What is esophageal Crohn’s Disease and how is treating and diagnosing it different from lower gastroenteric Crohn’s Disease?

Crohn’s Disease is an inflammatory bowel disease (IBD), causing chronic inflammation of the digestive system. It can affect any portion of the digestive tract, from the mouth to the anus, though it affects the ileum and colon most often. Among the most rare of Crohn’s Disease manifestations is esophageal Crohn’s Disease, only making up for 1.8% of cases in adults (Karam et al. 2024). Most of these cases are not isolated to the esophagus, but also involve lower GI involvement, especially of the ileum. Having such a small number of instances unfortunately means that research on the treatment and management of it is very limited. There is no standard line of treatment, however there are treatment options that have shown success in the past.

Diagnosing patients with esophageal Crohn’s disease can be a challenge, as it isn’t something that is typically looked for. The most common symptoms a patient might present with are dysphagia (the difficulty or inability to swallow), odynophagia (painful swallowing), and pain in the epigastric region. Other symptoms include heartburn, chest pain, and regurgitation. Endoscopic findings show that patients with esophageal Crohn’s disease often present with characteristic “punched out” ulcers, with 85% of patients showing them (Pimentel et al. 2019). Other findings are erythema (redness), erosion, fistulas, and stenoses. Histologically, granulomas are very common, with 57% of patients presenting with esophageal granulomas (Pimentel et al. 2019). 

Treatment of esophageal Crohn’s disease is based on that of ileocolonic Crohn’s. Most often systemic corticosteroids are used, as well as aminosalicylates, immunosuppressants, and biological therapy. Of patients that received treatment, 57.2% responded well and did not have relapse 21.4% responded to the treatment but relapsed, and 21.4% that didn’t respond well to the treatment (Pimentel et al. 2019). Esophageal dilation is a treatment that can be used to open up the esophagus a little more and make swallowing easier. In severe cases with frequent relapse or prevalent fistulas and stenoses, an esophagectomy may be necessary, which is a treatment that involves removing part or all of the esophagus and connecting the rest of the digestive tract in its place. 

Esophageal Crohn’s disease is a very rare presentation of Crohn’s disease, and as such has very little research on the best ways to treat or manage it. For my art project, I decided to draw the GI tract of an individual, and initially it looks normal. But when you hold the drawing up to the light, you see colors shine through. The purple indicates normal healthy parts of the tract, and the red indicates common spots that can get inflamed with Crohn’s disease. The materials used were a marker, paper, pencil, and acrylic paints.

Decker, G. A., Loftus, E. V., Pasha, T. M., Tremaine, W. J., & Sandborn, W. J. (2001). Crohn’s disease of the esophagus: Clinical features and outcomes. Inflammatory Bowel Diseases, 7(2), 113–119. https://doi.org/10.1097/00054725-200105000-00006

Karam, K., Fahmi, H., Bchara, S., Mrad, J., Nader, A. A., & Fiani, E. (2024). Crohn’s disease confined to the esophagus, with no involvement of the small or large bowel. European Journal of Case Reports in Internal Medicine. https://doi.org/10.12890/2024_004926Pimentel, A. M., Rocha, R., & Santana, G. O. (2019). Crohn’s disease of esophagus, stomach and duodenum. World Journal of Gastrointestinal Pharmacology and Therapeutics, 10(2), 35–49. https://doi.org/10.4292/wjgpt.v10.i2.35