Food protein induced enterocolitis syndrome (FPIES)

FOOD PROTEIN-INDUCED
ENTEROCOLITIS SYNDROME
(FPIES)
Prof. Dr. Saad S Al Ani
Senior Pediatric Consultant
Head of Pediatric Department
Khorfakkan Hospital
Sharjah ,UAE
aliraqialani@gmail.com

Food protein-induced
enterocolitis syndrome (FPIES)
 A non-immunoglobulin E (IgE)-mediated
gastrointestinal food hypersensitivity that
manifests as profuse, repetitive vomiting,
sometimes with diarrhea, leading to
dehydration and lethargy in the acute
setting, or weight loss and failure to thrive in
a chronic form(*)
*Muraro A, Werfel T, Hoffmann-Sommergruber K, et al. EAACI food allergy and anaphylaxis
guidelines: diagnosis and management of food allergy. Allergy 2014; 69:1008.
29/01/2019FPIESProf.Dr.SaadSAlAni
2

FPIES
This disease primarily affects infants.
It is most commonly caused by cow's milk
or soy protein, although other foods can
be triggers
3
Nowak-Węgrzyn A, Chehade M, Groetch ME, et al. 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 2017; 139:1111.

Immunologic reactions to
dietary proteins
May be classified as :
IgE mediated
Non-IgE mediated (T cell)
Mixed (IgE and T cell mediated)
4
https://www.uptodate.com/contents/food-protein-induced-enterocolitis-syndrome-fpies

dietary proteins (Cont.)
The entire gastrointestinal tract can be
affected, from the mouth to the rectum.
 Most of these disorders affect a specific
region of the gastrointestinal tract, such as:
 Eosinophilic esophagitis (EoE)
 Eosinophilic gastritis
 Food protein-induced enteropathy, enterocolitis,
proctitis
5
https://www.uptodate.com/contents/food-protein-induced-enterocolitis-syndrome-fpies

dietary proteins (Cont.)
FPIES has some features that overlap
with the:
 Other non-IgE-mediated gastrointestinal
allergic disorders
 Food protein-induced enteropathy
 Proctocolitis
6https://www.uptodate.com/contents/food-protein-induced-enterocolitis-syndrome-fpies

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FPIESProf.Dr.SaadSAlAni
http://www.aphysicianintraining.com/is-food-protein-induced-enterocolitis-syndrome-fpies-a-death-sentence/

DIFFERENCE OF FPIES FROM OTHER
COMMON FOOD ALLERGIES
It is usually a delayed reaction, and is
not caused by Immunoglobulin E (IgE)
antibodies.
Reactions only involve the gastrointestinal
system (no hives, welts or swelling are seen
on the face or body).
It is not associated with anaphylaxis.
Adrenaline autoinjectors are NOT used to
treat the reaction.
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https://www.allergy.org.au/patients/food-other-adverse-reactions/food-protein-induced-enterocolitis-syndrome-fpies

Difficulty to determine FPIES true
frequency in the general population.?
It is difficult to determine FPIES true
frequency in the general population due
to :
 The variable nature of the disorder
 The lack of recognition in the medical
community
 Frequent misdiagnosis
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https://rarediseases.org/rare-diseases/food-protein-induced-enterocolitis-syndrome/

EPIDEMIOLOGY
Non-immunoglobulin E (IgE)-mediated
and mixed gastrointestinal immune
reactions to cow's milk proteins are
estimated to account for up to 40 percent
of cow's milk protein hypersensitivity in
infants and young children
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NIAID-Sponsored Expert Panel, Boyce JA, Assa'ad A, et al. Guidelines for the diagnosis and management of food
allergy in the United States: report of the NIAID-sponsored expert panel. J Allergy Clin Immunol 2010; 126:S1.

EPIDEMIOLOGY (CONT.)
FPIES represents the severe end of the
spectrum of food protein-induced
gastrointestinal diseases in infants and is
far less common than proctocolitis
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Nowak-Wegrzyn A, Muraro A. Food protein-induced enterocolitis syndrome. Curr Opin
Allergy Clin Immunol 2009; 9:371.

