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

Author

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, and Bondioni, Maria Pia

Published
2023
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4. Benralizumab for eosinophilic granulomatosis with polyangiitis: a retrospective, multicentre, cohort study

Author

Bettiol, Alessandra, Urban, Maria Letizia, Padoan, Roberto, Groh, Matthieu, Lopalco, Giuseppe, Egan, Allyson, Cottin, Vincent, Fraticelli, Paolo, Crimi, Claudia, Del Giacco, Stefano, Losappio, Laura, Moi, Laura, Cinetto, Francesco, Caminati, Marco, Novikov, Pavel, Berti, Alvise, Cameli, Paolo, Cathébras, Pascal, Coppola, Angelo, Durel, Cécile-Audrey, Folci, Marco, Gullo, Alberto Lo, Lombardi, Carlo, Monti, Sara, Parronchi, Paola, Rivera, Carlos Martinez, Solans, Roser, Vacca, Angelo, Espígol-Frigolé, Georgina, Guarnieri, Gabriella, Bianchi, Fulvia Chieco, Marchi, Maria Rita, Tcherakian, Colas, Kahn, Jean-Emmanuel, Iannone, Florenzo, Venerito, Vincenzo, Desaintjean, Charlene, Moroncini, Gianluca, Nolasco, Santi, Costanzo, Giulia Anna Maria Luigia, Schroeder, Jan Walter, Ribi, Camillo, Tesi, Michelangelo, Gelain, Elena, Mattioli, Irene, Bello, Federica, Jayne, David, Prisco, Domenico, Vaglio, Augusto, and Emmi, Giacomo

Published
2023
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6. The Impact of SARS-CoV-2 Infection in Patients with Inborn Errors of Immunity: the Experience of the Italian Primary Immunodeficiencies Network (IPINet)

Author

Giardino, Giuliana, Milito, Cinzia, Lougaris, Vassilios, Punziano, Alessandra, Carrabba, Maria, Cinetto, Francesco, Scarpa, Riccardo, Dellepiane, Rosa Maria, Ricci, Silvia, Rivalta, Beatrice, Conti, Francesca, Marzollo, Antonio, Firinu, Davide, Cirillo, Emilia, Lagnese, Gianluca, Cancrini, Caterina, Martire, Baldassare, Danieli, Maria Giovanna, Pession, Andrea, Vacca, Angelo, Azzari, Chiara, Fabio, Giovanna, Soresina, Annarosa, Agostini, Carlo, Spadaro, Giuseppe, Badolato, Raffaele, Cicalese, Maria Pia, Aiuti, Alessandro, Plebani, Alessandro, Quinti, Isabella, and Pignata, Claudio

Published
2022
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8. COVID-19 Clinical Features and Outcome in Italian Patients Treated with Biological Drugs Targeting Type 2 Inflammation

Author

Sambugaro, Giada, primary, Brambilla, Elena, additional, Costanzo, Giulia, additional, Bonato, Vera, additional, Ledda, Andrea Giovanni, additional, Del Giacco, Stefano, additional, Scarpa, Riccardo, additional, Rattazzi, Marcello, additional, Favero, Elisabetta, additional, Cinetto, Francesco, additional, and Firinu, Davide, additional

Published
2024
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10. Breaking barriers: holistic assessment of ability to work in patients with sarcoidosis

Author

Drent, Marjolein, Russell, Anne-Marie, Saketkoo, Lesley Ann, Spagnolo, Paolo, Veltkamp, Marcel, Wells, Athol U, Goh, Nicole, Holland, Anne, Hochreiter, Johann, Kock, Selene, Verheyen, Karolien, Verbraecken, Johan, Cantin, André, Gillson, Ashley, Hambly, Nathan, Kolb, Martin, Bendstrup, Elisabeth, Leistiko, Anna, Møller, Janne, Kaarteenaho, Riitta, Crestani, Bruno, Israël-Biet, Dominique, Cohen Aubart, Fleur, Nunes, Hilario, Valeyre, Dominique, Bonella, Francesco, Buschulte, Katharina, Costabel, Ulrich, Kortsen, Peter, Kreuter, Michael, Luedike, Peter, Quadder, Bernd, Braune, Renate, Skowasch, Dirk, Antoniou, Katerina, Bouros, Demosthenes, Gogali, Athena, Margaritopoulos, George A., Papakosta, Despoina, Papiris, Spyros A, Manali, Effrosyni D, Tzouvelekis, Argyris, Tzilas, Vasilios, Agrawal, Aditya, Kashyap, Surender, Tyagi, Vijay Narain, Unnati, Desai, Abedini, Atefeh, Phelan, Sean, Bargagli, Elena, Cinetto, Francesco, Confalonieri, Marco, Confalonieri, Paola, Harari, Sergio, Luppi, Fabrizio, Martone, Filippo, Palmieri, Anna, Rottoli, Paola, Salton, Francesco, Scarpa, Ricardo, Tana, Claudio, Tomassetti, Sara, Azuma, Arata, Inui, Naoki, Nakamura, Yutaro, Inoue, Yoshikazu, Tanino, Yoshinori, Muhammad, Noorul Afidza, van Beek, Frouke, den Boer, Lenze, Boomars, Karin, Bresser, Paul, Claessen, Laura, Elfferich, Marjon, Erckens, Roel, van Helmondt, Sanne, Karakaya, Bekir, Grutters, Jan C., Hoitsma, Elske, Illy, Károly, Jansen, Tim L, Kahlmann, Vivienne, Knoet, Chris, Korenromp, Ingrid, Lenssen, Ton, Maassen vd Brinkdrs, Karen, Mager, Johannes Jurgen, Marcellis, Rik, Miedema, Jelle, Möller, Trudeke, Moonen, Linda A.A., Moor, Karen, Mostard, Rémy L.M., Nienhuis, Willemien, Nossent, Esther, Paulus, Michelle, Ponds, Rudolf, Nossent, George, Quanjel, Marian, Rottenberg, Felix, van de Sande, Jonathan S.O., Segers, Ruud, Soechitram, Shalini D., van Staa, AnneLoes, Strookappe, Bert, Swaans, Martin, van den Toorn, Leon, van de Ven, Marjo J.T., Voortman, Mareye, Vorselaars, Adriane Dore Marie, Vente, Lenneke, Wijnen, Petal A., Wijsenbeek, Marlies, De Vries, Jolanda, van Zanten, Arthur, Durheim, Michael, Rasch-Halvorsen, Øystein, Tøndell, Anders, Dubaniewicz, Anna, Piotrowski, Wojciech, Roter, Bartlomiej, Robalo Cordeiro, Carlos, Alfaro, Tiago, Strambu, Irina, Vizel, Alexander, Cano, Rafael, Balcells, Eva, Castillo, Diego, Francesqui, Joel, Mañá, Juan, Molina, Maria, Ojanguren, Inigo, Rodriguez Portal, Jose Antonio, Sellares, Jacobo, Arkema, Elizabeth, Darlington, Pernilla, Eldhagen, Per, Elfström, Katinka, Grunewald, Johan, Kullberg, Susanna, Rivera, Natalia V, Rossides, Marios, Werner, Joanna, Dincer, Irem, Koç, Gülcan, Yucesan, Canan, Ozdemir Kumbasar, Ozlem, Birring, Surinder, Boyd, Jeanette, Casimo, Leo, Huntley, Christopher, Kouranos, Vasileios, Newton, Jaqui, Spencer, Lisa, Spears, Mark, Barreras, Paula, Cozier, Yvette, Crouser, Elliott, Harper, Logan, James, W. Ennis, Maier, Lisa, Liao, Shu-Yi, Morgenthau, Adam, Obi, Ogugua Ndili, Patel, Divya, Rivera, Frank, Rossman, Milton D., Sharp, Michelle, Sharma, Maggie, Schupp, Jonas C., Stern, Barney J., Sweiss, Nadera, Tavee, Jinny, Zank, Daniel, Gumeniuk, Galyna, Gavrysyuk, Volodymyr, and Merenkova, Levgeniia

