Lung Transplantation for Bronchiectasis Due to Hyperimmunoglobulin E Syndrome.

Abstract

Hyperimmunoglobulin E syndrome (HIES) is one of the primary immunodeficiencies characterized by recurrent staphylococcal skin and lung infections that result in lung destruction and critically diminished pulmonary function. Despite the lack of definitive treatment, there have been no reports of successful lung transplantation for HIES patients. We report the case of a 42-year-old woman with HIES with progressive bronchiectasis whose pulmonary infection was controlled before transplantation, and subsequent lung transplantation was uneventful. Lung transplantation may be feasible in HIES if the patient is immunologically stable preoperatively and perioperative infections, especially Aspergillus infections, are well controlled.