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Abstract
Background
Pulmonary complications are responsible for high morbidity and mortality rates in patients with the rare immunodeficiency disorder
STAT
3 hyper‐IgE syndrome (
STAT
3‐
HIES
). The aim of this study was to expand knowledge about lung disease in
STAT
3‐
HIES
.
Methods
The course of pulmonary disease, radiological and histopathological interrelations, therapeutic management, and the outcome of 14
STAT
3‐
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
FEV
1 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
STAT
3‐
HIES
patients requires strict monitoring and intensive therapy.
Sprache
Englisch
Identifikatoren
ISSN: 0105-4538
eISSN: 1398-9995
DOI: 10.1111/all.13753
Titel-ID: cdi_crossref_primary_10_1111_all_13753
Format
–
Weiterführende Literatur
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