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Clinical diagnosis and management of food protein-induced enterocolitis syndrome

Leonard, Stephanie A.a; Nowak-Węgrzyn, Annab

doi: 10.1097/MOP.0b013e3283599ca1

Purpose of review To provide an overview of clinical manifestations, diagnosis and pathophysiology of food protein-induced enterocolitis syndrome (FPIES), an under-recognized and often misdiagnosed nonimmunoglobulin E-mediated food hypersensitivity. This review will highlight updates on natural history and clinical management.

Recent findings The main developments in FPIES involve epidemiology, common presentation and variants thereof, and natural history. Improved understanding and recognition of FPIES have influenced changes in clinical management.

Summary A large prospective population-based study reported cow's milk-FPIES cumulative incidence to be 0.34% by 1 year of age; immunoglobulin E-mediated cow's milk allergy was 0.5%. A case report has suggested that FPIES pathophysiology involves Th2 activation, and a shift away from Th2 signalling may be associated with resolution. Appreciation of the frequent incidence of multiple food-FPIES has influenced anticipatory guidance. Two case reports have described FPIES to food proteins in maternal breast milk. The threshold dose for FPIES reactivity may decrease with successive episodes. Reports from different populations indicate that children may outgrow FPIES sooner than previously thought.

aDepartment of Pediatrics, Division of Allergy & Immunology, University of California, San Diego, Rady Children's Hospital, San Diego, California

bJaffe Food Allergy Institute, Division of Pediatric Allergy, Mount Sinai School of Medicine, New York, New York, USA

Correspondence to Anna Nowak-Węgrzyn, MD, Jaffe Food Allergy Institute, Division of Pediatric Allergy, Mount Sinai School of Medicine, 1 Gustave L. Levy Place, Box 1198, New York, NY 10029-6574, USA. Tel: +1 212 241 5548; fax: +1 212 426 1902; e-mail:

© 2012 Lippincott Williams & Wilkins, Inc.