2526 A Rare Cause of Intestinal Pseudo-Obstruction: Gastrointestinal Amyloid

Abstract

INTRODUCTION: Amyloidosis is characterized by extracellular deposition of amyloid protein. It affects most commonly heart, kidney and GI tract. It can occur sporadically or in association with other conditions like multiple myeloma, chronic inflammatory diseases, infections etc. It has variable manifestations in GI tract from involving the stomach to the large bowel including liver. CASE DESCRIPTION/METHODS: This is 55 year old Caucasian male with past medical history of recent diagnosis of multiple myeloma, chronic kidney disease, hypertension, atrial flutter who presented to the hospital with chief complaints of multiple episodes of nausea and vomiting, diffuse abdominal pain, subjective fevers and chills from past 2 days. On presentation, vitals were stable. CBC- wnl, CMP – wnl, CT abdomen/pelvis without contrast revealed partial small bowel obstruction. Patient had similar symptoms in prior admissions. During the initial hospitalization 1 month ago, he underwent exploratory laparotomy with no findings to suggest mechanical small bowel obstruction. Subsequently, he had an EGD with push enteroscopy which identified enteritis and a Mallory-Weiss tear. Small bowel biopsies were performed with pathology noting: focal acute cryptitis and a background of moderate chronic nonspecific inflammation. Repeat EGD with push enteroscopy revealed non erosive esophagitis, mild gastritis and severe duodenitis, biopsies were obtained. Congo red staining of the biopsy revealed amyloidosis of the small bowel but the gastric biopsy was negative. The patient was then referred to higher center for consideration of bone marrow transplant. The fat pad biopsy was never obtained for systemic amyloidosis. DISCUSSION: The AL amyloidosis which is most commonly associated with monoclonal free light chains deposition, which was also previously called primary amyloidosis is most common. The AA amyloidosis (also called as secondary amyloidosis) is secondary to chronic inflammation in the body due to infection, rheumatoid arthritis etc. Diagnosis of amyloid, irrespective of the type, depends upon clinical, laboratory and histological findings. Serum and urine immunoelectrophoresis are the initial laboratory tests, but confirmation requires histological evaluation. Overall median survival in untreated patients or nonresponders with systemic amyloidosis is <2 years. Treatment should be directed at the underlying cause. GI complications are managed with symptomatic control.