Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
권호 표지
DOI QR코드

DOI QR Code

Epidemiology of Eosinophilic Esophagitis in Patients with Cystic Fibrosis: A Population-Based 5-Year Study

  • Alaber, Omar (Department of Pediatrics, Case Western Reserve University School of Medicine) ;
  • Sabe, Ramy (Department of Pediatrics, Case Western Reserve University School of Medicine) ;
  • Baez-Socorro, Virginia (Department of Pediatrics, Case Western Reserve University School of Medicine) ;
  • Sankararaman, Senthilkumar (Department of Pediatrics, Case Western Reserve University School of Medicine) ;
  • Roesch, Erica (Department of Pediatrics, Case Western Reserve University School of Medicine) ;
  • Sferra, Thomas J. (Department of Pediatrics, Case Western Reserve University School of Medicine)
  • Received : 2021.11.06
  • Accepted : 2022.05.19
  • Published : 2022.07.15
Download PDF
⟨ Previous Next ⟩

Abstract

Purpose: The prevalence of eosinophilic esophagitis (EoE) has been on the rise since it was first described in the 1990s. Several diseases and exogenous factors have been associated with EoE. Our aim was to investigate the epidemiology of EoE in cystic fibrosis (CF) patients. Methods: We identified individuals with CF from September 2014 to September 2019 within a database (IBM Explorys Solutions, Inc.). The prevalence of EoE in patients with CF was compared to the general population. Results: The database included 36,111,860 patients during the 5-year study period: 12,950 with CF (0.036%) and 28,090 with EoE (0.078%). EoE prevalence was higher in CF patients than the general population (46 in 10,000 vs. 7.8 in 10,000, p<0.001). Patients with CF and EoE were more likely to be male (50% vs. 33.5%, p<0.008), children (33.3% vs. 16.5%, p<0.001), and non-Hispanic (100% vs. 88.7%, p<0.001) than CF patients without EoE. CF with EoE patients were more likely to be children than EoE only (33.3% vs. 10.5%, p<0.001). Allergic conditions were generally more prevalent in CF with EoE than CF only (83.3% vs. 68.3%, p=0.01) and EoE only (83.3% vs. 69.3%, p=0.014). Conclusion: EoE is nearly 6-times more prevalent in CF patients. Those patients had higher incidence of other atopic conditions. EoE must be considered in the differential diagnosis of patients with CF presenting with dysphagia, refractory gastroesophageal reflux, vomiting, and other esophagus-related symptoms.

