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.

Common and uncommon CT findings in CVID-related GL-ILD: correlations with clinical parameters, therapeutic decisions and potential implications in the differential diagnosis / Scarpa, Riccardo; Cinetto, Francesco; Milito, Cinzia; Gianese, Sabrina; Soccodato, Valentina; Buso, Helena; Garzi, Giulia; Carrabba, Maria; Messina, Emanuele; Panebianco, Valeria; Catalano, Carlo; Morana, Giovanni; Lougaris, Vassilios; Landini, Nicholas; Pia Bondioni, Maria. - In: JOURNAL OF CLINICAL IMMUNOLOGY. - ISSN 0271-9142. - (2023). [10.1007/s10875-023-01552-1]

Common and uncommon CT findings in CVID-related GL-ILD: correlations with clinical parameters, therapeutic decisions and potential implications in the differential diagnosis

Cinzia Milito
Secondo
;
Valentina Soccodato;Giulia Garzi;Emanuele Messina;Valeria Panebianco;Carlo Catalano;Nicholas Landini
Penultimo
;
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.
2023
CVID; chest CT; GL-ILD; airway disease; bronchiectasis; interstitial lung disease
01 Pubblicazione su rivista::01a Articolo in rivista
Common and uncommon CT findings in CVID-related GL-ILD: correlations with clinical parameters, therapeutic decisions and potential implications in the differential diagnosis / Scarpa, Riccardo; Cinetto, Francesco; Milito, Cinzia; Gianese, Sabrina; Soccodato, Valentina; Buso, Helena; Garzi, Giulia; Carrabba, Maria; Messina, Emanuele; Panebianco, Valeria; Catalano, Carlo; Morana, Giovanni; Lougaris, Vassilios; Landini, Nicholas; Pia Bondioni, Maria. - In: JOURNAL OF CLINICAL IMMUNOLOGY. - ISSN 0271-9142. - (2023). [10.1007/s10875-023-01552-1]
File allegati a questo prodotto
File Dimensione Formato  
Scarpa_Common and_2023.pdf

accesso aperto

Note: Scarpa_Common and Uncommon CT Findings_2023
Tipologia: Versione editoriale (versione pubblicata con il layout dell'editore)
Licenza: Creative commons
Dimensione 1.4 MB
Formato Adobe PDF
1.4 MB Adobe PDF

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1686125
Citazioni
  • ???jsp.display-item.citation.pmc??? 2
  • Scopus 2
  • ???jsp.display-item.citation.isi??? 2
social impact