Published
2024
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12. High Prevalence of Long COVID in Common Variable Immunodeficiency:An Italian Multicentric Study

Author

Villa, Annalisa, Milito, Cinzia, Deiana, Carla Maria, Gambier, Renato Finco, Punziano, Alessandra, Buso, Helena, Bez, Patrick, Lagnese, Gianluca, Garzi, Giulia, Costanzo, Giulia, Giannuzzi, Gloria, Pagnozzi, Chiara, Dalm, Virgil A.S.H., Spadaro, Giuseppe, Rattazzi, Marcello, Cinetto, Francesco, Firinu, Davide, Villa, Annalisa, Milito, Cinzia, Deiana, Carla Maria, Gambier, Renato Finco, Punziano, Alessandra, Buso, Helena, Bez, Patrick, Lagnese, Gianluca, Garzi, Giulia, Costanzo, Giulia, Giannuzzi, Gloria, Pagnozzi, Chiara, Dalm, Virgil A.S.H., Spadaro, Giuseppe, Rattazzi, Marcello, Cinetto, Francesco, and Firinu, Davide

Abstract

The long-term effects of SARS-CoV-2 infection represent a relevant global health problem. Long COVID (LC) is defined as a complex of signs and symptoms developed during or after SARS-CoV-2 infection and lasting > 12 weeks. In common variable immunodeficiency (CVID) patients, we previously reported higher risk of hospitalization and death during SARS-CoV-2 infection, as well as prolonged swab positivity and frequent reinfections. The aim of the present study was to assess the risk of LC in an Italian cohort of CVID patients. We used a translated version of the survey proposed by Centers for Disease Control and Prevention (CDC) to collect data on LC. In the enrolled cohort of 175 CVID patients, we found a high prevalence of LC (65.7%). The most frequent LC symptoms were fatigue (75.7%), arthralgia/myalgia (48.7%), and dyspnea (41.7%). The majority of patients (60%) experienced prolonged symptoms, for at least 6 months after infection. In a multivariate analysis, the presence of complicated phenotype (OR 2.44, 95% CI 1.88-5.03; p = 0.015), obesity (OR 11.17, 95% CI 1.37-90.95; p = 0.024), and female sex (OR 2.06, 95% CI 1.09-3.89; p = 0.024) significantly correlated with the development of LC. In conclusion, in this multicenter observational cohort study, we demonstrated that CVID patients present an increased prevalence of LC when compared to the general population. Improved awareness on the risk of LC in CVID patients could optimize management of this new and alarming complication of SARS-CoV-2 infection.

