What Is FPIES Disease? Symptoms, Diagnosis & Triggers

Food Protein-Induced Enterocolitis Syndrome (FPIES) is a distinct and severe type of food allergy primarily affecting the gastrointestinal tract. Unlike more common food allergies, FPIES does not involve the immediate allergic antibody Immunoglobulin E (IgE). This condition is characterized by delayed reactions to specific food proteins, leading to a unique set of symptoms centered on the digestive system. FPIES primarily affects infants and young children.

Understanding FPIES

FPIES represents a non-IgE mediated immune response, operating through different immune pathways than typical IgE-mediated allergies. Standard allergic reactions, which involve IgE antibodies, usually manifest quickly with symptoms such as hives, wheezing, or swelling. In contrast, FPIES reactions are delayed, typically occurring one to four hours after consuming the trigger food.

The immune cells within the digestive system of an individual with FPIES react to specific food proteins, causing inflammation in the stomach and intestines. This localized inflammatory response leads to characteristic symptoms. Traditional allergy tests like skin prick tests or IgE blood tests are not helpful in diagnosing FPIES. The mechanism behind FPIES is a cell-mediated response.

Recognizing FPIES Reactions

FPIES reactions can present in two forms: acute and chronic. Acute FPIES reactions are often severe and involve repetitive, forceful vomiting, typically starting one to four hours after ingesting the trigger food. These reactions can lead to pallor, lethargy, and dehydration, sometimes progressing to shock due to fluid loss. Diarrhea may also occur, often several hours after the vomiting begins.

Chronic FPIES occurs when a trigger food is consumed regularly, leading to ongoing inflammation. Symptoms in chronic FPIES are typically milder and can include intermittent vomiting, chronic diarrhea, and poor weight gain or even weight loss. Common food triggers frequently include cow’s milk, soy, rice, and oats. Other triggers can be eggs, chicken, turkey, and certain seafood or vegetables like sweet potato and banana. Most children react to one or two specific foods.

Diagnosis and Management

Diagnosing FPIES relies heavily on a detailed clinical history, as there are no specific laboratory or skin tests for confirmation. Healthcare providers gather information about the timing, nature, and severity of symptoms following food ingestion. When the diagnosis remains unclear, a supervised oral food challenge (OFC) in a medical setting, such as a clinic or hospital, may be performed. During an OFC, small, measured amounts of a suspected trigger food are given, and the individual is closely monitored for a reaction, often for several hours.

The primary management strategy for FPIES is strict avoidance of identified trigger foods. In the event of an acute reaction, immediate treatment focuses on managing dehydration. This typically involves administering oral or intravenous fluids to replenish lost electrolytes and prevent shock. Anti-vomiting medications, such as ondansetron, may be used to help control severe emesis.

Unlike IgE-mediated allergies, epinephrine is generally not indicated for FPIES reactions unless there are atypical symptoms like hives or wheezing. Families are encouraged to work with their healthcare providers to develop an emergency action plan for accidental exposures.

Prognosis and Resolution

The outlook for FPIES is generally favorable, as the condition is often outgrown. Most children typically resolve their FPIES by three to five years of age, though the exact timeline can vary depending on the specific trigger food. For instance, FPIES reactions to cow’s milk and soy often resolve earlier than those to solid foods like rice or oats.

The resolution of FPIES is monitored through regular follow-up appointments with an allergist or gastroenterologist. These specialists can guide families on when and how to consider reintroducing trigger foods, often through another supervised oral food challenge, to confirm tolerance. This approach ensures that foods are reintroduced safely, helping to expand the individual’s diet and reduce dietary restrictions.