Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich syndrome): Frequency, clinical implication, and prognosis

Abisror, Noémie; Mékinian, A.; Dechartres, Agnès; Groh, Matthieu; Bérezné, Alice; Noël, Nicolas; Morati, Chafika; Haroche, Julien; Hunault, Mathilde; Agard, C.; Ackermann, Félix; Geffray, L.; Jeandel, Pierre-Yves; Trouillier, S.; Quéméneur, T.; Dufour, Jean–François; Lamaury, I.; Lhote, F.; Lefèvre, Guillaume; Fain, Olivier; Kahn, Jean‐Emmanuel

doi:10.1016/j.jaad.2019.02.001

Cited by 12 publications

(22 citation statements)

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“…Episodic angioedema with eosinophilia was first described by Gleich et al in 1984 in four adult patients. Since then, less than one hundred cases have been reported and pediatric cases are scarce . Overall, the two cases reported here and a subsequent literature review supports the fact that the clinical picture of pediatric cases mimics that of adult patients, with predominantly weight gain, angioedema, urticaria, gastrointestinal, and joint involvements, with spontaneous remission and recurrence thereafter .…”

Section: Discussionsupporting

confidence: 75%

“…Since then, less than one hundred cases have been reported and pediatric cases are scarce . Overall, the two cases reported here and a subsequent literature review supports the fact that the clinical picture of pediatric cases mimics that of adult patients, with predominantly weight gain, angioedema, urticaria, gastrointestinal, and joint involvements, with spontaneous remission and recurrence thereafter . Fever (contrasting with mild biological inflammatory parameters) is likely to occur in a non‐negligible proportion of patients, but the severity of angioedema flares seems to be mild (neither life‐threatening features, eg, severe laryngeal involvement—nor intensive care unit admission were reported).…”

Section: Discussionsupporting

confidence: 70%

“…Fifteen children (10 males, median age 10 years [range 2.5‐17]) with Gleich syndrome living in Europe, Asia, or the United States were identified (Table ) . The median time between the first angioedema flare and the diagnosis of EAE was 5 years (data available for n = 11 patients).…”

Section: Resultsmentioning

confidence: 99%

“…According to the ICOG‐EO classification, EAE might represent a subgroup of HES, yet several cases of EAE occurring in patients with HES L have been reported . Here, we report on two cases of EAE occurring in children (including one HES L ‐associated EAE).…”

Section: Introductionmentioning

confidence: 83%

“…By definition, hypereosinophilia (HE) is constant, usually growing along attacks, but can disappear between flares. Total IgE and polyclonal IgM levels are frequently elevated, while underlying HES L , detected in 40% of adults, is associated with a shorter time to flare while on effective treatment …”

Section: Discussionmentioning

confidence: 99%

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Episodic angioedema with eosinophilia (Gleich syndrome) in children: A clinical review

Bertrand

Boccara

Filhon

et al. 2019

Pediatric Allergy Immunology

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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 (HESL). The aim of this study was to identify and describe pediatric cases. 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‐CD4+ T cells. First‐line therapy relied on oral corticosteroids in all patients, and further lines (used in five patients) included interferon‐α, methotrexate, and cyclosporin. Two children developed eosinophilic myocarditis during follow‐up. 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.

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Section: Discussionsupporting

confidence: 75%

Section: Discussionsupporting

confidence: 70%

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 83%

Section: Discussionmentioning

confidence: 99%

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