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

Research output: Contribution to journalArticlepeer-review

36 Scopus citations

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.

Original languageEnglish
Pages (from-to)896-902
Number of pages7
JournalJournal of Clinical Immunology
Volume33
Issue number5
DOIs
StatePublished - Jul 2013
Externally publishedYes

Keywords

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

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