Details

Autor(en) / Beteiligte

• Kröner, Carolin
• Neumann, Jens
• Ley‐Zaporozhan, Julia
• Hagl, Beate
• Meixner, Iris
• Spielberger, Benedikt D.
• Dückers, Gregor
• Belohradsky, Bernd H.
• Niehues, Tim
• Borte, Michael
• Rosenecker, Joseph
• Kappler, Matthias
• Nährig, Susanne
• Reu, Simone
• Griese, Matthias
• Renner, Ellen D.

Titel

Lung disease in STAT 3 hyper‐IgE syndrome requires intense therapy

Ist Teil von

• Allergy (Copenhagen), 2019-09, Vol.74 (9), p.1691-1702

Erscheinungsjahr

2019

Quelle

Wiley Online Library

Beschreibungen/Notizen

• 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.