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Kröner, C.* ; Neumann, J.* ; Ley-Zaporozhan, J.* ; Hagl, B. ; Meixner, I.* ; Spielberger, B.D. ; Dückers, G.* ; Belohradsky, B.H.* ; Niehues, T.* ; Borte, M.* ; Rosenecker, J.* ; Kappler, M.* ; Nährig, S.* ; Reu, S.* ; Griese, M.* ; Renner, E.D.

Lung disease in STAT3 hyper-IgE syndrome requires intense therapy.

Allergy 74, 1691-1702 (2019)
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Background Pulmonary complications are responsible for high morbidity and mortality rates in patients with the rare immunodeficiency disorder STAT3 hyper-IgE syndrome (STAT3-HIES). The aim of this study was to expand knowledge about lung disease in STAT3-HIES. Methods The course of pulmonary disease, radiological and histopathological interrelations, therapeutic management, and the outcome of 14 STAT3-HIES patients were assessed. Results The patients' 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 years of age. Lung function revealed a mixed obstructive-restrictive impairment with reduced FEV1 and FVC in 75% of the patients. The severity of lung function impairment was associated with Aspergillus fumigatus infection and prior lung surgery. Severe lung tissue damage, with reduced numbers of ATP-binding cassette sub-family 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 years of age showed severe airway and parenchyma destruction. Lung surgeries frequently led to complications, including fistula formation. Long-term 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. Conclusions Due to its severity, pulmonary disease in STAT3-HIES patients requires strict monitoring and intensive therapy.
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Publication type Article: Journal article
Document type Scientific Article
Keywords Abca3 ; Bronchiectasis ; Lung Disease ; Pneumatocele ; Primary Immunodeficiency ; Stat3 Hyper-ige Syndrome; Pseudomonas-aeruginosa Infection; Signal Transducer; Key Findings; Mutations; Protein; Abca3; Transporter; Guidelines; Activator
ISSN (print) / ISBN 0105-4538
e-ISSN 1398-9995
Journal Allergy
Quellenangaben Volume: 74, Issue: 9, Pages: 1691-1702 Article Number: , Supplement: ,
Publisher Wiley
Publishing Place 111 River St, Hoboken 07030-5774, Nj Usa
Reviewing status Peer reviewed
Institute(s) Institute of Environmental Medicine (IEM)