Journal Article

Interventional pulmonary procedures and their outcomes in patients with STAT3 hyper IgE syndrome

Rekabi, Mahsa, Sheikhy, Kambiz, Fallahi, Mazdak, Hashemi-Moghaddam, Seyedeh Atefeh, Ghadimi, Soodeh, Mahdaviani, Seyed Alireza, Pejhan, Saviz, Jamee, Mahnaz, Puel, Anne, Rezaei, Nima, Daneshmandi, Zahra, Saghebi, Seyed Reza, Chavoshzadeh, Zahra, Kakhki, Abolghasem Daneshvar

2023

Overview

Background STAT3 hyperimmunoglobulin E syndrome (STAT3-HIES) also referred to as autosomal dominant HIES (AD-HIES) is an inborn error of immunity characterized by the classic triad of eczema, frequent opportunistic infections, and elevated serum IgE levels. As a consequence of lung sequels due to repeated infections and impaired tissue healing, patients may require interventional pulmonary procedures. Method Four patients with dominant-negative STAT3 mutations who had received interventional pulmonary procedures were enrolled. The demographic, clinical, and molecular characteristics were gathered through a medical record search. All reported STAT3-HIES patients in the literature requiring pulmonary procedures as part of their treatment were reviewed. Result Recurrent episodes of pneumonia and lung abscess were the most prevalent symptoms. The most common non-immunological features were scoliosis, failure to thrive, and dental problems such as primary teeth retention and disseminated decays. Bronchiectasis, lung abscess, pneumatocele, and cavitary lesion were the most prevalent finding on high-resolution computed tomography at the earliest recording. All patients underwent pulmonary surgery and two of them experienced complications. Conclusion Patients with STAT3-HIES have marked pulmonary infection susceptibility which may necessitate thoracic surgeries. Since surgical procedures involve a high risk of complication, surgical options are recommended to be utilized only in cases of drug resistance or emergencies.

• Publisher: BioMed Central,BioMed Central Ltd,Springer Nature B.V,BMC
• Subject: Abscesses / Advertising executives / Age / Bacterial infections / Bacterial pneumonia / Bronchiectasis / Bronchopleural fistula / Care and treatment / Complications / Computed tomography / Dermatitis / Disease prevention / Disease susceptibility / Dominant-negative STAT3 mutation / Drug resistance / Eczema / Family medical history / Fever / Fistula / Hyper-IgE syndrome / Imipenem / Immunoglobulin E / Immunoglobulins / Immunology / Infections / Internal Medicine / Job's syndrome / Laboratories / Lung surgery / Lungs / Lymphocytes / Medical records / Medicine / Medicine & Public Health / Mutation / Ostomy / Parents & parenting / Patient outcomes / Patients / Pneumatocele / Pneumonia / Scoliosis / Skin diseases / Stat3 protein / Surgery / Teeth / Thoracic surgery / Thorax