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Food protein-induced enterocolitis syndrome

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Food protein-induced enterocolitis syndrome (FPIES) is a systemic, non IgE-mediated food allergy to a specific trigger within food, most likely food protein. As opposed to the more common IgE food allergy, which presents within seconds with rash, hives, difficulty breathing or anaphylaxis, FPIES presents with a delayed reaction where vomiting is the primary symptom. In its acute form, FPIES presents with vomiting that typically begins 1 to 4 hours after trigger food ingestion, alongside paleness of the skin, lethargy, and potentially blood-tinged diarrhea. In the severe form of acute FPIES, continued vomiting may cause severe dehydration or hypotensive shock-like state, requiring hospitalization. In its chronic form, continued exposure to trigger foods results in chronic or episodic vomiting, poor weight gain, failure to thrive, and watery or blood-tinged diarrhea.[1] FPIES can potentially develop at any age, from infancy to adulthood, but most commonly develops within the first few years of life and resolves in early childhood.[1][2][3][4] Atypical FPIES presents with evidence of specific IgE-sensitization via positive specific serum or skin IgE testing to trigger foods; atypical FPIES may prolong time to disease resolution or increase risk of conversion to IgE-mediated food allergy.[5]

Epidemiology

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Historically, symptoms resembling FPIES were first reported in the 1960s, but awareness of the disease was limited for decades after. More recently, awareness has increased with establishment of an ICD-10 code in 2016, and the publication of the first international consensus guidelines for FPIES diagnosis by the AAAAI in 2017.[1][6]

To date, various studies have estimated FPIES incidence to be between 0.015% and 0.7%. However, establishing the true prevalence of FPIES is difficulty because of how few population-level (large-scale) studies there are, the relatively recent establishment of standard diagnostic criteria, and under-diagnosis due to disease rarity and lack of awareness.[7] A 2019 United States population-level survey estimated a FPIES prevalence of 0.51% in children.[8] Similar prevalences in children have been found in population-level estimates from Israeli and Spanish studies.[9]

Adult-onset FPIES is far rarer and not as well understood at this time. The prevalence is uncertain, but reported cases thus far have been predominantly female.[10] Median age of onset in reported cases has been between ages 20-40 with 6-8 years of symptoms before diagnosis.[5]

Comorbid Atopy

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Numerous studies have found that children with FPIES, compared to those who do not, have significantly higher rates of atopic conditions such as asthma, atopic dermatitis (eczema), IgE-mediated food allergy, and allergic rhinitis.[11][12]However, the data does not suggest that prior history of FPIES puts children at risk developing those atopic conditions in the future.

Diagnosis

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Diagnosis is based on the presence of certain symptoms (see below) and not any laboratory tests, as specific IgE and skin prick tests are typically negative (except in cases of atypical FPIES).[13] Additionally, potential trigger foods may be given in small amounts to patients to see whether an FPIES reaction occurs.[14] Differential diagnoses must also be ruled out (see section below). No laboratory test or procedure is currently recommended for FPIES diagnosis.

The underlying pathophysiology of FPIES is not understood at this time, though it is generally understood to be non-IgE mediated. One study found that in patients with non-IgE mediated food allergy, Th2 lymphoproliferative responses were similar to that of patients with IgE-mediated allergies, suggesting an underlying T-cell mechanism of action.[15] Another study found elevated IL-17 markers, elevated innate inflammatory markers, and increased T-cell activation after FPIES reaction.[16]

Acute FPIES

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Per international consensus guidelines published in 2017 by the American Academy of Allergy, Asthma and Immunology, acute FPIES diagnosis may be established in a patient who meets the following major criterion and at least three minor criteria:[17]

Major criterion: Vomiting approximately 1-4 hours following oral consumption of a suspected trigger food, without signs of classic IgE-mediated skin or respiratory allergic symptoms (i.e. hives, itchy skin, stridor, wheezing, tightness in throat).

Minor criteria:

  • Second episode of vomiting after eating same food which provoked first episode of vomiting
  • Repetitive vomiting 1-4 hours after eating a different food
  • Significant lethargy
  • Pallor (paleness of skin)
  • Required emergency department or urgent care visit due to reaction
  • Required IV fluid administration due to reaction
  • Diarrhea within 24 hours of consuming trigger (may or may not be bloody)
  • Hypotension
  • Hypothermia

Current acute FPIES guidelines further divide acute FPIES reactions into mild to moderate and severe disease presentation. Mild to moderate disease typically presents with 1-3 episodes of vomiting around 1-4 hours after trigger ingestion, reduced activity level pallor, which usually self-resolves without medical intervention, and/or mild diarrhea. Severe disease typically presents with 4+ episodes of bilious and/or projectile vomiting within 1-4 hours, along with possible hypotension, shock, severe dehydration, diarrhea, lethargy, hypothermia, abdominal distension, and/or need for IV rehydration.[1][17][2][3] Laboratory studies in more severe cases might reveal hypoalbuminemia, anemia, eosinophilia, and elevated white blood cell count with a left shift.

