Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich syndrome): Frequency, clinical implication, and prognosis,Journal of the American Academy of Dermatology

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Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich syndrome): Frequency, clinical implication, and prognosis
Journal of the American Academy of Dermatology ( IF 12.8 ) Pub Date : 2019-02-07 , DOI: 10.1016/j.jaad.2019.02.001
Noémie Abisror ₁ , Arsène Mekinian ₂ , Agnès Dechartres ₃ , Matthieu Groh ₃ , Alice Berezne 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 , François Lhote ₁₅ , Guillaume Lefèvre ₁₆ , Olivier Fain ₁ , Jean Emmanuel Kahn ₃ ,

Affiliation

AP-HP, Hôpital Saint Antoine, Service de Médecine Interne, Département Hospitalo-Universitaire Inflammation-Immunopathologie-Biotherapie (DHU i2B) (DHU i2B), Faculté de Médecine Sorbonne Université, Paris, France.
Sorbonne Université, Inserm U1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique, Département Biostatistique, Santé Publique, Information Médicale-Hôpital Pitié Salpêtrière, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France.
Service de Médecine Interne, Université Versailles Saint-Quentin-en-Yvelines, Centre de Référence des Syndromes Hyperéosinophiliques (CEREO), Hôpital Foch, Suresnes, France.
Service de Médecine Interne, CH Annecy Genevois, Annecy, France.
Department of Internal Medicine, Assistance-Publique Hôpitaux de Paris Paris XI University, Hôpital Bicêtre Le Kremlin-Bicêtre, France.
Department of Internal Medicine, French Reference Centre for Rare Autoimmune Systemic Diseases, e3m Institute, Assistance-Publique Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Paris, and Paris VI University (UPMC), Sorbonne Universités, Paris, France.
Maladies du Sang, CHU Angers, CRCINA, INSERM, Université de Nantes, Université d'Angers, Angers, France.
Service de Médecine Interne, CHU Hôtel-Dieu, Nantes, France.
Service de médecine interne, Centre Hospitalier, Lisieux, France.
Service de Médecine Interne, Centre Hospitalier Universitaire de Nice, Nice, France.
Service de Médecine Interne, Centre Hospitalier d'Aurillac, Aurillac, France.
Département de Néphrologie et Département de Médecine Interne, Centre Hospitalier de Valenciennes, Valenciennes, France.
Service de Médecine Interne et Post-Urgence, Centre Hospitalier de Bourg-en-Bresse, Bourg-en-Bresse, France.
Inserm-CIC 1424 et Service de Maladies Infectieuses et Tropicales, Centre Hospitalier Universitaire de Pointe-à-Pitre, Pointe-à-Pitre, France.
Service de Médecine Interne, Hôpital de Saint-Denis, Saint-Denis, France.
U995, LIRIC-Lille Inflammation Research International Center; INSERM, U995; Département de Médecine Interne et Immunologie Clinique, Centre National de Référence Maladies Systémiques et Auto-immunes Rares (Sclérodermie Systémique), Centre de Référence des Syndromes Hyperéosinophiliques (CEREO); and Institut d'Immunologie, CHU Lille, Université de Lille, Lille, France.

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.

中文翻译：

发作性血管性水肿伴嗜酸性粒细胞增多症（Gleich 综合征）的异常 T 细胞表型：频率、临床意义和预后

背景

发作性血管性水肿伴嗜酸性粒细胞增多症 (EAE)（Gleich 综合征）是一种罕见的疾病，由反复发作的血管性水肿、嗜酸性粒细胞增多症和频繁升高的血清 IgM 水平组成。

方法

我们对法国 EAE 患者的临床谱和治疗管理进行了一项回顾性多中心全国性研究。

结果

总共包括 30 名诊断时中位年龄为 41 岁（范围 5-84 岁）的患者。每次危象的中位持续时间为 5.5 天（范围 1-90 天），肿胀主要影响面部和上肢。20 名患者 (67%) 的总血清 IgM 水平升高。在 12 名患者 (40%) 中检测到异常的 T 细胞免疫表型，其中 5 名 (17%) 显示克隆性 T 细胞受体伽马位点基因 (TRG )重排的证据。中位随访时间为 53 个月（范围 31-99）。异常 T 细胞群的存在是与较短发作时间相关的唯一因素（风险比，4.15；95% 置信区间，1.18-14.66；P =.02）。在最后一次随访时，3 名患者 (10%) 能够撤消所有治疗，11 名 (37%) 患者在临床和生物学缓解中每天使用少于 10 mg 的泼尼松。