Hyper-IgE Syndromes and the Lung.

Abstract:

:Elevated serum IgE has many etiologies including parasitic infection, allergy and asthma, malignancy, and immune dysregulation. The hyper-IgE syndromes caused by mutations in STAT3, DOCK8, and PGM3 are monogenic primary immunodeficiencies associated with high IgE, eczema, and recurrent infections. These primary immunodeficiencies are associated with recurrent pneumonias leading to bronchiectasis; however, each has unique features and genetic diagnosis is essential in guiding therapy, discussing family planning, and defining prognosis. This article discusses the clinical features of these primary immunodeficiencies with a particular focus on the pulmonary manifestations and discussion of the genetics, pathogenesis, and approaches to therapy.

journal_name

Clin Chest Med

authors

Freeman AF,Olivier KN

doi

10.1016/j.ccm.2016.04.016

subject

Has Abstract

pub_date

2016-09-01 00:00:00

pages

557-67

issue

3

eissn

0272-5231

issn

1557-8216

pii

S0272-5231(16)30053-3

journal_volume

37

pub_type

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