S2298 Gastrointestinal Amyloidosis in the Setting of IBD: A Diagnostic Challenge

Abstract

INTRODUCTION: Amyloidosis is a clinical entity characterized by abnormal deposition of protein that impair organ function and is associated with a high morbidity and mortality. Secondary amyloidosis is a rare complication that occurs with infectious or inflammatory conditions, such as inflammatory bowel disease (IBD). We present a patient with gastrointestinal bleeding and increased ostomy output found to have secondary amyloidosis in the setting of long-standing IBD. CASE DESCRIPTION/METHODS: An 81-year-old male with a history of ulcerative colitis (UC) s/p total colectomy and ileostomy presented with increased ostomy output. The patient had multiple previous admissions for high ostomy output for which he underwent prior upper endoscopy and ileoscopy (Figure 1). Biopsies revealed chronic ileitis associated with neutrophilic cryptitis and focal crypt abscesses. Given concern for Crohn’s disease, he received oral steroids. During his recent hospitalization, he had increased ileostomy output and fecal calprotectin of 211 (4x ULN). Ileoscopy revealed erythematous mucosa with submucosal punctate hemorrhages, friability, and erosions throughout the distal ileum (Figure 2). Pathology revealed active ileitis with ulcerations. He received high dose steroids and IV octreotide for two weeks, but continued to have increased output and developed acute kidney injury. Repeat fecal calprotectin was 893. Given the lack of response to treatment, congo red staining was added to prior biopsies revealing amyloid deposition in both the gastric and duodenal specimens (Figure 3). Given there was no plasma cell dyscrasias, the amyloid deposition was felt secondary to his chronic inflammation from his IBD. Course was further complicated by small bowel bleeding requiring embolization. Unfortunately, his clinical status continued to deteriorate and the patient eventually died from hypoxic respiratory failure. DISCUSSION: Gastrointestinal amyloidosis (GIA) in the setting of IBD is exceedingly rare. While patients with UC can have extraintestinal disease following total colectomy, we have not found any reports in the literature of post-total colectomy GIA. Treatment of GIA is focused on the underlying etiology; however, given the non-specific signs and symptoms this can often delay the diagnosis. In severe cases, high dose steroids and somatostatin analogues may be used; although, some patients may be refractory as in this case. In conclusion, a high index of suspicion is needed to establish an early diagnosis, thus decreasing complications.Figure 1.: Ileoscopy revealing punctate submucosal hemorrhages and erosions within the distal ileum.Figure 2.: Ileoscopy revealing erythematous mucosa with submucosal punctate hemorrhages, friability, and erosions throughout the middle and distal portions of the ileum.Figure 3.: Congo red stain of the duodenal biopsy revealing vascular amyloid deposition (blue arrow). The waxy orange material represents the amyloid which deposits into the wall of a blood vessel.