Published
2024

13. Sarcoidosis versus Granulomatous and Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency: A Comparative Review.

Author

Buso, Helena, Discardi, Claudia, Bez, Patrick, Muscianisi, Francesco, Ceccato, Jessica, Milito, Cinzia, Firinu, Davide, Landini, Nicholas, Jones, Mark G., Felice, Carla, Rattazzi, Marcello, Scarpa, Riccardo, and Cinetto, Francesco

Subjects
COMMON variable immunodeficiency, INTERSTITIAL lung diseases, LUNG development, SARCOIDOSIS, QUALITY of life, OPEN-ended questions
Abstract

Sarcoidosis and Granulomatous and Lymphocytic Interstitial Lung Diseases (GLILD) are two rare entities primarily characterised by the development of Interstitial Lung Disease (ILD) in the context of systemic immune dysregulation. These two conditions partially share the immunological background and pathologic findings, with granuloma as the main common feature. In this narrative review, we performed a careful comparison between sarcoidosis and GLILD, with an overview of their main similarities and differences, starting from a clinical perspective and ending with a deeper look at the immunopathogenesis and possible target therapies. Sarcoidosis occurs in immunocompetent individuals, whereas GLILD occurs in patients affected by common variable immunodeficiency (CVID). Moreover, peculiar extrapulmonary manifestations and radiological and histological features may help distinguish the two diseases. Despite that, common pathogenetic pathways have been suggested and both these disorders can cause progressive impairment of lung function and variable systemic granulomatous and non-granulomatous complications, leading to significant morbidity, reduced quality of life, and survival. Due to the rarity of these conditions and the extreme clinical variability, there are still many open questions concerning their pathogenesis, natural history, and optimal management. However, if studied in parallel, these two entities might benefit from each other, leading to a better understanding of their pathogenesis and to more tailored treatment approaches. [ABSTRACT FROM AUTHOR]

Published
2024
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14. Long-term follow-up of 168 patients with X-linked agammaglobulinemia reveals increased morbidity and mortality

Author

Lougaris, Vassilios, Soresina, Annarosa, Baronio, Manuela, Montin, Davide, Martino, Silvana, Signa, Sara, Volpi, Stefano, Zecca, Marco, Marinoni, Maddalena, Baselli, Lucia Augusta, Dellepiane, Rosa Maria, Carrabba, Maria, Fabio, Giovanna, Putti, Maria Caterina, Cinetto, Francesco, Lunardi, Claudio, Gazzurelli, Luisa, Benvenuto, Alessio, Bertolini, Patrizia, Conti, Francesca, Consolini, Rita, Ricci, Silvia, Azzari, Chiara, Leonardi, Lucia, Duse, Marzia, Pulvirenti, Federica, Milito, Cinzia, Quinti, Isabella, Cancrini, Caterina, Finocchi, Andrea, Moschese, Viviana, Cirillo, Emilia, Crescenzi, Ludovica, Spadaro, Giuseppe, Marasco, Carolina, Vacca, Angelo, Cardinale, Fabio, Martire, Baldassare, Trizzino, Antonino, Licciardello, Maria, Cossu, Fausto, Di Matteo, Gigliola, Badolato, Raffaele, Ferrari, Simona, Giliani, Silvia, Pession, Andrea, Ugazio, Alberto, Pignata, Claudio, and Plebani, Alessandro

Published
2020
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18. Breaking barriers: holistic assessment of ability to work in patients with sarcoidosis

Author

Goh, Nicole, Holland, Anne, Hochreiter, Johann, Kock, Selene, Verheyen, Karolien, Verbraecken, Johan, Cantin, André, Gillson, Ashley, Hambly, Nathan, Kolb, Martin, Bendstrup, Elisabeth, Leistiko, Anna, Møller, Janne, Kaarteenaho, Riitta, Crestani, Bruno, Israël-Biet, Dominique, Cohen Aubart, Fleur, Nunes, Hilario, Valeyre, Dominique, Bonella, Francesco, Buschulte, Katharina, Costabel, Ulrich, Kortsen, Peter, Kreuter, Michael, Luedike, Peter, Quadder, Bernd, Braune, Renate, Skowasch, Dirk, Antoniou, Katerina, Bouros, Demosthenes, Gogali, Athena, Margaritopoulos, George A., Papakosta, Despoina, Papiris, Spyros A, Manali, Effrosyni D, Tzouvelekis, Argyris, Tzilas, Vasilios, Agrawal, Aditya, Kashyap, Surender, Tyagi, Vijay Narain, Unnati, Desai, Abedini, Atefeh, Phelan, Sean, Bargagli, Elena, Cinetto, Francesco, Confalonieri, Marco, Confalonieri, Paola, Harari, Sergio, Luppi, Fabrizio, Martone, Filippo, Palmieri, Anna, Rottoli, Paola, Salton, Francesco, Scarpa, Ricardo, Tana, Claudio, Tomassetti, Sara, Azuma, Arata, Inui, Naoki, Nakamura, Yutaro, Inoue, Yoshikazu, Tanino, Yoshinori, Muhammad, Noorul Afidza, van Beek, Frouke, den Boer, Lenze, Boomars, Karin, Bresser, Paul, Claessen, Laura, Elfferich, Marjon, Erckens, Roel, van Helmondt, Sanne, Karakaya, Bekir, Grutters, Jan C., Hoitsma, Elske, Illy, Károly, Jansen, Tim L, Kahlmann, Vivienne, Knoet, Chris, Korenromp, Ingrid, Lenssen, Ton, Maassen vd Brinkdrs, Karen, Mager, Johannes Jurgen, Marcellis, Rik, Miedema, Jelle, Möller, Trudeke, Moonen, Linda A.A., Moor, Karen, Mostard, Rémy L.M., Nienhuis, Willemien, Nossent, Esther, Paulus, Michelle, Ponds, Rudolf, Nossent, George, Quanjel, Marian, Rottenberg, Felix, van de Sande, Jonathan S.O., Segers, Ruud, Soechitram, Shalini D., van Staa, AnneLoes, Strookappe, Bert, Swaans, Martin, van den Toorn, Leon, van de Ven, Marjo J.T., Voortman, Mareye, Vorselaars, Adriane Dore Marie, Vente, Lenneke, Wijnen, Petal A., Wijsenbeek, Marlies, De Vries, Jolanda, van Zanten, Arthur, Durheim, Michael, Rasch-Halvorsen, Øystein, Tøndell, Anders, Dubaniewicz, Anna, Piotrowski, Wojciech, Roter, Bartlomiej, Robalo Cordeiro, Carlos, Alfaro, Tiago, Strambu, Irina, Vizel, Alexander, Cano, Rafael, Balcells, Eva, Castillo, Diego, Francesqui, Joel, Mañá, Juan, Molina, Maria, Ojanguren, Inigo, Rodriguez Portal, Jose Antonio, Sellares, Jacobo, Arkema, Elizabeth, Darlington, Pernilla, Eldhagen, Per, Elfström, Katinka, Grunewald, Johan, Kullberg, Susanna, Rivera, Natalia V, Rossides, Marios, Werner, Joanna, Dincer, Irem, Koç, Gülcan, Yucesan, Canan, Ozdemir Kumbasar, Ozlem, Birring, Surinder, Boyd, Jeanette, Casimo, Leo, Huntley, Christopher, Kouranos, Vasileios, Newton, Jaqui, Spencer, Lisa, Spears, Mark, Barreras, Paula, Cozier, Yvette, Crouser, Elliott, Harper, Logan, James, W. Ennis, Maier, Lisa, Liao, Shu-Yi, Morgenthau, Adam, Obi, Ogugua Ndili, Patel, Divya, Rivera, Frank, Rossman, Milton D., Sharp, Michelle, Sharma, Maggie, Schupp, Jonas C., Stern, Barney J., Sweiss, Nadera, Tavee, Jinny, Zank, Daniel, Gumeniuk, Galyna, Gavrysyuk, Volodymyr, Merenkova, Levgeniia, Drent, Marjolein, Russell, Anne-Marie, Saketkoo, Lesley Ann, Spagnolo, Paolo, Veltkamp, Marcel, and Wells, Athol U

