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Interstitial lung diseases in inborn errors of immunity
Neal P Sullivan1, Nivethietha Maniam2, Paul J Maglione2
1Internal Medicine, Department of Medicine.
Current Opinion in Allergy and Clinical Immunology
|October 12, 2023
Summary
Inborn errors of immunity (IEI) frequently cause interstitial lung disease (ILD). This review explores IEI-ILD links, diagnostic approaches, and emerging treatments for better patient outcomes.
Area of Science:
- Pulmonology
- Immunology
- Genetics
Background:
- Interstitial lung disease (ILD) is a significant complication in patients with inborn errors of immunity (IEI).
- Understanding the interplay between IEI and ILD is crucial for effective management.
- A broad spectrum of IEI, including antibody deficiencies and immune dysregulation disorders, are associated with ILD development.
Conclusions:
- ILD is a common and expanding clinical challenge in IEI patients.
- Early identification and understanding of molecular mechanisms are key to managing ILD in IEI.
- Further research is needed to address knowledge gaps and optimize therapeutic interventions for IEI-associated ILD.
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