Original Research

Journal of Clinical Immunology

, Volume 33, Issue 5, pp 896-902

First online:

Lung Parenchyma Surgery in Autosomal Dominant Hyper-IgE Syndrome

  • Alexandra F. FreemanAffiliated withLaboratory of Clinical Infectious Diseases, NIAID, NIH Email author 
  • , Ellen D. RennerAffiliated withUniversity Children’s Hospital at Dr. von Haunersches Kinderspital, Ludwig Maximilian University
  • , Carolyn HendersonAffiliated withLaboratory of Clinical Infectious Diseases, NIAID, NIH
  • , Anne LangenbeckAffiliated withUniversity Children’s Hospital at Dr. von Haunersches Kinderspital, Ludwig Maximilian University
  • , Kenneth N. OlivierAffiliated withLaboratory of Clinical Infectious Diseases, NIAID, NIH
  • , Amy P. HsuAffiliated withLaboratory of Clinical Infectious Diseases, NIAID, NIH
  • , Beate HaglAffiliated withUniversity Children’s Hospital at Dr. von Haunersches Kinderspital, Ludwig Maximilian University
  • , Annette BoosAffiliated withUniversity Children’s Hospital at Dr. von Haunersches Kinderspital, Ludwig Maximilian University
  • , Joie DavisAffiliated withLaboratory of Clinical Infectious Diseases, NIAID, NIH
    • , Beatriz E. MarcianoAffiliated withLaboratory of Clinical Infectious Diseases, NIAID, NIH
    • , Lisa BorisAffiliated withLaboratory of Clinical Infectious Diseases, NIAID, NIH
    • , Pamela WelchAffiliated withLaboratory of Clinical Infectious Diseases, NIAID, NIH
    • , Julie Sawalle-BelohradskyAffiliated withUniversity Children’s Hospital at Dr. von Haunersches Kinderspital, Ludwig Maximilian University
    • , Bernd H. BelohradskyAffiliated withUniversity Children’s Hospital at Dr. von Haunersches Kinderspital, Ludwig Maximilian University
    • , King F. KwongAffiliated withSection of Thoracic Oncology, Surgery Branch, CCR, NCI, NIH
    • , Steven M. HollandAffiliated withLaboratory of Clinical Infectious Diseases, NIAID, NIH

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Abstract

Purpose

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.

Methods

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.

Results

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.

Conclusion

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.

Keywords

Hyper-IgE syndrome STAT3 Job’s syndrome lung surgery pulmonary complications IgE bronchopleural fistula