Published
2024
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19. Double-blind, placebo-controlled, randomized trial on low-dose azithromycin prophylaxis in patients with primary antibody deficiencies

Author

Milito, Cinzia, Pulvirenti, Federica, Cinetto, Francesco, Lougaris, Vassilios, Soresina, Annarosa, Pecoraro, Antonio, Vultaggio, Alessandra, Carrabba, Maria, Lassandro, Giuseppe, Plebani, Alessandro, Spadaro, Giuseppe, Matucci, Andrea, Fabio, Giovanna, Dellepiane, Rosa Maria, Martire, Baldassarre, Agostini, Carlo, Abeni, Damiano, Tabolli, Stefano, and Quinti, Isabella

Published
2019
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20. Decline of gastric cancer mortality in common variable immunodeficiency in the years 2018-2022

Author

Milito, Cinzia, primary, Pulvirenti, Federica, additional, Garzi, Giulia, additional, Sculco, Eleonora, additional, Cinetto, Francesco, additional, Firinu, Davide, additional, Lagnese, Gianluca, additional, Punziano, Alessandra, additional, Discardi, Claudia, additional, Costanzo, Giulia, additional, Felice, Carla, additional, Spadaro, Giuseppe, additional, Ferrari, Simona, additional, and Quinti, Isabella, additional

Published
2023
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24. AUTOREACTIVE T CELLS TO ANGIOTENSIN II TYPE 1 RECEPTOR IN SALT-DEPENDENT HYPERTENSION DUE TO PRIMARY ALDOSTERONISM

Author

Scarpa, Riccardo, primary, Piazza, Maria, additional, Caroccia, Brasilina, additional, Carraro, Samuela, additional, Torresan, Francesca, additional, Iacobone, Maurizio, additional, Vianello, Fabrizio, additional, Cinetto, Francesco, additional, Agostini, Carlo, additional, Seccia, Teresa Maria, additional, and Rossi, Gian Paolo, additional

Published
2023
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27. A beacon in the dark: COVID-19 course in CVID patients from two European countries: Different approaches, similar outcomes

Author

Milito, Cinzia, Firinu, Davide, Bez, Patrick, Villa, Annalisa, Punziano, Alessandra, Lagnese, Gianluca, Costanzo, Giulia, van Leeuwen, Leanne P M, Piazza, Beatrice, Deiana, Carla Maria, d'Ippolito, Giancarlo, Del Giacco, Stefano Renato, Rattazzi, Marcello, Spadaro, Giuseppe, Quinti, Isabella, Scarpa, Riccardo, Dalm, Virgil A S H, Cinetto, Francesco, Virology, Internal Medicine, Milito, Cinzia, Firinu, Davide, Bez, Patrick, Villa, Annalisa, Punziano, Alessandra, Lagnese, Gianluca, Costanzo, Giulia, van Leeuwen, Leanne P M, Piazza, Beatrice, Deiana, Carla Maria, D'Ippolito, Giancarlo, Del Giacco, Stefano Renato, Rattazzi, Marcello, Spadaro, Giuseppe, Quinti, Isabella, Scarpa, Riccardo, Dalm, Virgil A S H, and Cinetto, Francesco

Subjects
Common Variable Immunodeficiency (CVID), SDG 3 - Good Health and Well-being, SARS-CoV-2, precision medicine, Immunology, outpatient, Immunology and Allergy, COVID-19, risk factors
Abstract

