Lung Parenchyma Surgery in Autosomal Dominant Hyper-IgE Syndrome
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Autosomal dominant hyper-IgE syndrome (AD-HIES) due to heterozygous STAT3 mutation is a primary immunodeficiency characterized by eczema, elevated serum IgE, recurrent infections, and connective tissue and skeletal findings. Healing of pneumonias is often abnormal with formation of pneumatoceles and bronchiectasis. We aimed to explore whether healing after lung surgery is also aberrant.
We retrospectively analyzed the medical records of 32 patients with AD-HIES who received lung surgery for the management of pulmonary infections from 1960 to 2011. We collected information including patient demographics, STAT3 mutation status, clinical history, surgical and medical procedures performed, complications, related medical treatments, and outcomes.
More than 50 % of lung surgeries had associated complications, with the majority being prolonged bronchopleural fistulae. These fistulae often led to empyemas that necessitated additional interventions including prolonged antibiotics, prolonged thoracostomy tube drainage and re-operations.
Lung surgery in AD-HIES patients is associated with high complication rates. STAT3 mutations likely lead to abnormalities in tissue remodelling that are further exacerbated by infection.
KeywordsHyper-IgE syndrome STAT3 Job’s syndrome lung surgery pulmonary complications IgE bronchopleural fistula
We thank all of the participants in this study, their families, and referring physicians who make our research possible. This research was supported by the Division of Intramural Research of the NIAID and the Center for Cancer Research of the National Cancer Institute, NIH, Bethesda, MD 20892. The views expressed in this article are those of the authors and do not reflect the official policy of the U.S. Government. This work was supported by the German Research Foundation (DFG RE2799/3-1), the Fritz-Thyssen research foundation grant (Az. 10.07.1.159) (to EDR), and Kindness for Kids (to J.S-B.).