High prevalence of eosinophilic esophagitis in patients with inherited connective tissue disorders

Background Eosinophilic esophagitis (EoE) is an emerging chronic inflammatory disease mediated by immune hypersensitization to multiple foods and strongly associated with atopy and esophageal remodeling. Objective We provide clinical and molecular evidence indicating a high prevalence of EoE in pati...

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Veröffentlicht in:	Journal of allergy and clinical immunology 2013-08, Vol.132 (2), p.378-386
Hauptverfasser:	Abonia, J. Pablo, MD, Wen, Ting, PhD, Stucke, Emily M., BA, Grotjan, Tommie, BS, Griffith, Molly S., BA, Kemme, Katherine A., BS, Collins, Margaret H., MD, Putnam, Philip E., MD, Franciosi, James P., MD, MS, MSCE, von Tiehl, Karl F., MD, Tinkle, Brad T., MD, PhD, Marsolo, Keith A., PhD, Martin, Lisa J., PhD, Ware, Stephanie M., MD, PhD, Rothenberg, Marc E., MD, PhD
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Allergy and Immunology Biological and medical sciences Biopsy Body mass index Child Child, Preschool Collagen Collagen Type VIII - genetics Connective Tissue Diseases - complications Connective Tissue Diseases - epidemiology Connective Tissue Diseases - genetics connective tissue disorders Disease Ehlers-Danlos syndrome Ehlers-Danlos Syndrome - complications Ehlers-Danlos Syndrome - epidemiology Ehlers-Danlos Syndrome - genetics eosinophil Eosinophilic esophagitis Eosinophilic Esophagitis - complications Eosinophilic Esophagitis - epidemiology Eosinophilic Esophagitis - genetics eosinophilic gastrointestinal disease Esophagus - metabolism Female Fundamental and applied biological sciences. Psychology Fundamental immunology Histology Humans hypermobility syndrome Male Marfan syndrome Marfan Syndrome - complications Marfan Syndrome - epidemiology Marfan Syndrome - genetics Medical sciences Patients Prevalence RNA, Messenger - genetics RNA, Messenger - metabolism Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Smooth muscle
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Zusammenfassung:	Background Eosinophilic esophagitis (EoE) is an emerging chronic inflammatory disease mediated by immune hypersensitization to multiple foods and strongly associated with atopy and esophageal remodeling. Objective We provide clinical and molecular evidence indicating a high prevalence of EoE in patients with inherited connective tissue disorders (CTDs). Methods We examined the rate of EoE among patients with CTDs and subsequently analyzed esophageal mRNA transcript profiles in patients with EoE with or without CTD features. Results We report a cohort of 42 patients with EoE with a CTD-like syndrome, representing 0.8% of patients with CTDs and 1.3% of patients with EoE within our hospital-wide electronic medical record database and our EoE research registry, respectively. An 8-fold risk of EoE in patients with CTDs (relative risk, 8.1; 95% confidence limit, 5.1-12.9; χ21 = 112.0; P
ISSN:	0091-6749 1097-6825
DOI:	10.1016/j.jaci.2013.02.030