Common Variable Immunodeficiency and Other Immunodeficiency Syndromes in Bronchiectasis.
1Section of Pulmonary and Critical Care Medicine, University of Texas Health Science Center at Tyler, Tyler, Texas.
Seminars in Respiratory and Critical Care Medicine
|July 14, 2021
Summary
Immunodeficiency, both primary and secondary, contributes to bronchiectasis development. Early recognition of specific immune deficiencies like CVID and XLA in bronchiectasis patients is crucial for improved care.
Area of Science:
- Pulmonology
- Immunology
- Genetics
Background:
- Immunodeficiency encompasses numerous diseases, significantly contributing to bronchiectasis.
- Both primary (genetic) and secondary (acquired) immunodeficiencies are implicated in bronchiectasis pathogenesis.
- Primary immune deficiencies are increasingly recognized as key factors in bronchiectasis development.
Purpose of the Study:
- To review primary and secondary immunodeficiencies associated with bronchiectasis.
- To highlight specific immune deficiencies linked to bronchiectasis.
- To emphasize the importance of recognizing immunodeficiency in idiopathic bronchiectasis.
Main Methods:
- Literature review of primary and secondary immunodeficiencies and their link to bronchiectasis.
- Discussion of specific primary immunodeficiencies: CVID, SAD, IgE deficiencies, DOCK8, PGM3, APDS, and XLA.
- Examination of secondary immunodeficiencies: GVHD, malignancy, and HIV.
Main Results:
- Several primary immune deficiencies, including CVID, SAD, IgE deficiencies, DOCK8, PGM3, APDS, and XLA, are closely associated with bronchiectasis.
- Secondary immunodeficiencies such as GVHD, malignancy, and HIV can also lead to bronchiectasis.
- Unique clinical presentations of these deficiencies necessitate vigilant recognition in bronchiectasis patients.
Conclusions:
- Improved recognition of specific immunodeficiencies in patients with idiopathic bronchiectasis is essential.
- Awareness of secondary immunodeficiencies is vital for optimizing patient care.
- Understanding the intricate relationship between bronchiectasis and immunodeficiency can elucidate underlying pathophysiology.
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