Interstitial lung disease in patients with common variable immunodeficiency disorders: several different pathologies?

Area of Science:

• Pulmonology
• Immunology
• Pathology

Background:

• Common variable immunodeficiency disorder (CVID) is associated with diverse lung pathologies.
• Histological and high-resolution computed tomography (HRCT) findings in CVID-related lung disease vary significantly.
• Interstitial lung disease (ILD) is a severe complication in CVID patients.

Purpose of the Study:

• To investigate the relationship between lung histology, HRCT findings, and clinical outcomes in CVID patients with suspected ILD.
• To identify distinct pathological processes contributing to lung disease in CVID.
• To determine the need for standardized pathological and immunochemical data in CVID ILD.

Main Methods:

• Retrospective analysis of data from the Oxford Primary Immune Deficiencies (PID) database (1986-2016).
• Re-analysis of lung biopsies from 16 adult, sporadic CVID patients with suspected ILD.
• Correlation of histological findings with HRCT, clinical outcomes, and immunochemistry (CD4/CD8 T cells).

Main Results:

• Lymphocytic infiltration without a recognizable pattern was the most common histological finding.
• No well-formed granulomata were observed in lung biopsies, despite systemic granulomata in some patients.
• HRCT nodules were frequent but not correlated with histological findings; outcomes were variable and not histology-related.

Conclusions:

• CVID-related lung disease exhibits diverse pathologies, often characterized by lymphocytic infiltration, which do not consistently correlate with HRCT findings or patient outcomes.
• The absence of granulomata in lung biopsies, even with systemic involvement, highlights the complexity of CVID lung pathologies.
• Standardized international pathology and immunochemical data are crucial for future longitudinal studies to clarify CVID ILD pathologies and guide therapy.