BackgroundCVID patients present an increased risk of prolonged SARS-CoV-2 infection and re-infection and a higher COVID-19-related morbidity and mortality compared to the general population. Since 2021, different therapeutic and prophylactic strategies have been employed in vulnerable groups (vaccination, SARS-CoV-2 monoclonal antibodies and antivirals). The impact of treatments over the last 2 years has not been explored in international studies considering the emergence of viral variants and different management between countries.MethodsA multicenter retrospective/prospective real-life study comparing the prevalence and outcomes of SARS-CoV-2 infection between a CVID cohort from four Italian Centers (IT-C) and one cohort from the Netherlands (NL-C), recruiting 773 patients.Results329 of 773 CVID patients were found positive for SARS-CoV-2 infection between March 1st, 2020 and September 1st 2022. The proportion of CVID patients infected was comparable in both national sub-cohorts. During all waves, chronic lung disease, “complicated” phenotype, chronic immunosuppressive treatment and cardiovascular comorbidities impacted on hospitalization, whereas risk factors for mortality were older age, chronic lung disease, and bacterial superinfections. IT-C patients were significantly more often treated, both with antivirals and mAbs, than NL-C patients. Outpatient treatment, available only in Italy, started from the Delta wave. Despite this, no significant difference was found for COVID-19 severity between the two cohorts. However, pooling together specific SARS-CoV-2 outpatient treatments (mAbs and antivirals), we found a significant effect on the risk of hospitalization starting from Delta wave. Vaccination with ≥ 3 doses shortened RT-PCR positivity, with an additional effect only in patients receiving antivirals.ConclusionsThe two sub-cohorts had similar COVID-19 outcomes despite different treatment approaches. This points out that specific treatment should now be reserved for selected subgroups of CVID patients, based on pre-existing conditions.

Published
2023

30. Biomarkers of Neutrophil Activation in Patients with Symptomatic Chronic Peripheral Artery Disease Predict Worse Cardiovascular Outcome

Author

Buso, Giacomo, primary, Faggin, Elisabetta, additional, Bressan, Alessandro, additional, Galliazzo, Silvia, additional, Cinetto, Francesco, additional, Felice, Carla, additional, Fusaro, Michele, additional, Erdmann, Andreas, additional, Pauletto, Paolo, additional, Rattazzi, Marcello, additional, and Mazzolai, Lucia, additional

Published
2023
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32. A beacon in the dark:COVID-19 course in CVID patients from two European countries: Different approaches, similar outcomes

Author

Milito, Cinzia, Firinu, Davide, Bez, Patrick, Villa, Annalisa, Punziano, Alessandra, Lagnese, Gianluca, Costanzo, Giulia, van Leeuwen, Leanne P.M., Piazza, Beatrice, Deiana, Carla Maria, d’Ippolito, Giancarlo, Del Giacco, Stefano Renato, Rattazzi, Marcello, Spadaro, Giuseppe, Quinti, Isabella, Scarpa, Riccardo, Dalm, Virgil A.S.H., Cinetto, Francesco, Milito, Cinzia, Firinu, Davide, Bez, Patrick, Villa, Annalisa, Punziano, Alessandra, Lagnese, Gianluca, Costanzo, Giulia, van Leeuwen, Leanne P.M., Piazza, Beatrice, Deiana, Carla Maria, d’Ippolito, Giancarlo, Del Giacco, Stefano Renato, Rattazzi, Marcello, Spadaro, Giuseppe, Quinti, Isabella, Scarpa, Riccardo, Dalm, Virgil A.S.H., and Cinetto, Francesco

Abstract

Background: CVID patients present an increased risk of prolonged SARS-CoV-2 infection and re-infection and a higher COVID-19-related morbidity and mortality compared to the general population. Since 2021, different therapeutic and prophylactic strategies have been employed in vulnerable groups (vaccination, SARS-CoV-2 monoclonal antibodies and antivirals). The impact of treatments over the last 2 years has not been explored in international studies considering the emergence of viral variants and different management between countries. Methods: A multicenter retrospective/prospective real-life study comparing the prevalence and outcomes of SARS-CoV-2 infection between a CVID cohort from four Italian Centers (IT-C) and one cohort from the Netherlands (NL-C), recruiting 773 patients. Results: 329 of 773 CVID patients were found positive for SARS-CoV-2 infection between March 1st, 2020 and September 1st 2022. The proportion of CVID patients infected was comparable in both national sub-cohorts. During all waves, chronic lung disease, “complicated” phenotype, chronic immunosuppressive treatment and cardiovascular comorbidities impacted on hospitalization, whereas risk factors for mortality were older age, chronic lung disease, and bacterial superinfections. IT-C patients were significantly more often treated, both with antivirals and mAbs, than NL-C patients. Outpatient treatment, available only in Italy, started from the Delta wave. Despite this, no significant difference was found for COVID-19 severity between the two cohorts. However, pooling together specific SARS-CoV-2 outpatient treatments (mAbs and antivirals), we found a significant effect on the risk of hospitalization starting from Delta wave. Vaccination with ≥ 3 doses shortened RT-PCR positivity, with an additional effect only in patients receiving antivirals. Conclusions: The two sub-cohorts had similar COVID-19 outcomes despite different treatment approaches. This points out that spe

Published
2023

33. NETosis in Acute Thrombotic Disorders.

Author

Bressan, Alessandro, Faggin, Elisabetta, Donato, Maristella, Tonon, Luca, Buso, Roberta, Nardin, Chiara, Tiepolo, Marta, Cinetto, Francesco, Scarpa, Riccardo, Agostini, Carlo, Pauletto, Paolo, Ventura, Laura, Fusaro, Michele, Felice, Carla, and Rattazzi, Marcello

Subjects
CHEST pain, LIPOCALIN-2, LEUCOCYTE elastase, ACUTE coronary syndrome, STROKE, RECEIVER operating characteristic curves
Abstract