Atypical FPIES has all the same symptoms as FPIES, but laboratory tests will show some IgE reaction to the trigger food, with IgE present either via blood testing or skin prick testing. This does not mean that atypical FPIES is controlled by IgE. Current studies suggest that children with atypical FPIES take longer to grow out of the condition than children with 'typical' FPIES. It is also possible that atypical FPIES may later transform into an IgE allergy.[5]

Adult-onset FPIES may present differently: based on limited data, adults most commonly present with severe cramping abdominal pain hours after trigger food ingestion; vomiting is only present in around 60% of cases. Nausea and diarrhea may also be present, but the pattern of symptoms varies. Adult-onset FPIES is not recognized under the 2017 consensus guidelines, which may lead to misdiagnosis or under-diagnosis. [5][10] [18]

Chronic FPIES

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Per current guidelines, chronic FPIES with more frequent exposure to trigger foods (typically either milk or soy in baby formula) has a severe presentation: progressive vomiting with diarrhea, which may result in dehydration, metabolic acidosis, failure to thrive and/or hospitalization. Less frequent exposure to trigger foods may present with mild, intermittent vomiting, diarrhea, and/or poor weight gain, but without dehydration or hypotension. Crucially, chronic FPIES is confirmed by the cessation of symptoms within days of removing suspected trigger foods from the diet; additionally, re-introduction of trigger food at a later date will result in an acute FPIES reaction.[1]

Differential Diagnosis

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FPIES can be told apart from IgE-mediated food allergy (the most common type of food allergy) by both timing and symptoms. IgE-mediated allergic reactions occur within seconds to minutes of food ingestion, whereas FPIES is a delayed reaction which presents at minimum 30-60 minutes after ingestion. Whereas IgE-mediated reactions may present with itchy rash, hives, wheezing, difficulty breathing, or anaphylaxis; these symptoms do not present in FPIES.[1][7]

Other differential diagnoses for FPIES include infectious gastroenteritis, celiac disease, inflammatory bowel disease, necrotizing enterocolitis, food protein-induced enteropathy, food protein-induced proctocolitis, and eosinophilic gastroenteritis, among others.[19]

Of note, some data indicates that current consensus diagnostic guidelines may under-diagnose in certain cases; one study found that up to 1/4 of patients with high clinical suspicion of FPIES in a multi-center cohort study in Spain did not meet the 2017 international consensus guidelines, which may suggest different FPIES presentation depending on geographic location, or varying severity of FPIES.[20]

Management

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Trigger Avoidance

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There is currently no treatment for FPIES except avoidance of known trigger foods. The most common FPIES triggers across most published studies have been cow's milk, soy, grains (especially oats and rice). However, reactions are possible to a number of solid foods, such as eggs, fish, shellfish, meats, peanut, tree nuts, sweet potatoes, and fruits (i.e. banana, avocado, etc.).[1][2][3][6][12] The list of potential food triggers is varied and can be somewhat region specific; for example, studies in Spain report higher rates of seafood FPIES.[6] There are also cases of FPIES being transmitted through foods in breast milk in rare occasions.[21]

At present, the only way to know whether an FPIES allergy has definitely resolved is to give the trigger food and observe whether a reaction occurs. This is called an oral food challenge (OFC) and is often done in an outpatient clinic setting to quickly control any symptoms. Current guidelines from the AAAAI in 2020 suggest giving 0.06 to 0.6 grams of food protein per kg of patient body weight, with larger amounts given for older children.[14] However, there is still some variety among allergists in their method of OFC.[22]

Management of FPIES reaction

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During an acute FPIES episode, if symptoms are mild (1-2 vomiting episodes) ondansetron or infacol may be given to control vomiting in children over 6 months of age, along with oral rehydration. If vomiting persists or if child has more severe symptoms (i.e. lethargy, pallor, hypotonia) then the child should be taken to an emergency room or hospital for intramuscular or intravenous ondansetron with IV rehydration.[7][23] The use of epinephrine or antihistamines is not recommended, as FPIES is not an IgE-mediated reaction.

Chronic FPIES reactions may present with more severe dehydration, hypotension, metabolic acidosis and/or failure to thrive, which requires hospitalization for IV rehydration and nutritional management.

