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Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich’s syndrome): frequency, clinical implication and prognosis

Noémie Abisror 1 Arsène Mekinian 1 Agnès Dechartres 2 Matthieu Groh 3 Alice Bérezné 4 Nicolas Noel 5 Chafika Morati 4 Julien Haroche 6 Mathilde Hunault-Berger 7 Christian Agard 8 Felix Ackermann 3 Loïk Geffray 9 Pierre-Yves Jeandel 10 Sébastien Trouillier 11 Thomas Quemeneur 12 Jean-François Dufour 13 Isabelle Lamaury 14, 15 François Lhote 16 Guillaume Lefèvre 17 Olivier Fain 18 Jean Emmanuel Kahn 3
1 DHU i2B - Service de rhumatologie, inflammation-immunopathologie- biothérapie [CHU Saint-Antoine]
2 Service de Biostatistique Santé Publique et Information Médicale [CHU Pitié-Salpêtrière]
3 Hôpital Foch [Suresnes]
4 Hôpital Cochin [AP-HP]
5 UNIROUEN UFR Santé - UNIROUEN - UFR Santé
6 Centre National de Référence du Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et Maladies Auto-immunes Systémiques Rares[CHU Pitié Salpêtrière]
7 CRCINA-ÉQUIPE 7 - Innate Immunity and Immunotherapy
CRCINA - Centre de Recherche en Cancérologie et Immunologie Nantes-Angers
8 Service de médecine interne [Nantes]
9 CH Robert Bisson - Centre Hospitalier Robert Bisson
10 Service de médecine interne [Nice]
11 CHU Clermont-Ferrand
12 VALENCIENNES - Med Int - Département de Médecine Interne
13 Centre Hospitalier de Bourg-en-Bresse
14 CHU Pointe-à-Pitre/Abymes [Guadeloupe]
15 CIC - Antilles Guyane - Centre d'Investigation Clinique Antilles-Guyane
16 Centre Hospitalier de Saint-Denis [Ile-de-France]
17 LIRIC - Lille Inflammation Research International Center - U 995
18 Service de Médecine Interne [CHU Saint-Antoine]
Abstract : 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. Conclusion: 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.
Keywords : Angioedema Hypereosinophilic syndrome Lymphoma (T-cell Peripheral) Recurrence Treatment Outcome.
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INSERM | CRCINA-3I | RIIP | UNIV-AG | CRCINA | UNICE | UNIV-COTEDAZUR | UNIV-NANTES | UNIROUEN | UNIV-LILLE | UNIV-ANGERS | CNRS | COMUE-NORMANDIE | SORBONNE-UNIVERSITE | SU-TI

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Noémie Abisror, Arsène Mekinian, Agnès Dechartres, Matthieu Groh, Alice Bérezné, et al.. Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich’s syndrome): frequency, clinical implication and prognosis. Journal of The American Academy of Dermatology, Elsevier, 2019, pp.S0190-9622(19)30196-3. ⟨10.1016/j.jaad.2019.02.001⟩. ⟨inserm-02909159⟩

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