The release of extracellular traps by neutrophils (NETs) represents a novel active mechanism of cell death that has been recently implicated in the pathogenesis of thrombotic disorders. The aim of this study was to investigate the generation of NETs in different groups of patients with acute thrombotic events (ATEs) and to establish whether NETs markers can predict the risk of new cardiovascular events. We performed a case–control study of patients with ATE, including acute coronary syndrome (n = 60), cerebrovascular accident (n = 50), and venous thromboembolism (n = 55). Control subjects (n = 70) were identified among patients admitted for acute chest pain and in which a diagnosis of ATE was excluded. Serum levels of NET markers and neutrophil activation, such as myeloperoxidase (MPO)-DNA complexes, neutrophil gelatinase-associated lipocalin, polymorphonuclear neutrophil elastase, lactoferrin, and MPO, were measured in each patient. We found that circulating levels of MPO-DNA complexes were significantly increased in patients with ATE (p < 0.001) compared with controls and that this association remained significant even after fully adjustment for traditional risk factors (p = 0.001). A receiver operating characteristics analysis of circulating MPO-DNA complexes in discriminating between controls and patients with ATE showed a significant area under the curve of 0.76 (95% confidence interval: 0.69–0.82). After a median follow-up of 40.7 (± 13.8) months, 24 out of the 165 patients with ATE presented a new cardiovascular event and 18 patients died. None of the markers under investigation influenced survival or the incidence of new cardiovascular events. In conclusion, we found that increase of markers of NETosis can be observed in acute thrombotic conditions, occurring both on the arterial and venous site. Nevertheless, the level of neutrophil markers measured during the ATE is not predictive of future risk of mortality and cardiovascular events. [ABSTRACT FROM AUTHOR]

Published
2023
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34. The Importance of Endoscopy with Biopsy: Real-World Evidence of Gastrointestinal Involvement in Primary Immunodeficiency in Two Main Northern Italian Centres

Author

Nicola, Stefania, primary, Cinetto, Francesco, additional, Della Mura, Stefano, additional, Lo Sardo, Luca, additional, Saracco, Elena, additional, Vitali, Ilaria, additional, Scarpa, Riccardo, additional, Buso, Helena, additional, Bonato, Vera, additional, Discardi, Claudia, additional, Rolla, Giovanni, additional, Felice, Carla, additional, Rattazzi, Marcello, additional, and Brussino, Luisa, additional

Published
2023
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36. Safety of mRNA COVID-19 Vaccines in Patients with Inborn Errors of Immunity: an Italian Multicentric Study

Author

Milito, Cinzia, primary, Cinetto, Francesco, additional, Garzi, Giulia, additional, Palladino, Andrea, additional, Puca, Marco, additional, Brambilla, Elena, additional, De Vitis, Camilla, additional, Costanzo, Giulia, additional, Scarpa, Riccardo, additional, Punziano, Alessandra, additional, Lagnese, Gianluca, additional, Del Giacco, Stefano, additional, Spadaro, Giuseppe, additional, Quinti, Isabella, additional, and Firinu, Davide, additional

Published
2022
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37. A bone-based 3D scaffold as an in-vitro model of microenvironment–DLBCL lymphoma cell interaction

Author

Ceccato, Jessica, primary, Piazza, Maria, additional, Pizzi, Marco, additional, Manni, Sabrina, additional, Piazza, Francesco, additional, Caputo, Ilaria, additional, Cinetto, Francesco, additional, Pisoni, Lorena, additional, Trojan, Diletta, additional, Scarpa, Riccardo, additional, Zambello, Renato, additional, Tos, Angelo Paolo Dei, additional, Trentin, Livio, additional, Semenzato, Gianpietro, additional, and Vianello, Fabrizio, additional

Published
2022
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38. Sarcoidosis and COVID-19: At the Cross-Road between Immunopathology and Clinical Manifestation

Author

Tana, Claudio, primary, Cinetto, Francesco, additional, Mantini, Cesare, additional, Bernardinello, Nicol, additional, Tana, Marco, additional, Ricci, Fabrizio, additional, Ticinesi, Andrea, additional, Meschi, Tiziana, additional, Scarpa, Riccardo, additional, Cipollone, Francesco, additional, Giamberardino, Maria Adele, additional, and Spagnolo, Paolo, additional

Published
2022
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39. Common and uncommon CT findings in CVID related GL-ILD: correlations with clinical parameters, therapeutic decisions and potential implications in the differential diagnosis

Author

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

Published
2022
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41. Struggling with COVID-19 in Adult Inborn Errors of Immunity Patients: A Case Series of Combination Therapy and Multiple Lines of Therapy for Selected Patients.

Author

Bez, Patrick, D'ippolito, Giancarlo, Deiana, Carla Maria, Finco Gambier, Renato, Pica, Andrea, Costanzo, Giulia, Garzi, Giulia, Scarpa, Riccardo, Landini, Nicholas, Cinetto, Francesco, Firinu, Davide, and Milito, Cinzia

Subjects
CONVALESCENT plasma, COVID-19, MONOCLONAL antibodies, VACCINE effectiveness, ADULTS, CHILD patients
Abstract

Background: The SARS-CoV-2 infection is now a part of the everyday lives of immunocompromised patients, but the choice of treatment and the time of viral clearance can often be complex, exposing patients to possible complications. The role of the available antiviral and monoclonal therapies is a matter of debate, as are their effectiveness and potential related adverse effects. To date, in the literature, the amount of data on the use of combination therapies and on the multiple lines of anti-SARS-CoV-2 therapy available to the general population and especially to inborn error of immunity (IEI) patients is small. Methods: Here, we report a case series of five adult IEI patients managed as inpatients at three Italian IEI referral centers (Rome, Treviso, and Cagliari) treated with combination therapy or multiple therapeutic lines for SARS-CoV-2 infection, such as monoclonal antibodies (mAbs), antivirals, convalescent plasma (CP), mAbs plus antiviral, and CP combined with antiviral. Results: This study may support the use of combination therapy against SARS-CoV-2 in complicated IEI patients with predominant antibody deficiency and impaired vaccine response. [ABSTRACT FROM AUTHOR]

