S2837 An Uncommon Diagnosis Presenting as Acute Blood Loss Anemia

Abstract

INTRODUCTION: Cryptogenic Multifocal Ulcerous Stenosing Enteritis (CMUSE) is a rare disease that is difficult to diagnose because of the nonspecific clinical manifestations and vague radiological findings across multiple diagnostic modalities. This abstract aims to present a case of CMUSE, re-iterate how its presentation can mimic other common GI conditions, and emphasize the importance of its consideration when the common GI conditions do not align with a patient's presentation. CASE DESCRIPTION/METHODS: 78 year old male presents with two episodes of hematochezia. Pt denied prior history of anemia or melena and was not on oral anticoagulation. He completed a colonoscopy which revealed pan diverticulosis and internal hemorrhoids. He continued to have intermittent hematochezia with significant anemia. A pill cam was then completed and showed distal small bowel with active bleeding from unclear etiology. A distal small bowel stricture was noted to be present, and for this reason, the pill cam was unable to pass. CT enterography was then recommended; this did not reveal a colonic stricture as was seen on the pill cam study, but rather, it revealed a 1cm enhancing nodule in the distal small bowel. Surgery was consulted for small bowel laparoscopy with plan for resection of the nodule to obtain pathology. Two small bowel tumors were observed in the distal ileum approximately 10cm apart in addition to a jejunal diverticulum with thickened tissue. Pathology reported non-specific shallow ulceration confined to the mucosa and submucosa and no identifiable granulomas. Working differential diagnoses at that time included: Crohn’s Disease, CMUSE, and Drug Induced Ulceration with Stenosis. Immunological work revealed negative C-ANCA, P-ANCA, Saccharomyces Cerevisiae Antibodies: IgG 4.5, IgA 5.2 (Relative Ranges 0–24.9). After negative antibody titers, the working diagnosis in this patient is CMUSE. DISCUSSION: Although this patient did not present with an ileus as many patients with CMUSE do, the anemia 2/2 GI bleeding, ulcerations and capsule retention in the distal small bowel are consistent with the diagnosis. Tao et al reported in 2019 that the most effective form of treatment is immunotherapy and surgery, the reported patient receiving the latter. However, the recurrence rate is high, and some patients become steroid dependent. Increasing awareness of this condition can contribute to identification of the optimal treatment of this rare condition in an effort to improve the lives of those affected.Figure 1.: One centimeter enhancing nodule in the distal small bowel noted on CT enterography.Figure 2.: Active bleeding in distal small bowel noted on pill cam study.