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Allergy. 2019 Feb 21. doi: 10.1111/all.13753. [Epub ahead of print]

Lung disease in STAT3-Hyper-IgE-Syndrome requires intense therapy.

Author information

1
Department of Pediatrics, Dr. von Hauner Children's Hospital, Ludwig Maximilian University, Munich, Germany.
2
Department of Pediatrics, Dr. von Hauner Children's Hospital, Ludwig Maximilian University, Munich, Germany and German Center for Lung research (DZL)Munich, Germany.
3
Institute of Pathology, Faculty of Medicine, Ludwig Maximilian, University, Munich, Germany.
4
Department of Radiology, Ludwig Maximilian University, Munich, Germany.
5
Environmental Medicine, UNIKA-T, Technical University of Munich and HelmholtzZentrum Munich, Munich/Augsburg, Germany.
6
HELIOS Children's Hospital, Krefeld, Germany.
7
Children's Hospital, St. Georg Klinikum, Leipzig, Germany.
8
Medizinische Klinik und Poliklinik, Ludwig Maximilian University, Munich, Germany.
9
Hochgebirgsklinik and Christine-Kühne-Center for Allergy Research and Education (CK-Care), Davos, Switzerland.

Abstract

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 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. This article is protected by copyright. All rights reserved.

KEYWORDS:

ABCA3; STAT3 hyper-IgE syndrome; STAT3-HIES; bronchiectasis; lung disease; pneumatocele; primary immunodeficiency

PMID:
30793327
DOI:
10.1111/all.13753

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