University of Texas Medical Branch Galveston, Texas
Jordan Malone, DO1, Ashlynn Joseph, BSc2, Giri Movva, MD1, Anni Chowdhury, DO1, Raakhi Menon, DO1, Valerie Quach, BSc2, Kian Abdul-Baki, DO1, Jaison S. John, MD1, Harshwardhan M. Thaker, MBBS, PhD1, Sreeram Parupudi, MD1 1University of Texas Medical Branch, Galveston, TX; 2John Sealy School of Medicine, University of Texas Medical Branch, Galveston, TX
Introduction: Crohn’s Disease (CD) is a gastrointestinal (GI) disorder normally categorized by discontinuous chronic inflammation with focal crypt architectural distortion and granuloma formation occurring anywhere along the GI tract. Clinically significant isolated upper GI disease occurs in only 0.5-4% of all patients with CD. We describe the case of a middle-aged man with recurrent esophageal and duodenal stenosis presumably secondary to limited CD of the upper GI tract.
Case Description/Methods: This is a 47-year-old man with an ongoing history of GERD, dysphagia, and post-prandial abdominal pain dating back to 2016. Labs were notable for low vitamin D, normal inflammatory markers, and a positive scl70 antibody. Other laboratory work-up was unremarkable with negative H. pylori, syphilis, HIV, QuantiFERON gold, and CMV. He has undergone multiple upper endoscopies that have demonstrated severe gastritis and duodenitis with esophageal and duodenal strictures requiring dilation. Biopsy results were initially unclear but have since shown active gastritis and duodenitis with cryptitis, crypt abscesses, surface erosions, and increased lymphoplasmacytic inflammatory cells within the lamina propria and muscularis mucosa. Calcium phosphate crystals with giant cell reactions were seen on gastric biopsy during one endoscopy. Esophageal biopsies have shown increased intra-epithelial lymphocytes at the areas of stricture as well as in the distal and proximal esophagus. A colonoscopy was endoscopically normal, and biopsies were normal from terminal ileum, right and left colon, and rectum. Symptoms have improved with previous trials of steroids. A multi-disciplinary conference was held given the chronicity and complexity of his case. There is concern for scleroderma overlap given positive scl70 antibody and calcifications on gastric biopsy, however, the consensus suspicion is of limited CD of the upper GI tract. The decision was made to pursue empiric treatment of his presumed limited CD with anti-TNF alpha biologic therapy.
Discussion: The Vienna and Montreal classifications are commonly utilized tools to categorize the phenotypical presentations of CD with L4 disease (isolated upper GI tract) being any involvement of the GI tract proximal to the terminal ileum; more specific varieties such as L4-esophagogastruduodenal disease have also been described. This case demonstrates a rare version of limited CD of the upper GI tract and highlights the importance of a multi-disciplinary approach to treatment.
Figure: Top: Stenosis of upper esophagus; Upper left: Chronic active gastritis; Upper right: Chronic active gastritis with neutrophils in lamina propria; Lower left: Duodenal bulb stenosis; Lower right: Chronic active duodenitis with neutrophilic and eosinophilic infiltrate in the lamina propria with crypt distortion
Disclosures:
Jordan Malone indicated no relevant financial relationships.
Ashlynn Joseph indicated no relevant financial relationships.
Giri Movva indicated no relevant financial relationships.
Anni Chowdhury indicated no relevant financial relationships.
Raakhi Menon indicated no relevant financial relationships.
Valerie Quach indicated no relevant financial relationships.
Kian Abdul-Baki indicated no relevant financial relationships.
Jaison John indicated no relevant financial relationships.
Harshwardhan Thaker indicated no relevant financial relationships.
Sreeram Parupudi indicated no relevant financial relationships.
Jordan Malone, DO1, Ashlynn Joseph, BSc2, Giri Movva, MD1, Anni Chowdhury, DO1, Raakhi Menon, DO1, Valerie Quach, BSc2, Kian Abdul-Baki, DO1, Jaison S. John, MD1, Harshwardhan M. Thaker, MBBS, PhD1, Sreeram Parupudi, MD1. P3675 - Something's Stuck: An Atypical Presentation of Crohn’s Disease, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.