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The aim of this study was to expand knowledge about lung disease in <jats:styled-content style=\"fixed-case\">STAT<\/jats:styled-content>3\u2010<jats:styled-content style=\"fixed-case\">HIES<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The course of pulmonary disease, radiological and histopathological interrelations, therapeutic management, and the outcome of 14 <jats:styled-content style=\"fixed-case\">STAT<\/jats:styled-content>3\u2010<jats:styled-content style=\"fixed-case\">HIES<\/jats:styled-content> patients were assessed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The patients\u2019 quality of life was compromised most by pulmonary disease. All 14 patients showed first signs of lung disease at a median onset of 1.5\u00a0years of age. Lung function revealed a mixed obstructive\u2010restrictive impairment with reduced <jats:styled-content style=\"fixed-case\">FEV<\/jats:styled-content>1 and <jats:styled-content style=\"fixed-case\">FVC<\/jats:styled-content> in 75% of the patients. The severity of lung function impairment was associated with <jats:italic>Aspergillus fumigatus<\/jats:italic> infection and prior lung surgery. Severe lung tissue damage, with reduced numbers of ATP\u2010binding cassette sub\u2010family A member 3 (ABCA3) positive type II pneumocytes, was observed in the histological assessment of two deceased patients. Imaging studies of all patients above 6\u00a0years of age showed severe airway and parenchyma destruction. Lung surgeries frequently led to complications, including fistula formation. Long\u2010term antifungal and antibacterial treatment proved to be beneficial, as were inhalation therapy, chest physiotherapy, and exercise. Regular immunoglobulin replacement therapy tended to stabilize lung function.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Due to its severity, pulmonary disease in <jats:styled-content style=\"fixed-case\">STAT<\/jats:styled-content>3\u2010<jats:styled-content style=\"fixed-case\">HIES<\/jats:styled-content> patients requires strict monitoring and intensive therapy.<\/jats:p><\/jats:sec>","DOI":"10.1111\/all.13753","type":"journal-article","created":{"date-parts":[[2019,2,22]],"date-time":"2019-02-22T03:49:32Z","timestamp":1550807372000},"page":"1691-1702","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Lung disease in <scp>STAT<\/scp>3 hyper\u2010IgE syndrome requires intense therapy"],"prefix":"10.1111","volume":"74","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9474-3870","authenticated-orcid":false,"given":"Carolin","family":"Kr\u00f6ner","sequence":"first","affiliation":[{"name":"Department of Pediatrics Dr. von Hauner Children's Hospital Ludwig Maximilian University Munich Germany"},{"name":"German Center for Lung research (DZL) Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens","family":"Neumann","sequence":"additional","affiliation":[{"name":"Institute of Pathology Faculty of Medicine Ludwig Maximilian University Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Ley\u2010Zaporozhan","sequence":"additional","affiliation":[{"name":"Department of Radiology Ludwig Maximilian University Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beate","family":"Hagl","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Dr. von 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