Service de Maladies Infectieuses et Tropicales, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Descartes, Paris, France.
Service de Pneumologie, Hôpital Foch, Suresnes, France; Faculté des Sciences de la Santé Simone Veil, Université Versailles-Saint-Quentin-en-Yvelines, Versailles, France; National Referral Center for Hypereosinophilic (CEREO).
J Allergy Clin Immunol Pract. 2019 Jul-Aug;7(6):1986-1995.e3. doi: 10.1016/j.jaip.2019.02.041. Epub 2019 Mar 13.
Autosomal-dominant signal transducer and activator of transcription 3 (STAT3) deficiency predisposes to recurrent bacterial pneumonia, complicated by bronchiectasis and cavitations. Aspergillosis is a major cause of morbidity in these patients. However, its diagnosis, classification, and treatment are challenging.
We aimed to assess the prevalence and describe the clinical, mycological, and radiological presentation and related therapy and outcome of Aspergillus infections of the respiratory tract in the STAT3-deficient patients of the National French cohort.
We performed a retrospective study of all pulmonary aspergillosis cases in STAT3-deficient patients (n = 74). Clinical and mycological data were collected up to October 2015 and imaging was centralized.
Twenty-one episodes of pulmonary aspergillosis in 13 (17.5%) STAT3-deficient patients were identified. The median age at first episode was 13 years (interquartile range, 10-26 years). Ninety percent of patients had previous bronchiectasis or cavitations. Infections were classified as follows: 5 single aspergilloma, 9 chronic cavity pulmonary aspergillosis, 5 allergic bronchopulmonary aspergillosis-like disease, and 2 mixed forms of concomitant allergic bronchopulmonary aspergillosis-like disease and chronic cavity pulmonary aspergillosis. No invasive aspergillosis cases were identified. Aspergillus species were isolated in 71% of episodes and anti-Aspergillus antibodies in 93%. Eleven episodes were breakthrough infections. Antifungal treatment was prolonged, with a median of 13 months, and 6 patients (7 episodes) required surgery, with a high rate of postsurgical complications. One patient died and 6 had a relapse.
Chronic and allergic forms of aspergillosis occurred in 17.5% of STAT3-deficient patients, mostly in lung cavities. Almost half had recurrences, despite prolonged antifungal treatment and/or surgery.
常染色体显性信号转导和转录激活因子 3（STAT3）缺陷易导致复发性细菌性肺炎，并伴有支气管扩张和空洞。曲霉菌病是这些患者发病的主要原因。然而，其诊断、分类和治疗具有挑战性。
我们旨在评估法国国家队列中 STAT3 缺陷患者呼吸道曲霉属感染的患病率，并描述其临床表现、真菌学、影像学表现以及相关治疗和结局。
我们对所有 STAT3 缺陷患者（n=74）的肺部曲霉菌病病例进行了回顾性研究。收集了截至 2015 年 10 月的临床和真菌学数据，并对影像学进行了集中分析。
在 13 名（17.5%）STAT3 缺陷患者中发现了 21 例肺部曲霉菌病。首次发病的中位年龄为 13 岁（四分位距，10-26 岁）。90%的患者既往有支气管扩张或空洞。感染分类如下：5 例单纯性曲霉菌瘤，9 例慢性空洞性肺曲霉病，5 例变应性支气管肺曲霉病样疾病，2 例混合性变应性支气管肺曲霉病样疾病合并慢性空洞性肺曲霉病。未发现侵袭性曲霉菌病。71%的病例中分离出曲霉属，93%的病例中检测到抗曲霉抗体。11 例为突破性感染。抗真菌治疗时间延长，中位数为 13 个月，6 例（7 例）患者需要手术，术后并发症发生率高。1 例患者死亡，6 例患者复发。
在 17.5%的 STAT3 缺陷患者中发生了慢性和变应性曲霉病，主要发生在肺部空洞中。尽管进行了延长的抗真菌治疗和/或手术，仍有近一半的患者复发。