Prognosis

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Data regarding time to FPIES resolution varies significantly, and appears to depend on factors such as the specific food trigger and whether it is atypical FPIES. On balance, available data suggests that a majority of FPIES cases to common trigger foods (cow's milk, rice, oat, soy) resolve by age 5, if not sooner.[1][12][24][25]

The impact of FPIES may also have significant psychological and social burdens on the family of those afflicted. Parents of kids with FPIES report more stress, worry, anxiety, and reduced self-efficacy, which negatively affects their healthcare-related quality of life.[26][27]

References

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  1. ^ a b c d e f g h Nowak-Węgrzyn A, Chehade M, et al. (2017). "International consensus guidelines for the diagnosis and management of food protein-induced enterocolitis syndrome: Executive summary-Workgroup Report of the Adverse Reactions to Foods Committee, American Academy of Allergy, Asthma & Immunology". J. Allergy Clin. Immunol. 139 (4): 1111–1126.e4. doi:10.1016/j.jaci.2016.12.966. hdl:10044/1/48017. PMID 28167094.
  2. ^ a b c Nowak-Węgrzyn A, Jarocka-Cyrta E, Moschione Castro A (2017). "Food Protein-Induced Enterocolitis Syndrome" (PDF). J Investig Allergol Clin Immunol. 27 (1): 1–18. doi:10.18176/jiaci.0135. PMID 28211341.
  3. ^ a b c Michelet M, Schluckebier D, Petit LM, Caubet JC (2017). "Food protein-induced enterocolitis syndrome - a review of the literature with focus on clinical management". J Asthma Allergy. 10: 197–207. doi:10.2147/JAA.S100379. PMC 5499953. PMID 28721077.
  4. ^ Ruffner, MA (November 2014). "Food Protein-Induced Enterocolitis Syndrome: Insights From Review of a Large Referral Population" (PDF). Pediatrics. 134: S157. doi:10.1542/peds.2014-1817PP. PMID 25363948. S2CID 46053309. Archived from the original (PDF) on 19 July 2018.
  5. ^ a b c d Anvari, Sara; Ruffner, Melanie A.; Nowak-Wegrzyn, Anna (April 2024). "Current and future perspectives on the consensus guideline for food protein-induced enterocolitis syndrome (FPIES)". Allergology International. 73 (2): 188–195. doi:10.1016/j.alit.2024.01.006. PMID 38326194.
  6. ^ a b c Bartnikas, Lisa M.; Nowak-Wegrzyn, Anna; Schultz, Fallon; Phipatanakul, Wanda; Bingemann, Theresa A. (May 2021). "The evolution of food protein–induced enterocolitis syndrome". Annals of Allergy, Asthma & Immunology. 126 (5): 489–497. doi:10.1016/j.anai.2021.01.001. PMC 8102378. PMID 33444729.
  7. ^ a b c Anvari, Sara; Ruffner, Melanie A.; Nowak-Wegrzyn, Anna (April 2024). "Current and future perspectives on the consensus guideline for food protein-induced enterocolitis syndrome (FPIES)". Allergology International. 73 (2): 188–195. doi:10.1016/j.alit.2024.01.006. PMID 38326194.
  8. ^ Nowak-Wegrzyn, Anna; Warren, Christopher M.; Brown-Whitehorn, Terri; Cianferoni, Antonella; Schultz-Matney, Fallon; Gupta, Ruchi S. (October 2019). "Food protein–induced enterocolitis syndrome in the US population–based study". Journal of Allergy and Clinical Immunology. 144 (4): 1128–1130. doi:10.1016/j.jaci.2019.06.032. PMC 7923683. PMID 31288044.
  9. ^ Katz, Yitzhak; Goldberg, Michael R.; Rajuan, Nelly; Cohen, Adi; Leshno, Moshe (March 2011). "The prevalence and natural course of food protein–induced enterocolitis syndrome to cow's milk: A large-scale, prospective population-based study". Journal of Allergy and Clinical Immunology. 127 (3): 647–653.e3. doi:10.1016/j.jaci.2010.12.1105. PMID 21377033.
