Pulmonary surveillance in inborn errors of immunity: current challenges and emerging approaches
-
By
-
Ernest Hoptioncann
-
Rachel L. Eddy
-
Catherine M. Biggs
-
Jonathan H. Rayment
-
May 20, 2026
-
Clinical Scorecard: Monitoring Lung Health in Genetic Immune Disorders: Ongoing Challenges and New Strategies
At a Glance
| Category | Detail |
|---|
| Condition | Inborn errors of immunity (IEIs) |
| Key Mechanisms | Recurrent infections and immune dysregulation leading to pulmonary complications. |
| Target Population | Individuals with inborn errors of immunity (pwIEIs). |
| Care Setting | Clinical settings specializing in immunology and pulmonary health. |
Key Highlights
- Nearly 40% of pwIEIs develop pulmonary complications, primarily affecting the airways.
- Pulmonary complications account for 17.4% of deaths in pwIEIs.
- Emerging techniques like multiple-breath washout and pulmonary MRI offer radiation-free assessments.
Guideline-Based Recommendations
Diagnosis
- Utilize sensitive tools for detecting pulmonary manifestations in pwIEIs.
Management
- Implement strategies to monitor and manage recurrent infections and immune dysregulation.
Monitoring & Follow-up
- Adopt emerging techniques for longitudinal monitoring of lung health.
Risks
- Be aware of the high mortality associated with pulmonary complications in specific IEIs.
Patient & Prescribing Data
Individuals with genetic immune disorders, particularly those with recurrent respiratory infections.
Focus on managing infections and monitoring for chronic lung disease.
Clinical Best Practices
- Regular pulmonary function testing and imaging to assess lung health.
- Consider radiation-free imaging techniques for children and sensitive populations.
- Monitor for signs of chronic airway disease and interstitial lung disease.
Related Resources & Content
