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Lung Parenchyma Surgery in Autosomal Dominant Hyper-IgE Syndrome |
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| Authors: | Alexandra F. Freeman Ellen D. Renner Carolyn Henderson Anne Langenbeck Kenneth N. Olivier Amy P. Hsu Beate Hagl Annette Boos Joie Davis Beatriz E. Marciano Lisa Boris Pamela Welch Julie Sawalle-Belohradsky Bernd H. Belohradsky King F. Kwong Steven M. Holland |
| Affiliation: | 1. Laboratory of Clinical Infectious Diseases, NIAID, NIH, NIH Building 10 Room 11 N234, 9000 Rockville Pike, Bethesda, MD, 20892, USA 2. University Children’s Hospital at Dr. von Haunersches Kinderspital, Ludwig Maximilian University, Munich, Germany 3. Section of Thoracic Oncology, Surgery Branch, CCR, NCI, NIH, Bethesda, MD, USA |
| Abstract: | PurposeAutosomal 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.MethodsWe 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.ResultsMore 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.ConclusionLung 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. |
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