Lung disease in STAT3 hyper-IgE syndrome requires intense therapy

Allergy
Carolin KrönerEllen D Renner

Abstract

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. The course of pulmonary disease, radiological and histopathological interrelations, therapeutic management, and the outcome of 14 STAT3-HIES patients were assessed. 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...Continue Reading

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Citations

Sep 12, 2020·Orphanet Journal of Rare Diseases·Iris MeixnerEllen D Renner
Feb 7, 2021·The Journal of Allergy and Clinical Immunology. in Practice·Pia-Charlotte StadlerAndreas Wollenberg
Apr 21, 2021·The CRISPR Journal·Andreas C EberherrEllen D Renner
May 1, 2021·European Respiratory Review : an Official Journal of the European Respiratory Society·Sebastian F N BodeAndrea Heinzmann
May 2, 2021·Journal of Clinical Immunology·Christo TsilifisAndrew R Gennery
Mar 13, 2021·Mycopathologia·Pei Yee TiewSanjay H Chotirmall

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