Spectrum of Pulmonary Aspergillosis in Hyper-IgE Syndrome with Autosomal-Dominant STAT3 Deficiency Article - Août 2019

Amélie Duréault, Colas Tcherakian, Sylvain Poiree, Emilie Catherinot, François Danion, Grégory Jouvion, Marie Elisabeth Bougnoux, Nizar Mahlaoui, Claire Givel, Martin Castelle, Capucine Picard, Marie Olivia Chansdesris, Olivier Lortholary, Fanny Lanternier, H. Coignard, K. Amazzough, F. Suarez, S. Blanche, B. Sendid, M. Cornu, J.F. Bervar, A. Deschildre, L. Wemeau, C. Fieschi, A. Alanio, C. Menetrey, A. Senechal, F. Ader, P. Tattevin, C. Pison, L. Grandiere-Perez, D. Garcia-Hermoso, F. Botterel-Chartier

Amélie Duréault, Colas Tcherakian, Sylvain Poiree, Emilie Catherinot, François Danion, Grégory Jouvion, Marie Elisabeth Bougnoux, Nizar Mahlaoui, Claire Givel, Martin Castelle, Capucine Picard, Marie Olivia Chansdesris, Olivier Lortholary, Fanny Lanternier, H. Coignard, K. Amazzough, F. Suarez, S. Blanche, B. Sendid, M. Cornu, J.F. Bervar, A. Deschildre, L. Wemeau, C. Fieschi, A. Alanio, C. Menetrey, A. Senechal, F. Ader, P. Tattevin, C. Pison, L. Grandiere-Perez, D. Garcia-Hermoso, F. Botterel-Chartier, « Spectrum of Pulmonary Aspergillosis in Hyper-IgE Syndrome with Autosomal-Dominant STAT3 Deficiency  », Journal of Allergy and Clinical Immunology : In Practice, août 2019, 1986 - 1995.e3. ISSN 2213-2198

Abstract

Background : 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. Objective : 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. Methods : 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. Results : 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. Conclusions : 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.

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