Recurrent Hemorrhagic Enteritis: A Challenging Case of Presumed Proximal Small Bowel Crohn's Disease

Background:
Isolated upper gastrointestinal (GI) Crohn’s disease can present a significant diagnostic challenge. It is relatively uncommon and has protean clinical manifestations. We present a case of recurrent hemorrhagic jejunal ulcerations likely related to Crohn’s disease.
Methods:
A 47-year-old male with unexplained recurrent bleeding proximal small bowel ulcers was referred to our inflammatory bowel disease center for further evaluation. He was admitted a year earlier to his local hospital for symptomatic anemia and melena and was found to have a hemoglobin of 10 g/dL. Upper endoscopy and colonoscopy revealed non-bleeding duodenal ulceration. He was managed conservatively and discharged home. However, he was readmitted to the same hospital twice more for recurrent melena and symptomatic anemia, with his hemoglobin decreasing further to 7 g/dL. Repeat upper endoscopy was notable for bleeding ulcers in the distal duodenum and proximal jejunum. Biopsies showed moderate acute and chronic inflammation in the lamina propria with focal mild villous blunting. Helicobacter pylori testing and gastrin levels were normal; a Meckel scan was negative. After 3 more episodes of GI bleeding requiring hospitalization, he was eventually referred to our center. Additional workup including repeating the previously mentioned tests, video capsule endoscopy, CT angiography, and MRI were all unrevealing. Ultimately, he underwent a partial small bowel resection with pathology reporting multiple ulcerations, transmural inflammation, and aphthous ulcers from the distal duodenum to proximal jejunum.
Results:
Two weeks later, he returned to our emergency department with hemodynamically unstable GI bleeding. A small bowel enteroscopy demonstrated recurrent ulceration both proximal and distal to the anastomotic site. Fecal calprotectin was markedly elevated at 1810 µg/mg and mesenteric duplex was otherwise normal. Additional outpatient work up including labs to rule out vasculitis, angioedema, and autoimmune enteropathy were all unremarkable. A presumptive diagnosis of small bowel Crohn’s was made, and he was started on budesonide. A therapeutic trial of Infliximab was ineffective with frequent flares. Most recently, he was started on risankizumab and has been in clinical remission for the last 10 months, as well as an improvement noted in the jejunal ulceration.
Conclusions:
Isolated upper GI Crohn’s disease presents unique diagnostic and management challenges due to its heterogeneous presentation and refractory nature. While nausea, vomiting, and weight loss are typical, our patient experienced several episodes of life-threatening GI bleeding from duodenal and jejunal ulcers. Curiously, despite surgery, the disease recurred rapidly. Anti-TNF therapy has been the mainstay of treatment for upper GI Crohn’s disease. However, there is limited data on therapy selection in cases of anti-TNF failure for this condition. To our knowledge, this report is the first to document the induction of remission for isolated upper GI Crohn’s disease with risankizumab. These findings provide anecdotal justification for considering risankizumab as a treatment option in refractory cases of isolated upper GI Crohn’s disease.