Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in Common Variable Immunodeficiency (CVID): A Multicenter Retrospective Study of Patients From Italian PID Referral Centers
In: Frontiers in Immunology ; volume 12 ; ISSN 1664-3224, 2021
academicJournal
Zugriff:
Background: Granulomatous and Lymphocytic Interstitial Lung Diseases (GLILD) is a severe non-infectious complication of Common Variable Immunodeficiency (CVID), often associated with extrapulmonary involvement. Due to a poorly understood pathogenesis, GLILD diagnosis and management criteria still lack consensus. Accordingly, it is a relevant cause of long-term loss of respiratory function and is closely associated with a markedly reduced survival. The aim of this study was to describe clinical, immunological, laboratory and functional features of GLILD, whose combination in a predictive model might allow a timely diagnosis. Methods: In a multicenter retrospective cross-sectional study we enrolled 73 CVID patients with radiologic features of interstitial lung disease (ILD) associated to CVID (CVID-ILD) and 125 CVID patients without ILD (controls). Of the 73 CVID-ILD patients, 47 received a definite GLILD diagnosis while 26 received a clinical-radiologic diagnosis of CVID related ILD defined as uILD. Results: In GLILD group we found a higher prevalence of splenomegaly (84.8 vs. 39.2%), autoimmune cytopenia (59.6 vs. 6.4%) and bronchiectasis (72.3 vs. 28%), and lower IgA and IgG serum levels at CVID diagnosis. GLILD patients presented lower percentage of switched-memory B cells and marginal zone B cells, and a marked increase in the percentage of circulating CD21lo B cells (14.2 vs. 2.9%). GLILD patients also showed lower total lung capacity (TLC 87.5 vs. 5.0%) and gas transfer (DLCO 61.5 vs. 5.0%) percent of predicted. By univariate logistic regression analysis, we found IgG and IgA levels at CVID diagnosis, presence of splenomegaly and autoimmune cytopenia, CD21lo B cells percentage, TLC and DCLO percent of predicted to be associated to GLILD. The joint analysis of four variables (CD21lo B cells percentage, autoimmune cytopenia, splenomegaly and DLCO percent of predicted), together in a multiple logistic regression model, yielded an area under the ROC curve (AUC) of 0.98 (95% CI: 0.95-1.0). The AUC was only ...
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Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in Common Variable Immunodeficiency (CVID): A Multicenter Retrospective Study of Patients From Italian PID Referral Centers
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Autor/in / Beteiligte Person: | Cinetto, Francesco ; Scarpa, Riccardo ; Carrabba, Maria ; Firinu, Davide ; Lougaris, Vassilios ; Buso, Helena ; Garzi, Giulia ; Gianese, Sabrina ; Soccodato, Valentina ; Punziano, Alessandra ; Lagnese, Gianluca ; Tessarin, Giulio ; Costanzo, Giulia ; Landini, Nicholas ; Vio, Stefania ; Bondioni, Maria Pia ; Consonni, Dario ; Marasco, Carolina ; Del Giacco, Stefano ; Rattazzi, Marcello ; Vacca, Angelo ; Plebani, Alessandro ; Fabio, Giovanna ; Spadaro, Giuseppe ; Agostini, Carlo ; Quinti, Isabella ; Milito, Cinzia |
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Zeitschrift: | Frontiers in Immunology ; volume 12 ; ISSN 1664-3224, 2021 |
Veröffentlichung: | Frontiers Media SA, 2021 |
Medientyp: | academicJournal |
DOI: | 10.3389/fimmu.2021.627423 |
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