EPIDEMIOLOGY(CONT.)
 There is a slight male predilection (52 to
60 percent) in FPIES
Reports of FPIES in siblings of an affected
child are rare
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Katz Y, Goldberg MR, Rajuan N, et al. The prevalence and natural course of food protein-induced
enterocolitis syndrome to cow's milk: a large-scale, prospective population-based study. J Allergy Clin
Immunol 2011; 127:647.

SIGNS & SYMPTOMS
The symptoms and severity of FPIES can vary
greatly from one individual to another.
 Some individuals will experience vomiting
and diarrhea that is not severe
Other individuals can develop severe, even
life-threatening complications due to profuse
vomiting, diarrhea and other symptoms.
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SIGNS & SYMPTOMS (CONT.)
Symptoms may be chronic
Onset is usually during the first year of life,
although the disorder can develop later
during childhood.
Specific rare cases due to fish or mollusks
have been identified older children and
adults.
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Vomiting and diarrhea, often profuse and
repetitive, are the two most common
symptoms associated with FPIES.
Vomiting usually occurs 1-4 hours after
ingesting the offending food.
Diarrhea usually occurs 3-6 hours after
ingestion.
Bloody diarrhea may occur in severe cases.
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Additional symptoms often occur including:
Pallor, lethargy, abdominal distension, and
cyanosis
Hypothermia and thrombocytosis , have also
been reported.
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 Failure to thrive in infants or children who
have multiple FPIES episodes
Most children outgrown FPIES by two or
three years of age, however, in some cases
the disorder persists.
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Approximately 30% of affected individuals
eventually develop an atopic disorder such
as:
Atopic dermatitis
Asthma
Allergic rhinitis
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CAUSES
The exact underlying cause of FPIES is
unknown.
The disorder occurs due to an improper
response of the immune system to
proteins found in specific foods.
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CAUSES (CONT.)
Eating the offending food causes localized
inflammation in the small and large
intestines, allows intestinal permeability
and fluid shift
The two most common foods are cow’s milk
and soy.(~ 40% both cow’s milk and soy)
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CAUSES (CONT.)
Rice is the most common solid food
associated with the disorder.
Wheat, chicken, turkey, fish, mollusks,
oats, barley, egg whites, vegetables,
peanuts, white potatoes, and sweet
potatoes have also been implicated.
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CAUSES (CONT.)
In recent years, children with FPIES due to
ingestion of fruit proteins have also been
noted
In approximately 70% of cases, individuals
react to one to two foods.
 FPIES is rarely reported in infants that are
exclusively breastfed,
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AFFECTED POPULATIONS
FPIES is an uncommon disorder that
affects males slightly more often than
females.
The incidence and prevalence is
unknown.
The number of FPIES cases has risen
in the last few decades.
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AFFECTED POPULATIONS (CONT.)
In studies in ‘Israel’ and Australia, they
estimated the incidence of 0.34%.
 FPIES most often affects infants or young
children.
 In extremely rare cases, FPIES has
developed in older children or adults as a
reaction to shellfish.
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RELATED DISORDERS
There are numerous conditions that can
mimic the symptoms of FPIES:
 Eosinophilic gastroenteritis
 Viral gastrointestinal illness
 Proctocolitis
 Food protein-induced enteropathy
 Sepsis
 Isomaltase- sucrase deficiency
 Various metabolic disorders
 Celiac disease
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REMEMBER
Classic food allergies can be distinguished
from FPIES by the presence of common,
additional symptoms including hives,
asthma, angioedema .
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DIAGNOSIS
FPIES is a clinical diagnosis based
upon the:
 exclusion of other causes
 identification of characteristic
symptoms
 thorough clinical evaluation
including a detailed patient history.
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DIAGNOSIS (CONT.)
The absence of symptoms commonly
associated with IgE-mediated food
allergies including skin reactions, asthma,
and angioedema may be indicative of
FPIES.
 Misdiagnosis and delays in diagnosis of
FPIES are common.
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ORAL FOOD CHALLENGE (OFC)
Oral food challenge (OFC) may be used to help
obtain a diagnosis of FPIES.
A procedure in which the suspected offending
food is gradually given to an affected child in
a controlled clinical environment.
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ORAL FOOD CHALLENGE (OFC) (CONT.)
For FPIES is a high risk procedure that
requires medical supervision and is
conducted following a specific protocol.
May be also used to determine whether
FPIES has resolved or persists as an
affected child ages.
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ATOPY PATCH TESTING (APT)
The studies show:
Poor results when using ATP as a predictor
of tolerance in children with FPIES.
The use of ATP remains controversial.
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TREATMENT
Removal of the offending food from the
diet of an affected individual
Some infants with FPIES may be treated by
being exclusively breastfed
Many older infants need additional foods
to meet caloric intake.
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TREATMENT
In infants who are on formula, a casein
hydroxylase-based formula or an
elemental-amino acid formula is
recommended
Severe episodes of FPIES require
intravenous fluids.
For acute FPIES reactions, ondansetron
intravenously for acute symptoms of
vomiting.is used
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PROGNOSIS
 Many children will grow out of FPIES
over time usually by 3 or 4 years of age.
Only 40-80% of those with FPIES to
rice, and 60% to dairy tolerated these
foods by three to four
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https://www.allergy.org.au/patients/food-other-adverse-reactions/food-protein-induced-enterocolitis-
syndrome-fpies