Published
2023
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42. Real-life data on monoclonal antibodies and antiviral drugs in Italian inborn errors of immunity patients during COVID-19 pandemic

Author

Garzi, Giulia, primary, Cinetto, Francesco, additional, Firinu, Davide, additional, Di Napoli, Giulia, additional, Lagnese, Gianluca, additional, Punziano, Alessandra, additional, Bez, Patrick, additional, Cinicola, Bianca Laura, additional, Costanzo, Giulia, additional, Scarpa, Riccardo, additional, Pulvirenti, Federica, additional, Rattazzi, Marcello, additional, Spadaro, Giuseppe, additional, Quinti, Isabella, additional, and Milito, Cinzia, additional

Published
2022
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43. Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis (EGPA): a European multicenter observational study

Author

Bettiol, A, Urban, M, Dagna, L, Cottin, V, Franceschini, F, Del Giacco, S, Schiavon, F, Neumann, T, Lopalco, G, Novikov, P, Baldini, C, Lombardi, C, Berti, A, Alberici, F, Folci, M, Negrini, S, Sinico, R, Quartuccio, L, Lunardi, C, Parronchi, P, Moosig, F, Espígol-Frigolé, G, Schroeder, J, Kernder, A, Monti, S, Silvagni, E, Crimi, C, Cinetto, F, Fraticelli, P, Roccatello, D, Vacca, A, Mohammad, A, Hellmich, B, Samson, M, Bargagli, E, Cohen Tervaert, J, Ribi, C, Fiori, D, Bello, F, Fagni, F, Moroni, L, Ramirez, G, Nasser, M, Marvisi, C, Toniati, P, Firinu, D, Padoan, R, Egan, A, Seeliger, B, Iannone, F, Salvarani, C, Jayne, D, Prisco, D, Vaglio, A, Emmi, G, Bettiol, Alessandra, Urban, Maria Letizia, Dagna, Lorenzo, Cottin, Vincent, Franceschini, Franco, Del Giacco, Stefano, Schiavon, Franco, Neumann, Thomas, Lopalco, Giuseppe, Novikov, Pavel, Baldini, Chiara, Lombardi, Carlo, Berti, Alvise, Alberici, Federico, Folci, Marco, Negrini, Simone, Sinico, Renato Alberto, Quartuccio, Luca, Lunardi, Claudio, Parronchi, Paola, Moosig, Frank, Espígol-Frigolé, Georgina, Schroeder, Jan, Kernder, Anna Luise, Monti, Sara, Silvagni, Ettore, Crimi, Claudia, Cinetto, Francesco, Fraticelli, Paolo, Roccatello, Dario, Vacca, Angelo, Mohammad, Aladdin J, Hellmich, Bernhard, Samson, Maxime, Bargagli, Elena, Cohen Tervaert, Jan Willem, Ribi, Camillo, Fiori, Davide, Bello, Federica, Fagni, Filippo, Moroni, Luca, Ramirez, Giuseppe Alvise, Nasser, Mouhamad, Marvisi, Chiara, Toniati, Paola, Firinu, Davide, Padoan, Roberto, Egan, Allyson, Seeliger, Benjamin, Iannone, Florenzo, Salvarani, Carlo, Jayne, David, Prisco, Domenico, Vaglio, Augusto, Emmi, Giacomo, Bettiol, A, Urban, M, Dagna, L, Cottin, V, Franceschini, F, Del Giacco, S, Schiavon, F, Neumann, T, Lopalco, G, Novikov, P, Baldini, C, Lombardi, C, Berti, A, Alberici, F, Folci, M, Negrini, S, Sinico, R, Quartuccio, L, Lunardi, C, Parronchi, P, Moosig, F, Espígol-Frigolé, G, Schroeder, J, Kernder, A, Monti, S, Silvagni, E, Crimi, C, Cinetto, F, Fraticelli, P, Roccatello, D, Vacca, A, Mohammad, A, Hellmich, B, Samson, M, Bargagli, E, Cohen Tervaert, J, Ribi, C, Fiori, D, Bello, F, Fagni, F, Moroni, L, Ramirez, G, Nasser, M, Marvisi, C, Toniati, P, Firinu, D, Padoan, R, Egan, A, Seeliger, B, Iannone, F, Salvarani, C, Jayne, D, Prisco, D, Vaglio, A, Emmi, G, Bettiol, Alessandra, Urban, Maria Letizia, Dagna, Lorenzo, Cottin, Vincent, Franceschini, Franco, Del Giacco, Stefano, Schiavon, Franco, Neumann, Thomas, Lopalco, Giuseppe, Novikov, Pavel, Baldini, Chiara, Lombardi, Carlo, Berti, Alvise, Alberici, Federico, Folci, Marco, Negrini, Simone, Sinico, Renato Alberto, Quartuccio, Luca, Lunardi, Claudio, Parronchi, Paola, Moosig, Frank, Espígol-Frigolé, Georgina, Schroeder, Jan, Kernder, Anna Luise, Monti, Sara, Silvagni, Ettore, Crimi, Claudia, Cinetto, Francesco, Fraticelli, Paolo, Roccatello, Dario, Vacca, Angelo, Mohammad, Aladdin J, Hellmich, Bernhard, Samson, Maxime, Bargagli, Elena, Cohen Tervaert, Jan Willem, Ribi, Camillo, Fiori, Davide, Bello, Federica, Fagni, Filippo, Moroni, Luca, Ramirez, Giuseppe Alvise, Nasser, Mouhamad, Marvisi, Chiara, Toniati, Paola, Firinu, Davide, Padoan, Roberto, Egan, Allyson, Seeliger, Benjamin, Iannone, Florenzo, Salvarani, Carlo, Jayne, David, Prisco, Domenico, Vaglio, Augusto, and Emmi, Giacomo

