterol on lung function of hospitalized patients with cystic fibrosis. Pediatr Pulmonol. 1999;27(1):43–53. https://doi.org/10.1002/(sici)1099-0496(199901)27:1<43::aid-ppul9>3.0.co;2-e.CrossRefPubMed

40.

Bargon J, Viel K, Dauletbaev N, Wiewrodt R, Buhl R. Short-term effects of regular salmeterol treatment on adult cystic fibrosis patients. Eur Respir J. 1997;10(10):2307–11. https://doi.org/10.1183/09031936.97.10102307.CrossRefPubMed

41.

Smith S, Edwards CT. Long-acting inhaled bronchodilators for cystic fibrosis. Cochrane Database Syst Rev. 2017;12:CD012102. https://doi.org/10.1002/14651858.CD012102.pub2.CrossRefPubMed

42.

• Kuruvilla ME, Lee FE-H, Lee GB. Understanding asthma phenotypes, endotypes, and mechanisms of disease. Clin Rev Allergy Immunol. 2019;56(2):219–33. https://doi.org/10.1007/S12016-018-8712-1Comment: Comprehensive review of the complex biologic processes in asthma.CrossRefPubMedPubMedCentral

43.

Erdem E, Ersu R. Inhaled corticosteroids in treatment of cystic fibrosis. Antiinflamm Antiallergy Agents Med Chem. 2012;11(3):206–9. https://doi.org/10.2174/1871523011202030206.CrossRefPubMed

44.

van Haren EH, Lammers JW, Festen J, Heijerman HG, Groot CA, van Herwaarden CL. The effects of the inhaled corticosteroid budesonide on lung function and bronchial hyperresponsiveness in adult patients with cystic fibrosis. Respir Med. 1995;89(3):209–14. https://doi.org/10.1016/0954-6111(95)90249-x.CrossRefPubMed

45.

De Boeck K, Vermeulen F, Wanyama S, Thomas M. Inhaled corticosteroids and lower lung function decline in young children with cystic fibrosis. Eur Respir J. 2011;37(5):1091–5. https://doi.org/10.1183/09031936.00077210.CrossRefPubMed

46.

Balfour-Lynn IM, Lees B, Hall P, Phillips G, Khan M, Flather M, et al. Multicenter randomized controlled trial of withdrawal of inhaled corticosteroids in cystic fibrosis. Am J Respir Crit Care Med. 2006;173(12):1356–62. https://doi.org/10.1164/rccm.200511-1808OC.CrossRefPubMed

47.

Balfour-Lynn IM, Welch K, Smith S. Inhaled corticosteroids for cystic fibrosis. Cochrane Database Syst Rev. 2019;7(7):Cd001915. https://doi.org/10.1002/14651858.CD001915.pub6.CrossRefPubMed

48.

Ng D, Salvio F, Hicks G. Anti-leukotriene agents compared to inhaled corticosteroids in the management of recurrent and/or chronic asthma in adults and children. Cochrane Database Syst Rev. 2004;(2):Cd002314. https://doi.org/10.1002/14651858.CD002314.pub2.

49.

Schmitt-Grohé S, Eickmeier O, Schubert R, Bez C, Zielen S. Anti-inflammatory effects of montelukast in mild cystic fibrosis. Ann Allergy Asthma Immunol. 2002;89(6):599–605. https://doi.org/10.1016/s1081-1206(10)62108-4.CrossRefPubMed

50.

Schumock GT, Lee TA, Joo MJ, Valuck RJ, Stayner LT, Gibbons RD. Association between leukotriene-modifying agents and suicide: what is the evidence? Drug Saf. 2011;34(7):533–44. https://doi.org/10.2165/11587260-000000000-00000.CrossRefPubMed

51.

Konstan MW, Sharma A, Moroni-Zentgraf P, Wang F, Koker P. Safety, tolerability, and plasma exposure of tiotropium Respimat® in children and adults with cystic fibrosis. J Aerosol Med Pulm Drug Deliv. 2015;28(2):137–44. https://doi.org/10.1089/jamp.2013.1115.CrossRefPubMed

52.

Bradley JM, Koker P, Deng Q, Moroni-Zentgraf P, Ratjen F, Geller DE, et al. Testing two different doses of tiotropium Respimat® in cystic fibrosis: phase 2 randomized trial results. PLoS One. 2014;9(9):e106195. https://doi.org/10.1371/journal.pone.0106195.CrossRefPubMedPubMedCentral

53.

