Journal of Clinical Immunology

, Volume 33, Issue 5, pp 896–902

Lung Parenchyma Surgery in Autosomal Dominant Hyper-IgE Syndrome

Authors

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

DOI: 10.1007/s10875-013-9890-5

Cite this article as:
Freeman, A.F., Renner, E.D., Henderson, C. et al. J Clin Immunol (2013) 33: 896. doi:10.1007/s10875-013-9890-5

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 syndromeSTAT3Job’s syndromelung surgerypulmonary complicationsIgEbronchopleural fistula

Copyright information

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