B0017 - Alpha-Gal Syndrome Complicating the Management of Suspected Pancreatic Exocrine Insufficiency

Nathan Richards, MD¹, Jeffrey Wilson, MD, PhD¹, Thomas Platts-Mills, MD, PhD, FRS², Robert Richards, MD^3
1University of Virginia, Charlottesville, VA; ²UVA Health, Charlottesville, VA; ³Gastroenterology Associates of Central Virginia, Lynchburg, VA

Introduction: IgE antibodies to the oligosaccharide galactose-α-1,3-galactose (α-gal) are an important cause of allergic reactions to mammalian meat and other mammal-derived products. The symptoms of α-gal syndrome (AGS) can involve urticaria or anaphylaxis, but increasingly we are aware that GI tract symptoms, including diarrhea, are also a major feature of AGS. Pancreatic exocrine insufficiency (PEI) is a common cause of diarrhea and treatment involves the use of pancreatic replacement enzymes (PRE). PRE are porcine derived and contain α-gal. Patients receiving PRE who are α-gal IgE positive are at risk for allergic reactions and GI symptoms due to α-gal sensitivity. Here we reviewed patients with suspected PEI and concomitant α-gal IgE sensitization in the practice of one gastroenterologist in Virginia.

Methods: Retrospective chart review was carried out using inclusion criteria of i) diarrhea, ii) low fecal elastase (< 200 μg/g feces), and iii) α-gal IgE sensitization ( >0.10 kU/L).

Results: 15 patients were identified with mean fecal elastase of 123 and median IgE α-gal level 0.96 (Table 1). 9 improved off of mammalian-containing food products and 3 of these did not require PRE. 11 patients received PRE. Of 5 patients with pre-existing systemic allergy symptoms to mammalian meat, 1 improved off of mammalian products and did not require PRE, 1 had increased diarrhea with Creon and was lost to follow up, 1 tolerated Creon with pruritus, 1 experienced hives from Creon but successfully underwent office-based desensitization, and 1 patient avoided PRE due to the severity of allergy symptoms. 6 patients without the classic cutaneous allergy symptoms of AGS tolerated PRE, though 1 developed some urticaria.

Discussion: In this series of 15 patients with suspected PEI who had concomitant α-gal IgE >0.1 kU/L, 60% improved with removal of α-gal containing products from the diet and 20% did not require PRE. Of the 11 patients who were treated with ongoing PRE, 3 experienced urticaria and 1 had increased diarrhea, but none had severe allergic symptoms. In our experience, patients who are sensitized to α-gal can usually tolerate PRE. Practitioners should also be aware that worsening diarrhea during PRE treatment could be a consequence of α-gal-related hypersensitivity, rather than medication non-compliance. Recognition of AGS superimposed upon PEI will allow improve management in this complex patient population.



Disclosures:


Nathan Richards, MD¹, Jeffrey Wilson, MD, PhD¹, Thomas Platts-Mills, MD, PhD, FRS², Robert Richards, MD³. B0017 - Alpha-Gal Syndrome Complicating the Management of Suspected Pancreatic Exocrine Insufficiency, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.