Ratjen F, Koker P, Geller DE, Langellier-Cocteaux B, Le Maulf F, Kattenbeck S, et al. Tiotropium Respimat in cystic fibrosis: phase 3 and pooled phase 2/3 randomized trials. J Cyst Fibros. 2015;14(5):608–14. https://doi.org/10.1016/j.jcf.2015.03.004.CrossRefPubMed

54.

Brandt C, Thronicke A, Roehmel JF, Krannich A, Staab D, Schwarz C. Impact of long-term tiotropium bromide therapy on annual lung function decline in adult patients with cystic fibrosis. PLoS One. 2016;11(6):e0158193. https://doi.org/10.1371/journal.pone.0158193.CrossRefPubMedPubMedCentral

55.

Kew KM, Dahri K. Long-acting muscarinic antagonists (LAMA) added to combination long-acting beta2-agonists and inhaled corticosteroids (LABA/ICS) versus LABA/ICS for adults with asthma. Cochrane Database Syst Rev. 2016;(1):Cd011721. https://doi.org/10.1002/14651858.CD011721.pub2.

56.

Ramamoorthy S, Cidlowski JA. Corticosteroids: mechanisms of action in health and disease. Rheum Dis Clin N Am. 2016;42(1):15–31, vii. https://doi.org/10.1016/j.rdc.2015.08.002.CrossRef

57.

Eigen H, Rosenstein BJ, FitzSimmons S, Schidlow DV. A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group. J Pediatr. 1995;126(4):515–23. https://doi.org/10.1016/s0022-3476(95)70343-8.CrossRefPubMed

58.

Dovey M, Aitken ML, Emerson J, McNamara S, Waltz DA, Gibson RL. Oral corticosteroid therapy in cystic fibrosis patients hospitalized for pulmonary exacerbation: a pilot study. Chest. 2007;132(4):1212–8. https://doi.org/10.1378/chest.07-0843.CrossRefPubMed

59.

Ghdifan S, Couderc L, Michelet I, Leguillon C, Masseline B, Marguet C. Bolus methylprednisolone efficacy for uncontrolled exacerbation of cystic fibrosis in children. Pediatrics. 2010;125(5):e1259–64. https://doi.org/10.1542/peds.2009-2042.CrossRefPubMed

60.

Hester KL, Powell T, Downey DG, Elborn JS, Jarad NA. Glucocorticoids as an adjuvant treatment to intravenous antibiotics for cystic fibrosis pulmonary exacerbations: a UK survey. J Cyst Fibros. 2007;6(4):311–3. https://doi.org/10.1016/j.jcf.2006.12.009.CrossRefPubMed

61.

Kapnadak SG, Dimango E, Hadjiliadis D, Hempstead SE, Tallarico E, Pilewski JM, et al. Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease. J Cyst Fibros. 2020;19(3):344–54. https://doi.org/10.1016/j.jcf.2020.02.015.CrossRefPubMed

62.

Johnson N, Varughese B, De La Torre MA, Surani SR, Udeani G. A review of respiratory biologic agents in severe asthma. Cureus. 2019;11(9):e5690. https://doi.org/10.7759/cureus.5690.CrossRefPubMedPubMedCentral

63.

Agache I, Rocha C, Beltran J, Song Y, Posso M, Solà I, et al. Efficacy and safety of treatment with biologicals (benralizumab, dupilumab and omalizumab) for severe allergic asthma: a systematic review for the EAACI guidelines-recommendations on the use of biologicals in severe asthma. Allergy. 2020;75(5):1043–57. https://doi.org/10.1111/all.14235.CrossRefPubMed

64.

Thomson NC, Chaudhuri R. Omalizumab: clinical use for the management of asthma. Clin Med Insights. 2012;6:CCRPM.S7793. https://doi.org/10.4137/ccrpm.S7793.CrossRef

65.

Humbert M, Beasley R, Ayres J, Slavin R, Hébert J, Bousquet J, et al. Benefits of omalizumab as add-on therapy in patients with severe persistent asthma who are inadequately controlled despite best available therapy (GINA 2002 step 4 treatment): INNOVATE. Allergy. 2005;60(3):309–16. https://doi.org/10.1111/j.1398-9995.2004.00772.x.CrossRefPubMed

66.

