Conoscenza della Food Protein-Induced
Enterocolitis Syndrome (FPIES)
tra i pediatri di famiglia
Pasquale Comberiati
Clinica Pediatrica
Università di Verona
A multicentre retrospective study of 66 Italian children
with FPIES: different management for different
phenotypes Sopo MS, CEA 2012; 42:1257
No. of FPIES diagnosis at the 3 centers
 Retrospective study
(2004-2010): Rome,
Florence, Benevento
18–
16 –
17
14 –
 66 children, 165 episodes
 35 (53%) OFC diagnosed vs
31 (47%) clinically diagnosed
P < 0.001
12 –
13
10 –
8–
6–
 Tolerance OFC 1-year after
last reaction
15
4–
4
4
2004
2005
6
6
2006
2007
2–
0
0000
2008
2009
2010
The prevalence and natural course of FPIES to cow’s
milk: A large-scale, prospective population based
study.
Katz Y, JACI 2011; 127:647
Incidence of
CM-FPIES vs IgE-CMA
 Cohort of 13,019 infant
(2004-2006)
0.6 –
 28 (64%) OFC vs 16 (36%)
clinically diagnosed
 CM-FPIES Incidence
0.5%
0.4 –
%
 44 with CM-FPIES and
66 with IgE-CMA
0.2 –
0
0.34%
FPIES
(n= 44)
IgE-CMA
(n= 66)
FPIES: The diagnosis is often delayed
• Lack of suspition
• No specific diagnostic tests
• Non-specific presenting symptoms
Fiocchi A, Curr Opin Allergy Clin Immunol 2014, 14:246–254
Differential diagnosis of food protein-induced
enterocolitis syndrome (FPIES)
Fiocchi A, Curr Opin All Clin Immunol 2014; 14:246
SEPSIS
METABOLIC
DISORDES
GASTROENTERITIS
FPIES
SURGICAL
ABDOMINAL
CONDITION
PRIMARY
IMMUNODEFICIENCY
ANAPHYLAXIS
Food Protein-Induced Enterocolitis Syndrome
(FPIES): 16-Year Experience
Mehr S, Pediatrics 2009; 123:e459
Episodes of Acute FPIES
before diagnosis
 Retrospective study
(1992-2007)
40 –
 35 patients, with 66 acute
FPIES
30 –
%
29%
 Causative foods, clinical
features, treatments,
and outcomes
26%
20 –
26%
20%
10 –
0
1
Episodes
2
3
Episodes Episodes
4
Episodes
Food Protein-Induced Enterocolitis Syndrome
(FPIES): 16-Year Experience
Mehr S, Pediatrics 2009; 123:e459
Additional investigations of
FPIES episodes
 Retrospective study
(1992-2007)
 Causative foods, clinical
features, treatments,
and outcomes
30 –
34%
28%
%
 35 patients, with 66 acute
FPIES
40 –
20 –
22%
10 –
2%
0
Abdominal
Imaging
Septic
evaluation
Surgical Laparotomy
consultat
OBIETTIVI E METODI
• Indagine conoscitiva «FPIES»
• Questionario «on-line» anonimo
• Google-drive platform
• 16 domande risposta multipla
• 194 Pediatri di famiglia
• Verona (70%) e Torino (30%)
Clinica Pediatrica
Università di Verona
Torino
RISULTATI
Conosco sintomi,
diagnosi e terapia
12%
Non ho familiarità
diagnosi e terapia
49%
Sentito solo parlare
31%
Mai sentito parlare
8%
0
Clinica Pediatrica
Università di Verona
10
20
%
30
40
50
RISULTATI – Alimenti trigger
34
Latte Vaccino
5
Latte di Soia
20% ha in cura
b.ni con FPIES
14
Pesce
6
Riso
Altri cibi solidi
(Uovo, Cereali,
Pollo, Tacchino,
Frutta o Verdura)
Clinica Pediatrica
Università di Verona
24
0
10
20
30
40
50
Prevalence of trigger foods in FPIES
Ruffner
Caubet
Mehr
Sopo
(n. 462)
(n. 160)
(n. 35)
(n. 66)
Cow’s Milk
67%
44%
20%
67%
Soy
41%
41%
34%
4%
Rice
Oats
19%
16%
22%
16%
40%
6%
4%
-
Fish
1%
10%
3%
12%
Poultry
10%
5%
3%
3%
Egg
11%
3%
-
6%
Banana
3.5%
-
3%
-
Caubet JC, JACI 2014; 134: 382-9
Ruffner MA. JACI:In Practice 2013; 1: 343-9
Mehr S. Pediatrics 2009; 123:e459.