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Five Myths About FPIES

SUMMARY
Food Protein-Induced
Enterocolitis Syndrome
(FPIES) is a rare but serious
type of food allergy
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SUMMARY
Although there are
‘common trigger’ foods ,
any food can cause a
FRIES reaction
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SUMMARY
The classic pattern of an
FPIES reaction is when a
healthy infant or child
develops symptoms shortly
after eating a food.
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SUMMARY
There is a characteristic
delay of 2-3 hours before
onset of severe and repetitive
vomiting and eventually
diarrhea
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SUMMARY
A severe FIPES reaction can
include:
 Profound vomiting
 Pallor
 Lethargy
 Diarrhea
 Dehydration
 Shock
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SUMMARY
Signs of shock for an individual
with FPIES are severe and
include:
 Lethargy
 Pale/gray skin
 Drastic changes in body
temperature and heart rate
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SUMMARY
FPIES is usually a delayed
reaction, and is not caused
by Immunoglobulin E (IgE)
antibodies.
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SUMMARY
Reactions only involve the
gastrointestinal system (no
hives, welts or swelling are
seen on the face or body).
29/01/2019
43

SUMMARY
FPIES is not associated with
anaphylaxis
Adrenaline autoinjectors
are NOT used to treat the
reaction.
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SUMMARY
With proper medical
attention and a personalized
dietary plan to ensure proper
nutrition, children with
FPIES can grow and thrive.
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http://fpiesfoundation.org/about-fpies-3/

REFERENCES
 *Muraro A, Werfel T, Hoffmann-Sommergruber K, et al. EAACI food allergy and anaphylaxis
guidelines: diagnosis and management of food allergy. Allergy 2014; 69:1008
 Nowak-Węgrzyn A, Chehade M, Groetch ME, et al. 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 2017; 139:1111
 https://www.uptodate.com/contents/food-protein-induced-enterocolitis-syndrome-fpies
o NIAID-Sponsored Expert Panel, Boyce JA, Assa'ad A, et al. Guidelines for the diagnosis and
management of food allergy in the United States: report of the NIAID-sponsored expert panel. J
Allergy Clin Immunol 2010; 126:S1.
 https://rarediseases.org/rare-diseases/food-protein-induced-enterocolitis-syndrome
 http://www.aphysicianintraining.com/is-food-protein-induced-enterocolitis-syndrome-fpies-
a-death-sentence/
 https://www.allergy.org.au/patients/food-other-adverse-reactions/food-protein-induced-
enterocolitis-syndrome-fpies
 http://fpiesfoundation.org/about-fpies-3/
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Food protein induced enterocolitis syndrome (FPIES)

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