Braunstahl G-J, Leo J, Chen C-W, Maykut R, Georgiou P, Peachey G. The eXpeRience registry: monitoring the “real-world” effectiveness of omalizumab in allergic asthma. Eur Respir J. 2011;38(Suppl 55):3953.

67.

Ching-Hsiung L, Shih-Lung C. A review of omalizumab for the management of severe asthma. Drug Design Dev Therapy. 2016;10:2369–78. https://doi.org/10.2147/DDDT.S112208.CrossRef

68.

Ashkenazi M, Sity S, Sarouk I, Bat El Bar A, Dagan A, Bezalel Y, et al. Omalizumab in allergic bronchopulmonary aspergillosis in patients with cystic fibrosis. J Asthma Allergy. 2018;11:101–7. https://doi.org/10.2147/JAA.S156049.CrossRefPubMedPubMedCentral

69.

Perisson C, Destruys L, Grenet D, Bassinet L, Derelle J, Sermet-Gaudelus I, et al. Omalizumab treatment for allergic bronchopulmonary aspergillosis in young patients with cystic fibrosis. Respir Med. 2017;133:12–5. https://doi.org/10.1016/j.rmed.2017.11.007.CrossRefPubMed

70.

Mazza JA, Lewis J, Gooyers T. A case report of ABPA, cystic fibrosis and asthma treated with omalizumab. World Allergy Organizat J. 2015;8(Suppl 1):A86-A. https://doi.org/10.1186/1939-4551-8-S1-A86.CrossRef

71.

Deeks ED. Mepolizumab: a review in eosinophilic asthma. BioDrugs. 2016;30(4):361–70. https://doi.org/10.1007/s40259-016-0182-5.CrossRefPubMed

72.

Zhang L, Borish L, Smith A, Somerville L, Albon D. Use of mepolizumab in adult patients with cystic fibrosis and an eosinophilic phenotype: case series. Allergy, Asthma Clin Immunol. 2020;16:3. https://doi.org/10.1186/s13223-019-0397-3.CrossRef

73.

Bleecker ER, Wechsler ME, FitzGerald JM, Menzies-Gow A, Wu Y, Hirsch I, et al. Baseline patient factors impact on the clinical efficacy of benralizumab for severe asthma. Eur Respir J. 2018;52(4):1800936. https://doi.org/10.1183/13993003.00936-2018.CrossRefPubMedPubMedCentral

74.

Tan LD, Bratt JM, Godor D, Louie S, Kenyon NJ. Benralizumab: a unique IL-5 inhibitor for severe asthma. J Asthma Allergy. 2016;9:71–81. https://doi.org/10.2147/JAA.S78049.CrossRefPubMedPubMedCentral

75.

Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, et al. Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med. 2015;373(3):220–31. https://doi.org/10.1056/NEJMoa1409547.CrossRefPubMedPubMedCentral

76.

Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011;365(18):1663–72. https://doi.org/10.1056/NEJMoa1105185.CrossRefPubMedPubMedCentral

77.

• Middleton PG, Mall MA, Dřevínek P, Lands LC, McKone EF, Polineni D, et al. Elexacaftor-tezacaftor-ivacaftor for cystic fibrosis with a single Phe508del allele. N Engl J Med. 2019;381(19):1809–19. https://doi.org/10.1056/NEJMOA1908639Comment: Randomized, double-blind, plecebo controlled phase 3trail showing efficacy of triple combination therapy in patients with CF who have a single copy of Fdel508 mutation.CrossRefPubMedPubMedCentral

Title: Asthma in Cystic Fibrosis: Definitions and Implications of This Overlap Syndrome
Authors: Chad R. Marion
Manuel Izquierdo
Holly C. Hanes
Christopher Barrios
Publication date: 01-02-2021
Publisher: Springer US
Keywords: Cystic Fibrosis
Omalizumab
Bronchial Asthma
Cystic Fibrosis
Mixed Connective Tissue Disease
Published in: Current Allergy and Asthma Reports / Issue 2/2021
Print ISSN: 1529-7322
Electronic ISSN: 1534-6315
DOI: https://doi.org/10.1007/s11882-020-00985-7

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Asthma in Cystic Fibrosis: Definitions and Implications of This Overlap Syndrome

Abstract

Purpose of Review

Recent Findings

Summary

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

Purpose of Review

Recent Findings

Summary

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