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A5179 - Gastrointestinal Amyloidosis Presenting as Acute Gastroenteritis in a Hemodialysis Patient
Author Block: P. Sharma, P. Macauley, M. Marcelin; Internal Medicine, Maimonides Medical Center, Brooklyn, NY, United States.
Introduction Amyloidosis is characterized by systemic or local deposition of abnormal fibrous proteins in the interstitium of organs and tissues resulting in dysfunction. Secondary Amyloidosis results from deposition of AA protein, an acute phase reactant associated with chronic inflammatory states. Dialysis related amyloidosis (DRA) usually presents with joint involvement; gastrointestinal involvement is rare. We present a case of an 81 year old male who presents with symptoms of acute gastroenteritis. Case presentation An 81 year old male with end stage renal disease on hemodialysis, presented with non-bloody, non-bilious vomiting and watery diarrhea for one day. Patient was hypotensive with BP of 50/25mmHg and was resuscitated with crystalloid infusion and initiation of an inotropic agent. Broad spectrum antibiotics were also started. CT abdomen and pelvis showed pancolitis, most severe in the rectum and sigmoid with mild small bowel obstruction. Stool studies showed negative clostridium difficile toxin and antigen, ova and parasites; atypical yeast cells were however present. Anti-saccharomyces cerevisiae antibody was positive with a negative p-ANCA. Diarrhea continued with hemodynamic stability. Flexible sigmoidoscopy showed diffusely inflamed erythematous, edematous and friable mucosa of rectum and sigmoid. Mesalamine therapy was initiated. The biopsy specimen of the rectum and sigmoid followed by staining with Congo red yielded positive for amyloid deposition. Immunohistochemical staining showed no kappa or lambda restriction. Serum immunoglobulin kappa and lambda were high with a normal ratio. The presumed diagnosis was secondary AA as a result of chronic hemodialysis. Fifteen days after admission he became hemodynamically unstable with a change in mental status and was transferred to the MICU. During intubation he had massive hematemesis and became pulseless unfortunately, patient could not be resuscitated and subsequently expired. Conclusion DRA is thought to be due to increased levels of β2 microglobulin due to a marked reduction of its catabolism which over the course of time deposits in tissues. Gastrointestinal amyloidosis should be considered in hemodialysis patients who present with unexplained diarrhea. Although there is no treatment for DRA, it can be prevented by early renal transplantation in those patients that will be candidates. In those that are not, studies have suggested that highly biocompatible and high-flux membranes used in dialysis decreases plasma β2 microglobulin levels. References: Dialysis-related amyloidosis: challenges and solutions. 2016. Scarpioni R, Ricardi M, Albertazzi V, De Amicis S, Rastelli F, Zerbini L. Int J Nephrol Renovasc Dis. 7;9:319-328