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  11. ^ Ruffner, Melanie A.; Wang, Kathleen Y.; Dudley, Jesse W.; Cianferoni, Antonella; Grundmeier, Robert W.; Spergel, Jonathan M.; Brown-Whitehorn, Terri F.; Hill, David A. (March 2020). "Elevated Atopic Comorbidity in Patients with Food Protein–Induced Enterocolitis". The Journal of Allergy and Clinical Immunology: In Practice. 8 (3): 1039–1046. doi:10.1016/j.jaip.2019.10.047. PMC 7064432. PMID 31759160.
  12. ^ a b c Caubet, Jean Christoph; Ford, Lara Simone; Sickles, Laura; Järvinen, Kirsi M.; Sicherer, Scott H.; Sampson, Hugh A.; Nowak-Węgrzyn, Anna (August 2014). "Clinical features and resolution of food protein–induced enterocolitis syndrome: 10-year experience". Journal of Allergy and Clinical Immunology. 134 (2): 382–389.e4. doi:10.1016/j.jaci.2014.04.008. PMID 24880634.
  13. ^ NIAID-Sponsored Expert Panel; Boyce, J. A.; Assa'Ad, A.; Burks, A. W.; Jones, S. M.; Sampson, H. A.; Wood, R. A.; Plaut, M.; Cooper, S. F.; Fenton, M. J.; Arshad, S. H.; Bahna, S. L.; Beck, L. A.; Byrd-Bredbenner, C.; Camargo Jr, C. A.; Eichenfield, L.; Furuta, G. T.; Hanifin, J. M.; Jones, C.; Kraft, M.; Levy, B. D.; Lieberman, P.; Luccioli, S.; McCall, K. M.; Schneider, L. C.; Simon, R. A.; Simons, F. E.; Teach, S. J.; Yawn, B. P.; Schwaninger, J. M. (December 2010). "Guidelines for the Diagnosis and Management of Food Allergy in the United States: Report of the NIAID-Sponsored Expert Panel". Journal of Allergy and Clinical Immunology. 126 (6): S1–S58. doi:10.1016/j.jaci.2010.10.007. PMC 4241964. PMID 21134576.
  14. ^ a b Bird, J. Andrew; Leonard, Stephanie; Groetch, Marion; Assa'ad, Amal; Cianferoni, Antonella; Clark, April; Crain, Maria; Fausnight, Tracy; Fleischer, David; Green, Todd; Greenhawt, Matthew; Herbert, Linda; Lanser, Bruce J.; Mikhail, Irene; Mustafa, Shahzad (January 2020). "Conducting an Oral Food Challenge: An Update to the 2009 Adverse Reactions to Foods Committee Work Group Report". The Journal of Allergy and Clinical Immunology: In Practice. 8 (1): 75–90.e17. doi:10.1016/j.jaip.2019.09.029. PMID 31950914.
  15. ^ Morita, Hideaki; Nomura, Ichiro; Orihara, Kanami; Yoshida, Koichi; Akasawa, Akira; Tachimoto, Hiroshi; Ohtsuka, Yoshikazu; Namai, Yoshiyuki; Futamura, Masaki; Shoda, Tetsuo; Matsuda, Akio; Kamemura, Norio; Kido, Hiroshi; Takahashi, Takao; Ohya, Yukihiro (February 2013). "Antigen-specific T-cell responses in patients with non–IgE-mediated gastrointestinal food allergy are predominantly skewed to TH2". Journal of Allergy and Clinical Immunology. 131 (2): 590–592.e6. doi:10.1016/j.jaci.2012.09.005. PMID 23083674.
  16. ^ Berin, M. Cecilia; Lozano-Ojalvo, Daniel; Agashe, Charuta; Baker, Mary Grace; Bird, J. Andrew; Nowak-Wegrzyn, Anna (September 2021). "Acute FPIES reactions are associated with an IL-17 inflammatory signature". Journal of Allergy and Clinical Immunology. 148 (3): 895–901.e6. doi:10.1016/j.jaci.2021.04.012. PMC 8675150. PMID 33891982.
  17. ^ a b Nowak-Węgrzyn, Anna; Chehade, Mirna; Groetch, Marion E.; Spergel, Jonathan M.; Wood, Robert A.; Allen, Katrina; Atkins, Dan; Bahna, Sami; Barad, Ashis V.; Berin, Cecilia; Brown Whitehorn, Terri; Burks, A. Wesley; Caubet, Jean-Christoph; Cianferoni, Antonella; Conte, Marisa (April 2017). "International consensus guidelines for the diagnosis and management of food protein–induced enterocolitis syndrome: Executive summary—Workgroup Report of the Adverse Reactions to Foods Committee, American Academy of Allergy, Asthma & Immunology". Journal of Allergy and Clinical Immunology. 139 (4): 1111–1126.e4. doi:10.1016/j.jaci.2016.12.966. hdl:10807/130792. PMID 28167094.