Abstract

Objective: Mepolizumab proved efficacious for eosinophilic granulomatosis with polyangiitis (EGPA, former Churg-Strauss) at the dosage of 300mg/4 weeks in the randomized controlled MIRRA trial. Few successful real-life experiences with the dosage approved for severe eosinophilic asthma (100mg/4 weeks) were recently reported. We retrospectively assessed the effectiveness and safety of mepolizumab 100 and 300mg/4 weeks in a large European EGPA cohort. Methods: We included all EGPA patients treated with mepolizumab at the recruiting centres in 2015-2020. Treatment response was evaluated from month 3 through 24 (T3-T24) after mepolizumab starting. Complete response (CR) was defined as no disease activity (Birmingham Vasculitis Activity Score, BVAS=0) and a prednisone dose ≤4mg/day. Respiratory outcomes included asthma and ear-nose-throat (ENT) exacerbations. Results: We included 203 patients, of whom 191 at stable dosage (158 mepolizumab 100mg/4 weeks, 33 300mg/4 weeks). At T3, 25 patients (12.3%) had a CR. CR rates increased to 30.4% and 35.7% at T12 and T24 and were comparable between mepolizumab 100 and 300mg/4 weeks. Mepolizumab led to a significant reduction in BVAS, prednisone dose, eosinophil counts from T3 through T24, with no significant differences between 100 and 300 mg/4weeks. Eighty-two patients (40.4%) experienced asthma exacerbations [57/158 (36%) on 100mg/4 weeks; 17/33 (52%) on 300mg/4 weeks]. Thirty-one (15.3%) experienced ENT exacerbations. Forty-four patients (21.7%) experienced adverse events, most being non-serious (38/44). Conclusion: Mepolizumab both at 100 and 300mg/4 weeks is effective for EGPA. The two dosages should be compared in the setting of a controlled trial.

Published
2022

44. Mortality in Severe Antibody Deficiencies Patients during the First Two Years of the COVID-19 Pandemic: Vaccination and Monoclonal Antibodies Efficacy

Author

Milito, Cinzia, primary, Cinetto, Francesco, additional, Palladino, Andrea, additional, Garzi, Giulia, additional, Punziano, Alessandra, additional, Lagnese, Gianluca, additional, Scarpa, Riccardo, additional, Rattazzi, Marcello, additional, Pesce, Anna Maria, additional, Pulvirenti, Federica, additional, Di Napoli, Giulia, additional, Spadaro, Giuseppe, additional, Carsetti, Rita, additional, and Quinti, Isabella, additional

Published
2022
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45. Comorbidities of sarcoidosis

Author

Tana, Claudio, primary, Drent, Marjolein, additional, Nunes, Hilario, additional, Kouranos, Vasilis, additional, Cinetto, Francesco, additional, Jessurun, Naomi T., additional, and Spagnolo, Paolo, additional

Published
2022
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46. Impact of Hypogammaglobulinemia on the Course of COVID-19 in a Non-Intensive Care Setting: A Single-Center Retrospective Cohort Study

Author

Scarpa, Riccardo, primary, Dell’Edera, Alessandro, additional, Felice, Carla, additional, Buso, Roberta, additional, Muscianisi, Francesco, additional, Finco Gambier, Renato, additional, Toffolo, Sara, additional, Grossi, Ugo, additional, Giobbia, Mario, additional, Barberio, Giuseppina, additional, Landini, Nicholas, additional, Facchini, Cesarina, additional, Agostini, Carlo, additional, Rattazzi, Marcello, additional, and Cinetto, Francesco, additional

Published
2022
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47. Surgical Management of Hemorrhoidal Disease in Inflammatory Bowel Disease: A Systematic Review with Proportional Meta-Analysis

Author

Grossi, Ugo, primary, Gallo, Gaetano, additional, Di Tanna, Gian Luca, additional, Bracale, Umberto, additional, Ballo, Mattia, additional, Galasso, Elisa, additional, Kazemi Nava, Andrea, additional, Zucchella, Martino, additional, Cinetto, Francesco, additional, Rattazzi, Marcello, additional, Felice, Carla, additional, and Zanus, Giacomo, additional

Published
2022
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49. Comparison between Dexamethasone and Methylprednisolone Therapy in Patients with COVID-19 Pneumonia Admitted to Non-Intensive Medical Units

Author

Buso, Roberta, primary, Cinetto, Francesco, additional, Dell’Edera, Alessandro, additional, Veneran, Nicola, additional, Facchini, Cesarina, additional, Biscaro, Valeria, additional, Schiavon, Stefania, additional, Vian, Elisa, additional, Grossi, Ugo, additional, Zanus, Giacomo, additional, Giobbia, Mario, additional, Scarpa, Riccardo, additional, Agostini, Carlo, additional, Rattazzi, Marcello, additional, and Felice, Carla, additional

Published
2021
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50. The Effect of Isotonic Saline Nasal Lavages in Improving Symptoms in SARS-CoV-2 Infection: A Case-Control Study

Author

Spinato, Giacomo, primary, Fabbris, Cristoforo, additional, Costantini, Giulio, additional, Conte, Federica, additional, Scotton, Pier Giorgio, additional, Cinetto, Francesco, additional, De Siati, Rosalba, additional, Matarazzo, Alessandro, additional, Citterio, Marco, additional, Contro, Giacomo, additional, De Filippis, Cosimo, additional, Agostini, Carlo, additional, Emanuelli, Enzo, additional, Boscolo-Rizzo, Paolo, additional, and Frezza, Daniele, additional

Published
2021
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