Sopo MS. CEA 2012;42:1257-65.
RISULTATI – Chi ha fatto Diagnosi
49%
Allergologo
Gastroenterologo
8%
Pediatra Ospedale/
Pronto Soccorso
5%
38%
Me Medesimo
0
10
20
30
%
Clinica Pediatrica
Università di Verona
40
50
RISULTATI – Casi FPIES
FPIES ACUTA
FPIES CRONICA
FPIES CRONICA
5 mesi, 2 episodi
di vomito profuso,
pallore e
disidratazione
comparsi dopo
alcune h
assunzione crema
di riso.
2 mesi, LF dalla
nascita, scarsa
crescita
ponderale,
diarrea cronica
con muco
± sangue nelle
feci,
± leucocitosi.
3 mesi, LF dalla
nascita, scarsa
crescita
ponderale,
diarrea cronica
con muco
± sangue nelle
feci, persistenza
sintomi anche
dopo inizio eHCF.
54%
47%
23%
13%
Clinica Pediatrica
Università di Verona
RISULTATI – Test Diagnostici 1
DIAGNOSI FPIES : Prick Test e/o
sIgE devono essere negativi?
70 –
60 –
50 –
40 –
56%
30 –
44%
20 –
Caubet JC, JACI 2014; 134: 382-9
10 –
00
Among 160 subjects with
FPIES, 24% had positive
specific IgE to the
FPIES food,
which is referred to as
‘‘atypical’’ FPIES
SI
Clinica Pediatrica
Università di Verona
NO
RISULTATI – Test Diagnostici 2
DIAGNOSI FPIES: sempre
necessario il TPO?
70 –
60 –
50 –
57%
40 –
43%
30 –
20 –
10 –
00
SI
NO
FPIES: Diagnosis
• There are no in vivo or in vitro
tests with demonstrated
specificity and sensitivity
to diagnose FPIES
• OFC may not be necessary to
make the diagnosis if
the history of FPIES is very
compelling (2 or more
reactions with typical symptoms
to the same food in a 6-month
period and elimination of
symptoms when the causative
food is removed from the diet)
Boyce J, J Allergy Clin Immunol 2010;126:S1-S58
Clinica Pediatrica
Università di Verona
RISULTATI – Terapia 1
TERAPIA FPIES «ACUTA» :
Adrenalina cardine?
70 –
74%
60 –
50 –
40 –
30 –
20 –
26%
10 –
00
SI
Clinica Pediatrica
Università di Verona
NO
FPIES: Acute
Managment
• Oral rehydration fluids (if mild)
• Intravenous fluids, 20 ml/kg
boluses of isotonic saline
(if moderate to severe)
• Metilprednisolone ev, 1mg/kg
(max 60-80mg)
(if moderate to severe)
• Epinephrin for hypotension for
severe or unresponsive to fluids
Järvinen KM, JACI in Pract; 2013; 1:317-22
Natural history of food protein-induced enterocolitis
syndrome (FPIES) Katz Y, Curr Opin All Clin Immunol 2014; 14:229
Cumulative probability of recovery
from CMP-induced FPIES
 Around 90% of FPIES patients
recover by the age 3–5 years.
 Recovery from FPIES to solid
foods occurs later than FPIES
to Cow’s Milk or Soy.
RISULTATI – Terapia 2
A quanti mesi dall’ultima FPIES
«acuta» consiglieresti la
Reintroduzione dell’alimento?
70 –
60 –
60%
50 –
40 –
30 –
20 –
10 –
17%
15%
6-11
mesi
12-18
mesi
00
Clinica Pediatrica
Università di Verona
8%
18-24
mesi
Allergologo
FPIES: Long term
Managment
• Around 90% of FPIES patients
recover by the age 3–5 years.
• Oral Food Challenge is
warranted to determine
when FPIES has resolved
• 1st Tolerance-OFC with cow’s
milk at 18-20 months of age.
• For other foods reports are
insufficient, and should be
considered for reintroduction to
the diet 12–18 months after the
last reaction, in a physiciansupervised setting
Järvinen KM, JACI in Pract; 2013; 1:317-22
CONCLUSIONE
È necessaria una maggiore informazione
riguardo all’FPIES, in merito alle diverse
forme cliniche ed alla gestione terapeutica
acuta ed a lungo termine.
Clinica Pediatrica
Università di Verona