Episodic angioedema with eosinophilia (Gleich syndrome) in children: A clinical review.
Adolescent
Adrenal Cortex Hormones
/ therapeutic use
Angioedema
/ complications
Child
Child, Preschool
Eosinophilia
/ complications
Eosinophils
/ immunology
Female
Humans
Hypereosinophilic Syndrome
/ complications
Immunoglobulin M
/ blood
Immunophenotyping
Male
Myocarditis
/ complications
Recurrence
Retrospective Studies
T-Lymphocytes
/ immunology
Treatment Outcome
Urticaria
/ complications
Gleich syndrome
children
episodic angioedema with eosinophilia
Journal
Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology
ISSN: 1399-3038
Titre abrégé: Pediatr Allergy Immunol
Pays: England
ID NLM: 9106718
Informations de publication
Date de publication:
04 2020
04 2020
Historique:
received:
28
07
2019
revised:
20
09
2019
accepted:
07
11
2019
pubmed:
15
11
2019
medline:
5
3
2021
entrez:
15
11
2019
Statut:
ppublish
Résumé
Episodic angioedema with eosinophilia (EAE, Gleich syndrome) is a rare disease, consisting of recurrent angioedema with hypereosinophilia and frequent increased serum immunoglobulin M levels. Less than 100 patients have been reported, mainly adults, sometimes with underlying lymphocytic variant of hypereosinophilic syndrome (HES We performed a retrospective study of all pediatric cases of EAE referred within the French National Referral Center for Hypereosinophilic Syndrome (CEREO). Next, the PRISMA guidelines were applied in order to perform a systematic review (data sources: PubMed, Web of Science). Among the two reported and 15 previously published cases of EAE occurring in children, the main clinical findings mimicked those of adults, including recurrent angioedema, hives, and weight gain. The median time between the first angioedema flare and the diagnosis of EAE was 5 years in published cases. Hypereosinophilia was constant, usually worsening with each attack, but seldom disappeared between flares. Total IgM serum levels were elevated in 16 patients. Four children had evidence of abnormal CD3 Pediatricians should be aware that EAE is a diagnosis to consider in children. T-cell immunophenotyping is warranted in this setting. Prognosis seems fair, yet eosinophil-related organ damage may occur in patients with persistent eosinophilia.
Sections du résumé
BACKGROUND
Episodic angioedema with eosinophilia (EAE, Gleich syndrome) is a rare disease, consisting of recurrent angioedema with hypereosinophilia and frequent increased serum immunoglobulin M levels. Less than 100 patients have been reported, mainly adults, sometimes with underlying lymphocytic variant of hypereosinophilic syndrome (HES
METHODS
We performed a retrospective study of all pediatric cases of EAE referred within the French National Referral Center for Hypereosinophilic Syndrome (CEREO). Next, the PRISMA guidelines were applied in order to perform a systematic review (data sources: PubMed, Web of Science).
RESULTS
Among the two reported and 15 previously published cases of EAE occurring in children, the main clinical findings mimicked those of adults, including recurrent angioedema, hives, and weight gain. The median time between the first angioedema flare and the diagnosis of EAE was 5 years in published cases. Hypereosinophilia was constant, usually worsening with each attack, but seldom disappeared between flares. Total IgM serum levels were elevated in 16 patients. Four children had evidence of abnormal CD3
CONCLUSION
Pediatricians should be aware that EAE is a diagnosis to consider in children. T-cell immunophenotyping is warranted in this setting. Prognosis seems fair, yet eosinophil-related organ damage may occur in patients with persistent eosinophilia.
Substances chimiques
Adrenal Cortex Hormones
0
Immunoglobulin M
0
Types de publication
Case Reports
Journal Article
Systematic Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
297-302Commentaires et corrections
Type : CommentIn
Type : CommentIn
Informations de copyright
© 2019 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.
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