Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich syndrome): Frequency, clinical implication, and prognosis - 14/04/23
, Arsène Mekinian, MD, PhD b, Agnès Dechartres, MD, PhD c, Matthieu Groh, MD, MS c, Alice Berezne, MD, MS d, Nicolas Noel, MD, PhD e, Chafika Morati, MD d, Julien Haroche, MD, PhD f, Mathilde Hunault-Berger, MD, PhD g, Christian Agard, MD, PhD h, Felix Ackermann, MD, MS c, Loïk Geffray, MD i, Pierre-Yves Jeandel, MD, PhD j, Sébastien Trouillier, MD k, Thomas Quemeneur, MD l, Jean-François Dufour, MD m, Isabelle Lamaury, MD n, François Lhote, MD o, Guillaume Lefèvre, MD, PhD p, Olivier Fain, MD, PhD a, Jean Emmanuel Kahn, MD, PhD cfor the
French Eosinophil Network
Abstract |
Background |
Episodic angioedema with eosinophilia (EAE) (Gleich syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia, and frequent elevated serum IgM level.
Methods |
We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France.
Results |
A total of 30 patients with a median age at diagnosis of 41 years (range, 5-84) were included. The median duration of each crisis was 5.5 days (range, 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%), of whom 5 (17%) showed evidence of clonal T-cell receptor gamma locus gene (TRG) rearrangement. The median duration of follow-up was 53 months (range, 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; 95% confidence interval, 1.18-14.66; P = .02). At last follow-up, 3 patients (10%) were able to have all treatments withdrawn and 11 (37%) were in clinical and biologic remission with less than 10 mg of prednisone daily.
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.
Le texte complet de cet article est disponible en PDF.Key words : angioedema, hypereosinophilic syndrome, lymphoma (T-cell, peripheral), recurrence, treatment outcome
Abbreviations used : EAE, HES, HESL, IL, TCR
Plan
| Funding sources: None. |
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| Disclosure: Dr Groh, Dr Lefèvre, and Dr Kahn report consulting fees from GlaxoSmithKline, and Dr Kahn and Dr Groh report travel support and research grant from GlaxoSmithKline outside the submitted work. Dr Abisror, Dr Mekinian, Dr Dechartres, Dr Berezne, Dr Noel, Dr Morati, Dr Haroche, Dr Hunault-Berger, Dr Agard, Dr Ackermann, Dr Geffray, Dr Jeandel, Dr Trouillier, Dr Quemeneur, Dr Dufour, Dr Lamaury, Dr Lhote, and Dr Fain have no conflicts of interest to disclose. |
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| Presented at the 67th Congress of the Société Nationale Française de Médecine Interne in Marseille, France on June 7, 2013; the 10th International Congress on Autoimmunity in Leipzig, Germany, on April 7, 2016, and the 2018 Annual Congress of the European League Against Rheumatism in Amsterdam, The Netherlands, on June 13, 2018. |
Vol 88 - N° 5
P. e243-e250 - mai 2023 Retour au numéro
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