Abnormal T-cell phenotype in episodic ...
Document type :
Article dans une revue scientifique: Article original
PMID :
Permalink :
Title :
Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich''s syndrome): frequency, clinical implication and prognosis.
Author(s) :
Abisror, Noemie [Auteur]
Sorbonne Université [SU]
Mekinian, Arsene [Auteur]
Sorbonne Université [SU]
Dechartres, Agnes [Auteur]
Université de Versailles Saint-Quentin-en-Yvelines [UVSQ]
Groh, Matthieu [Auteur]
Université de Versailles Saint-Quentin-en-Yvelines [UVSQ]
Berezne, Alice [Auteur]
Noel, Nicolas [Auteur]
Université Paris-Sud - Paris 11 - Faculté de médecine [UP11 UFR Médecine]
Morati, Chafika [Auteur]
Haroche, Julien [Auteur]
Sorbonne Université [SU]
Hunault-Berger, Mathilde [Auteur]
LUNAM Université [Nantes Angers Le Mans]
Agard, Christian [Auteur]
Ackermann, Felix [Auteur]
Université de Versailles Saint-Quentin-en-Yvelines [UVSQ]
Geffray, Loik [Auteur]
Jeandel, Pierre-Yves [Auteur]
Trouillier, Sebastien [Auteur]
Quemeneur, Thomas [Auteur]
Dufour, Jean-Francois [Auteur]
Lamaury, Isabelle [Auteur]
Centre d'Investigation Clinique Antilles-Guyane [CIC - Antilles Guyane]
Lhote, Francois [Auteur]
Lefevre, Guillaume [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Fain, Olivier [Auteur]
Sorbonne Université [SU]
Kahn, Jean-Emmannuel [Auteur]
498252|||Lille Inflammation Research International Center - U 995 [LIRIC] (OLD)
Université de Versailles Saint-Quentin-en-Yvelines [UVSQ]
Sorbonne Université [SU]
Mekinian, Arsene [Auteur]
Sorbonne Université [SU]
Dechartres, Agnes [Auteur]
Université de Versailles Saint-Quentin-en-Yvelines [UVSQ]
Groh, Matthieu [Auteur]
Université de Versailles Saint-Quentin-en-Yvelines [UVSQ]
Berezne, Alice [Auteur]
Noel, Nicolas [Auteur]
Université Paris-Sud - Paris 11 - Faculté de médecine [UP11 UFR Médecine]
Morati, Chafika [Auteur]
Haroche, Julien [Auteur]
Sorbonne Université [SU]
Hunault-Berger, Mathilde [Auteur]
LUNAM Université [Nantes Angers Le Mans]
Agard, Christian [Auteur]
Ackermann, Felix [Auteur]
Université de Versailles Saint-Quentin-en-Yvelines [UVSQ]
Geffray, Loik [Auteur]
Jeandel, Pierre-Yves [Auteur]
Trouillier, Sebastien [Auteur]
Quemeneur, Thomas [Auteur]
Dufour, Jean-Francois [Auteur]
Lamaury, Isabelle [Auteur]
Centre d'Investigation Clinique Antilles-Guyane [CIC - Antilles Guyane]
Lhote, Francois [Auteur]
Lefevre, Guillaume [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Fain, Olivier [Auteur]
Sorbonne Université [SU]
Kahn, Jean-Emmannuel [Auteur]
498252|||Lille Inflammation Research International Center - U 995 [LIRIC] (OLD)
Université de Versailles Saint-Quentin-en-Yvelines [UVSQ]
Journal title :
Journal of The American Academy of Dermatology
Abbreviated title :
J. Am. Acad. Dermatol.
Volume number :
88
Pages :
e243-e250
Publication date :
2023-05
ISSN :
1097-6787
HAL domain(s) :
Sciences du Vivant [q-bio]
English abstract : [en]
BACKGROUND: Episodic Angioedema with eosinophilia (EAE, Gleich's syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia and frequent elevated serum Immunoglobin M.
METHODS: We ...
Show more >BACKGROUND: Episodic Angioedema with eosinophilia (EAE, Gleich's syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia and frequent elevated serum Immunoglobin M. METHODS: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France. RESULTS: Thirty patients were included with a median age at diagnosis of 41 years [5-84]. The median duration of each crisis was 5.5 days [1-90] with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%) among which 5 (17%) showed evidence of clonal TCR γ gene rearrangement. Median follow-up duration was 53 months [31-99]. The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio 4.15 [CI 95% 1.18-14.66; p=0.02). At last follow-up, 3 patients (10%) were able to withdraw all treatments and 11 (37%) were in clinical and biological remission with less than 10 mg of daily prednisone. CONCLUSIONS: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare.Show less >
Show more >BACKGROUND: Episodic Angioedema with eosinophilia (EAE, Gleich's syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia and frequent elevated serum Immunoglobin M. METHODS: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France. RESULTS: Thirty patients were included with a median age at diagnosis of 41 years [5-84]. The median duration of each crisis was 5.5 days [1-90] with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%) among which 5 (17%) showed evidence of clonal TCR γ gene rearrangement. Median follow-up duration was 53 months [31-99]. The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio 4.15 [CI 95% 1.18-14.66; p=0.02). At last follow-up, 3 patients (10%) were able to withdraw all treatments and 11 (37%) were in clinical and biological remission with less than 10 mg of daily prednisone. CONCLUSIONS: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare.Show less >
Language :
Anglais
Audience :
Internationale
Popular science :
Non
Administrative institution(s) :
CHU Lille
Inserm
Université de Lille
Inserm
Université de Lille
Submission date :
2019-10-22T08:09:10Z
2024-04-23T11:42:36Z
2024-04-23T11:42:36Z