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Open Access 07-08-2023 | Computed Tomography | Original Article

Common and Uncommon CT Findings in CVID-Related GL-ILD: Correlations with Clinical Parameters, Therapeutic Decisions and Potential Implications in the Differential Diagnosis

Authors: Riccardo Scarpa, Francesco Cinetto, Cinzia Milito, Sabrina Gianese, Valentina Soccodato, Helena Buso, Giulia Garzi, Maria Carrabba, Emanuele Messina, Valeria Panebianco, Carlo Catalano, Giovanni Morana, Vassilios Lougaris, Nicholas Landini, Maria Pia Bondioni

Published in: Journal of Clinical Immunology | Issue 8/2023

Abstract

Purpose

To investigate computed tomography (CT) findings of Granulomatous Lymphocytic Interstitial Lung Disease (GL-ILD) in Common Variable Immunodeficiency (CVID), also in comparison with non-GL-ILD abnormalities, correlating GL-ILD features with functional/immunological parameters and looking for GL-ILD therapy predictive elements.

Methods

CT features of 38 GL-ILD and 38 matched non-GL-ILD subjects were retrospectively described. Correlations of GL-ILD features with functional/immunological features were assessed. A logistic regression was performed to find a predictive model of GL-ILD therapeutic decisions.

Results

Most common GL-ILD CT findings were bronchiectasis, non-perilymphatic nodules, consolidations, Ground Glass Opacities (GGO), bands and enlarged lymphnodes. GL-ILD was usually predominant in lower fields. Multiple small nodules (≤10 mm), consolidations, reticulations and fibrotic ILD are more indicative of GL-ILD. Bronchiectasis, GGO, Reticulations and fibrotic ILD correlated with decreased lung performance. Bronchiectasis, GGO and fibrotic ILD were associated with low IgA levels, whereas high CD4+ T cells percentage was related to GGO. Twenty out of 38 patients underwent GL-ILD therapy. A model combining Marginal Zone (MZ) B cells percentage, IgA levels, lower field consolidations and lymphnodes enlargement showed a good discriminatory capacity with regards to GL-ILD treatment.

Conclusions

GL-ILD is a lower field predominant disease, commonly characterized by bronchiectasis, non-perilymphatic small nodules, consolidations, GGO and bands. Multiple small nodules, consolidations, reticulations and fibrotic ILD may suggest the presence of GL-ILD in CVID. MZ B cells percentage, IgA levels at diagnosis, lower field consolidations and mediastinal lymphnodes enlargement may predict the need of a specific GL-ILD therapy.

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Title: Common and Uncommon CT Findings in CVID-Related GL-ILD: Correlations with Clinical Parameters, Therapeutic Decisions and Potential Implications in the Differential Diagnosis
Authors: Riccardo Scarpa
Francesco Cinetto
Cinzia Milito
Sabrina Gianese
Valentina Soccodato
Helena Buso
Giulia Garzi
Maria Carrabba
Emanuele Messina
Valeria Panebianco
Carlo Catalano
Giovanni Morana
Vassilios Lougaris
Nicholas Landini
Maria Pia Bondioni
Publication date: 07-08-2023
Publisher: Springer US
Keywords: Computed Tomography
Computed Tomography
Bronchiectasis
Published in: Journal of Clinical Immunology / Issue 8/2023
Print ISSN: 0271-9142
Electronic ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-023-01552-1

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