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Updated: May 9, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Common variable immunodeficiency-associated granulomatous and interstitial lung disease.
Antje Prasse1, Gian Kayser, Klaus Warnatz
1Department of Pneumology, University Medical Clinic Freiburg, Freiburg, Germany. antje.prasse@uniklinik-freiburg.de
Current Opinion in Pulmonary Medicine
|July 25, 2013
Summary
Common variable immunodeficiency (CVID) can cause interstitial lung disease (ILD). While some ILD cases are stable, others require treatment, with prednisone showing a 50-66% response rate.
Area of Science:
- Immunology
- Pulmonology
- Rheumatology
Background:
- Common variable immunodeficiency (CVID) is a primary immunodeficiency marked by immunoglobulin deficiency.
- Approximately 30% of CVID patients develop autoimmune and granulomatous disease, often affecting the lungs.
- Interstitial lung disease (ILD) occurs in 5-15% of CVID patients, frequently at diagnosis.
Purpose of the Study:
- To review recent studies on interstitial lung disease (ILD) in common variable immunodeficiency (CVID).
- To discuss the current understanding of ILD in CVID, including its presentation, diagnosis, and management.
Main Methods:
- Review of recently published literature on ILD in CVID.
- Analysis of high-resolution computed tomography (HRCT) findings in three new articles.
- Evaluation of treatment responses reported in recent studies.
Main Results:
- ILD in CVID histologically presents as sarcoid-like granuloma, organizing pneumonia, lymphocytic interstitial pneumonitis, or nonspecific interstitial pneumonia, often co-occurring.
- Recent studies analyzed HRCT findings and treatment responses.
- Prednisone is a common first-line treatment with a 50-66% response rate.
Conclusions:
- ILD in CVID can be stable, potentially not requiring immunosuppressive therapy.
- For progressive disease with lung function decline, combined immunosuppressive treatment is recommended.
- Further research is needed to fully understand and manage ILD in CVID.
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