Journal of Clinical Immunology

, Volume 33, Issue 5, pp 896–902

Lung Parenchyma Surgery in Autosomal Dominant Hyper-IgE Syndrome

  • 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
Original Research

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 

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Copyright information

© Springer Science+Business Media New York (outside the USA) 2013

Authors and Affiliations

  • Alexandra F. Freeman
    • 1
  • Ellen D. Renner
    • 2
  • Carolyn Henderson
    • 1
  • Anne Langenbeck
    • 2
  • Kenneth N. Olivier
    • 1
  • Amy P. Hsu
    • 1
  • Beate Hagl
    • 2
  • Annette Boos
    • 2
  • Joie Davis
    • 1
  • Beatriz E. Marciano
    • 1
  • Lisa Boris
    • 1
  • Pamela Welch
    • 1
  • Julie Sawalle-Belohradsky
    • 2
  • Bernd H. Belohradsky
    • 2
  • King F. Kwong
    • 3
  • Steven M. Holland
    • 1
  1. 1.Laboratory of Clinical Infectious DiseasesNIAID, NIHBethesdaUSA
  2. 2.University Children’s Hospital at Dr. von Haunersches Kinderspital, Ludwig Maximilian UniversityMunichGermany
  3. 3.Section of Thoracic Oncology, Surgery BranchCCR, NCI, NIHBethesdaUSA

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