  18. ^ Du, Yue (Jennifer); Nowak-Węgrzyn, Anna; Vadas, Peter (February 2018). "FPIES in adults". Annals of Allergy, Asthma & Immunology. 121 (6): 736–738. doi:10.1016/j.anai.2018.08.003. PMID 30121366.
  19. ^ Feuille E, Nowak-Węgrzyn A (2015). "Food Protein-Induced Enterocolitis Syndrome, Allergic Proctocolitis, and Enteropathy". Curr Allergy Asthma Rep. 15 (8): 50. doi:10.1007/s11882-015-0546-9. PMID 26174434. S2CID 6651513.
  20. ^ Vazquez-Ortiz, Marta; Argiz, Laura; Machinena, Adrianna; Echeverria, Luis; Blasco, Cristina; Prieto, Ana; Infante, Sonsoles; Vila, Leticia; Garcia, Emilio; Gonzalez-Delgado, Purificación; Vazquez-Cortes, Sonia; Barni, Simona; Martinon-Torres, Frederico; Gomez-Carballa, A.; Boyle, R. (May 2020). "Diagnostic criteria for acute FPIES: What are we missing?". The Journal of Allergy and Clinical Immunology: In Practice. 8 (5): 1717–1720.e2. doi:10.1016/j.jaip.2019.11.034. PMID 31821916.
  21. ^ Monti G, Castagno E, Liguori SA, Lupica MM, Tarasco V, Viola S, et al. Food protein-induced enterocolitis syndrome by cow's milk proteins passed through breast milk. The Journal of Allergy and Clinical Immunology 2011; 127:679-80. PMID: 21146866
  22. ^ Nicolaides, Rory; Bird, J. Andrew; Cianferoni, Antonella; Brown-Whitehorn, Terri; Nowak-Wegrzyn, Anna (October 2021). "Oral Food Challenge for FPIES in Practice—A Survey: Report from the Work Group on FPIES Within the Adverse Reactions to Foods Committee, FAED IS, AAAAI". The Journal of Allergy and Clinical Immunology: In Practice. 9 (10): 3608–3614.e1. doi:10.1016/j.jaip.2021.06.061. PMID 34483087.
  23. ^ Leonard, Stephanie A.; Miceli Sopo, Stefano; Baker, Mary Grace; Fiocchi, Alessandro; Wood, Robert A.; Nowak-Węgrzyn, Anna (May 2021). "Management of acute food protein-induced enterocolitis syndrome emergencies at home and in a medical facility". Annals of Allergy, Asthma & Immunology. 126 (5): 482–488.e1. doi:10.1016/j.anai.2021.01.020. PMID 33493640.
  24. ^ Wang, Kathleen Y.; Lee, Juhee; Cianferoni, Antonella; Ruffner, Melanie A.; Dean, Amy; Molleston, Jerome M.; Pawlowski, Nicholas A.; Heimall, Jennifer; Saltzman, Rushani W.; Ram, Gita S.; Fiedler, Joel; Gober, Laura M.; Spergel, Jonathan M.; Brown-Whitehorn, Terri F. (February 2019). "Food Protein–Induced Enterocolitis Syndrome Food Challenges: Experience from a Large Referral Center". The Journal of Allergy and Clinical Immunology: In Practice. 7 (2): 444–450. doi:10.1016/j.jaip.2018.09.009. PMID 30243880.
  25. ^ Cherian S, Varshney P (April 2018). "Food Protein-Induced Enterocolitis Syndrome (FPIES): Review of Recent Guidelines". Curr Allergy Asthma Rep. 18 (4): 28. doi:10.1007/s11882-018-0767-9. PMID 29623454. S2CID 4705873.
  26. ^ Flom, Julie D.; Groetch, Marion; Kovtun, Kyle; Westcott-Chavez, Amity; Schultz, Fallon; Nowak-Wegrzyn, Anna (September 2023). "Feeding difficulties in children with food protein–induced enterocolitis syndrome". The Journal of Allergy and Clinical Immunology: In Practice. 11 (9): 2939–2941.e3. doi:10.1016/j.jaip.2023.05.035. PMID 37263350.
  27. ^ Maciag, Michelle C.; Herbert, Linda J.; Sicherer, Scott H.; Young, Michael C.; Schultz, Fallon; Westcott-Chavez, Amity A.; Phipatanakul, Wanda; Bingemann, Theresa A.; Bartnikas, Lisa M. (November 2020). "The Psychosocial Impact of Food Protein-Induced Enterocolitis Syndrome". The Journal of Allergy and Clinical Immunology: In Practice. 8 (10): 3508–3514.e5. doi:10.1016/j.jaip.2020.06.011. PMC 7655634. PMID 32569756.
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