Keywords

References

  1. Carr S, Chan ES, Watson W. Eosinophilic esophagitis. Allergy Asthma Clin Immunol 2018;14(Suppl 2):58. Erratum in: Allergy Asthma Clin Immunol 2019;15:22. https://doi.org/10.1186/s13223-018-0287-0
  2. Spechler SJ. Eosinophilic esophagitis: novel concepts regarding pathogenesis and clinical manifestations. J Gastroenterol 2019;54:837-44. https://doi.org/10.1007/s00535-019-01604-7
  3. Davis BP, Rothenberg ME. Mechanisms of disease of eosinophilic esophagitis. Annu Rev Pathol 2016;11:365-93. https://doi.org/10.1146/annurev-pathol-012615-044241
  4. Sanders DB, Fink AK. Background and epidemiology. Pediatr Clin North Am 2016;63:567-84. https://doi.org/10.1016/j.pcl.2016.04.001
  5. Sathe MN, Freeman AJ. Gastrointestinal, pancreatic, and hepatobiliary manifestations of cystic fibrosis. Pediatr Clin North Am 2016;63:679-98. https://doi.org/10.1016/j.pcl.2016.04.008
  6. Goralski JL, Lercher DM, Davis SD, Dellon ES. Eosinophilic esophagitis in cystic fibrosis: a case series and review of the literature. J Cyst Fibros 2013;12:9-14. https://doi.org/10.1016/j.jcf.2012.09.002
  7. Capucilli P, Cianferoni A, Grundmeier RW, Spergel JM. Comparison of comorbid diagnoses in children with and without eosinophilic esophagitis in a large population. Ann Allergy Asthma Immunol 2018;121:711-6. https://doi.org/10.1016/j.anai.2018.08.022
  8. Dziekiewicz MA, Banaszkiewicz A, Urzykowska A, Lisowska A, Rachel M, Sands D, et al. Gastroesophageal reflux disease in children with cystic fibrosis. Adv Exp Med Biol 2015;873:1-7. https://doi.org/10.1007/5584_2015_154
  9. Button BM, Roberts S, Kotsimbos TC, Levvey BJ, Williams TJ, Bailey M, et al. Gastroesophageal reflux (symptomatic and silent): a potentially significant problem in patients with cystic fibrosis before and after lung transplantation. J Heart Lung Transplant 2005;24:1522-9. https://doi.org/10.1016/j.healun.2004.11.312
  10. Hauser B, De Schepper J, Malfroot A, De Wachter E, De Schutter I, Keymolen K, et al. Gastric emptying and gastro-oesophageal reflux in children with cystic fibrosis. J Cyst Fibros 2016;15:540-7. https://doi.org/10.1016/j.jcf.2015.12.015
  11. Mellor X, Schindler T, Saab S, Roesch E, Sferra T, Sankararaman S. Eosinophilic esophagitis in cystic fibrosis: a case series with long-term follow-up. Pediatr Pulmonol 2022;57:1557-61. https://doi.org/10.1002/ppul.25912
  12. Coffey MJ, Nielsen S, Wemheuer B, Kaakoush NO, Garg M, Needham B, et al. Gut microbiota in children with cystic fibrosis: a taxonomic and functional dysbiosis. Sci Rep 2019;9:18593. https://doi.org/10.1038/s41598-019-55028-7
  13. Hayden HS, Eng A, Pope CE, Brittnacher MJ, Vo AT, Weiss EJ, et al. Fecal dysbiosis in infants with cystic fibrosis is associated with early linear growth failure. Nat Med 2020;26:215-21. https://doi.org/10.1038/s41591-019-0714-x
  14. Thavamani A, Salem I, Sferra TJ, Sankararaman S. Impact of altered gut microbiota and its metabolites in cystic fibrosis. Metabolites 2021;11:123. https://doi.org/10.3390/metabo11020123
  15. Tam RY, van Dorst JM, McKay I, Coffey M, Ooi CY. Intestinal Inflammation and alterations in the gut microbiota in cystic fibrosis: a review of the current evidence, pathophysiology and future directions. J Clin Med 2022;11:649. https://doi.org/10.3390/jcm11030649
  16. Arias A, Lucendo AJ. Epidemiology and risk factors for eosinophilic esophagitis: lessons for clinicians. Expert Rev Gastroenterol Hepatol 2020;14:1069-82. https://doi.org/10.1080/17474124.2020.1806054
  17. D'Souza SM, Houston K, Keenan L, Yoo BS, Parekh PJ, Johnson DA. Role of microbial dysbiosis in the pathogenesis of esophageal mucosal disease: a paradigm shift from acid to bacteria? World J Gastroenterol 2021;27:2054-72. https://doi.org/10.3748/wjg.v27.i18.2054
  18. Wypych TP, Marsland BJ. Antibiotics as instigators of microbial dysbiosis: implications for asthma and allergy. Trends Immunol 2018;39:697-711. https://doi.org/10.1016/j.it.2018.02.008
  19. Faulkner AL, Grayling M, Shillitoe B, Brodlie M, Michaelis LJ. Characterising the allergic profile of children with cystic fibrosis. Immun Inflamm Dis 2022;10:60-9. https://doi.org/10.1002/iid3.540
  20. Fluge G, Olesen HV, Gilljam M, Meyer P, Pressler T, Storrosten OT, et al. Co-morbidity of cystic fibrosis and celiac disease in Scandinavian cystic fibrosis patients. J Cyst Fibros 2009;8:198-202. https://doi.org/10.1016/j.jcf.2009.02.002
  21. Lloyd-Still JD. Crohn's disease and cystic fibrosis. Dig Dis Sci 1994;39:880-5. https://doi.org/10.1007/BF02087437
  22. Trigo Salado C, Leo Carnerero E, de la Cruz Ramirez MD. Crohn's disease and cystic fibrosis: there is still a lot to learn. Rev Esp Enferm Dig 2018;110:835-6.
  23. Lyles J, Rothenberg M. Role of genetics, environment, and their interactions in the pathogenesis of eosinophilic esophagitis. Curr Opin Immunol 2019;60:46-53. https://doi.org/10.1016/j.coi.2019.04.004
  24. O'Shea KM, Aceves SS, Dellon ES, Gupta SK, Spergel JM, Furuta GT, et al. Pathophysiology of eosinophilic esophagitis. Gastroenterology 2018;154:333-45. https://doi.org/10.1053/j.gastro.2017.06.065
  25. Witmer CP, Susi A, Min SB, Nylund CM. Early infant risk factors for pediatric eosinophilic esophagitis. J Pediatr Gastroenterol Nutr 2018;67:610-5. https://doi.org/10.1097/MPG.0000000000002123
  26. Jensen ET, Kuhl JT, Martin LJ, Rothenberg ME, Dellon ES. Prenatal, intrapartum, and postnatal factors are associated with pediatric eosinophilic esophagitis. J Allergy Clin Immunol 2018;141:214-22. https://doi.org/10.1016/j.jaci.2017.05.018
  27. Dellon ES, Shaheen O, Koutlas NT, Chang AO, Martin LJ, Rothenberg ME, et al. Early life factors are associated with risk for eosinophilic esophagitis diagnosed in adulthood. Dis Esophagus 2021;34:doaa074. https://doi.org/10.1093/dote/doaa074
  28. Radano MC, Yuan Q, Katz A, Fleming JT, Kubala S, Shreffler W, et al. Cesarean section and antibiotic use found to be associated with eosinophilic esophagitis. J Allergy Clin Immunol Pract 2014;2:475-7.e1. https://doi.org/10.1016/j.jaip.2014.02.018
  29. Ng J, Friedmacher F, Pao C, Charlesworth P. Gastroesophageal reflux disease and need for antireflux surgery in children with cystic fibrosis: a systematic review on incidence, surgical complications, and postoperative outcomes. Eur J Pediatr Surg 2021;31:106-14. https://doi.org/10.1055/s-0040-1718750
  30. Woodley FW, Hayes D Jr, Kopp BT, Moore-Clingenpeel M, Machado RS, Nemastil CJ, et al. Gastroesophageal reflux in cystic fibrosis across the age spectrum. Transl Gastroenterol Hepatol 2019;4:69. https://doi.org/10.21037/tgh.2019.08.11
  31. Maqbool A, Pauwels A. Cystic fibrosis and gastroesophageal reflux disease. J Cyst Fibros 2017;16 Suppl 2:S2-13. https://doi.org/10.1016/j.jcf.2017.07.007
  32. Merwat SN, Spechler SJ. Might the use of acid-suppressive medications predispose to the development of eosinophilic esophagitis? Am J Gastroenterol 2009;104:1897-902. https://doi.org/10.1038/ajg.2009.87