Epidemiology of Non-Malignant Gastrointestinal Disorders in Individuals With Cystic Fibrosis in the U.S. From 2013 to 2018: A Population-Based National Study

Abstract

Introduction: Gastrointestinal (GI) manifestations in patients with cystic fibrosis (CF) have been explored, both anecdotally and in previous small-scale studies. The stronger association of CF with GI malignancy is well established; however, other non-malignant GI conditions appear to occur more frequently in CF patients. We sought to describe the epidemiology of GERD, constipation, intestinal obstruction, inflammatory bowel disease, celiac disease, and eosinophilic esophagitis in patients with CF. Methods: We queried a commercial database (Explorys Inc, Cleveland, OH), an aggregate of Electronic Health Record data from 26 major integrated healthcare systems in the US from 1999 to 2018. We identified an aggregated patient cohort of eligible patients with “Cystic Fibrosis” (CF), and aforementioned GI disorders between June 2013 and 2018, based on Systemized Nomenclature Of Medicine - Clinical Terms. We calculated the prevalence of the aforementioned GI disorders in CF. Results: Of the 34,589,040 total patients in the database between 2013 and 2018, 17,610 were diagnosed with CF (51 per 100,000). Compared to the general population, patients with CF had a significantly higher rate of GERD (26% vs 12%, OR: 2.47,CI: 2.39-2.56, P<.0001), constipation (16% vs 6.0%, OR: 2.97, CI: 2.85-3.09, P<.0001), intestinal obstruction ( 5.5% vs 1.1%, OR: 5.23, CI:4.90-5.58, P<.0001), Crohn’s Disease (1.1% vs 0.4%, OR: 2.57, CI:2.23-2.96, P<.0001), Ulcerative Colitis (0.8% vs 0.3%, OR: 2.13,CI: 1.81-2.52, P<.0001), Celiac disease ( 0.9% vs 0.3%, OR: 3.22, CI: 2.74-3.78, P<.0001), and Eosinophilic Esophagitis (0.2% vs .07%, OR: 3.15, CI: 2.30-4.29, P<.0001). Conclusion: This large study concurs with established research that shows that CF patients have significantly higher comorbid rates of GI disease compared to the general population. CFTR channels found in the epithelium throughout the GI tract may contribute to obstructive, inflammatory, and autoimmune disease in these patients. Because of these findings, clinicians should have higher suspicion for these conditions when treating CF patients.126_A Figure 1. Demographics of populations126_B Figure 2. Incidence and odds ratio of different GI conditions in cystic fibrosis and non CF patients126_C Figure 3. Prevalence of GI disorders in patients with and without CF