Granulomatosis-associated common variable immunodeficiency disorder: a case-control study versus sarcoidosis
Diane Bouvry1, Luc Mouthon, Pierre-Yves Brillet
1Assistance Publique – Hôpitaux de Paris, Service de Pneumologie, and Université Paris 13, PRES Sorbonne-Paris-Cité, EA 2363, Hôpital Universitaire Avicenne, Bobigny, France.
The European Respiratory Journal
|August 21, 2012
Summary
Interstitial lung disease in common variable immunodeficiency disorder-associated granulomatous disease presents unique symptoms and imaging findings, differing significantly from pulmonary sarcoidosis. This condition has a higher mortality rate due to CVID complications.
Area of Science:
- Pulmonology
- Immunology
- Radiology
Background:
- Common variable immunodeficiency disorder (CVID) can be associated with granulomatous disease (GD) affecting the lungs.
- Pulmonary involvement in CVID/GD shares some features with sarcoidosis, necessitating differentiation.
Purpose of the Study:
- To investigate the similarities and differences between interstitial lung disease (ILD) in CVID-associated granulomatous disease and pulmonary sarcoidosis.
- To characterize the clinical, radiological, and pathological features of ILD in CVID/GD.
Main Methods:
- Retrospective study of 20 patients with CVID/GD compared to 60 controls with sarcoidosis.
- Analysis of clinical data, thoracic computed tomography (CT) scans, bronchoalveolar lavage (BAL), and pathological findings.
Main Results:
- Patients with CVID/GD exhibited more frequent crackles on clinical examination.
- CT scans revealed more frequent nodules, air bronchograms, halo signs, and bronchiectasis in CVID/GD patients.
- Bronchoalveolar lavage showed lower T-cell CD4/CD8 ratios in CVID/GD patients compared to sarcoidosis controls.
Conclusions:
- Interstitial lung disease in CVID/GD has a distinct clinical presentation, radiological features, and immunological profile compared to sarcoidosis.
- Mortality in CVID/GD patients was higher, primarily due to CVID complications, highlighting a different disease trajectory.
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