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107 results on '"Marvisi, C"'

1. POS0115 EOSINOPHILIC GIANT CELL ARTERITIS: A DIFFERENT SUBSET OF DISEASE?

Author

Marvisi, C., primary, Boiardi, L., additional, Macchioni, P., additional, Cavazza, A., additional, Croci, S., additional, Besutti, G., additional, Spaggiari, L., additional, Giorgi Rossi, P., additional, Cimino, L., additional, Pipitone, N., additional, Ricordi, C., additional, Muratore, F., additional, and Salvarani, C., additional

Published
2024
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2. POS0932 AORTIC INVOLVEMENT IN GCA: A MULTICENTER RETROSPECTIVE STUDY

Author

Marvisi, C., primary, Guggenberger, K. V., additional, Ricordi, C., additional, Werner, R. A., additional, Besutti, G., additional, Serfling, S. E., additional, Farì, R., additional, Fröhlich, M., additional, Durmo, R., additional, Gernert, M., additional, Spaggiari, L., additional, Versari, A., additional, Pattacini, P., additional, Salvarani, C., additional, Schmalzing, M., additional, Muratore, F., additional, and Bley, T., additional

Published
2024
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4. AB1261 AVACOPAN IN THE TREATMENT OF ANCA-ASSOCIATED VASCULITIS: MULTICENTER PROSPECTIVE REAL-LIFE STUDY ON EFFICACY, SAFETY, AND IMPACT ON QUALITY OF LIFE

Author

Treppo, E., primary, De Martino, M., additional, Bello, F., additional, Padoan, R., additional, Monti, S., additional, Moroni, L., additional, Affatato, S., additional, Conticini, E., additional, Galli, E., additional, Marvisi, C., additional, Tombetti, E., additional, Frediani, B., additional, Alberici, F., additional, Dagna, L., additional, Delvino, P., additional, Schiavon, F., additional, Emmi, G., additional, Salvarani, C., additional, Isola, M., additional, and Quartuccio, L., additional

Published
2024
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9. Management of pregnancy in autoimmune rheumatic diseases: maternal disease course, gestational and neonatal outcomes and use of medications in the prospective Italian P-RHEUM.it study

Author

Andreoli, L, Gerardi, M, Gerosa, M, Rozza, D, Crisafulli, F, Erra, R, Lini, D, Trespidi, L, Padovan, M, Ruffilli, F, Serale, F, Cuomo, G, Raffeiner, B, Semeraro, P, Tani, C, Chimenti, M, Conigliaro, P, Hoxha, A, Nalli, C, Fredi, M, Lazzaroni, M, Filippini, M, Taglietti, M, Franceschini, F, Zatti, S, Loardi, C, Orabona, R, Ramazzotto, F, Zanardini, C, Fontana, G, Gozzoli, G, Barison, C, Bizioli, P, Caporali, R, Carrea, G, Ossola, M, Maranini, B, Silvagni, E, Govoni, M, Morano, D, Verteramo, R, Doria, A, Del Ross, T, Favaro, M, Calligaro, A, Tonello, M, Larosa, M, Zen, M, Zambon, A, Mosca, M, Zucchi, D, Elefante, E, Gori, S, Iannone, F, Anelli, M, Lavista, M, Abbruzzese, A, Fasano, C, D'Angelo, S, Cutro, M, Picerno, V, Carbone, T, Padula, A, Rovere-Querini, P, Canti, V, De Lorenzo, R, Cavallo, L, Ramoni, V, Montecucco, C, Codullo, V, Milanesi, A, Pazzola, G, Comitini, G, Marvisi, C, Salvarani, C, Epis, O, Benedetti, S, Di Raimondo, G, Gagliardi, C, Lomater, C, Crepaldi, G, Bellis, E, Bellisai, F, Garcia Gonzalez, E, Pata, A, Zerbinati, M, Urban, M, Mattioli, I, Iuliano, A, Sebastiani, G, Brucato, A, Bizzi, E, Cutolo, M, Santo, L, Tonetta, S, Landolfi, G, Carrara, G, Bortoluzzi, A, Scirè, C, Tincani, A, Andreoli, Laura, Gerardi, Maria Chiara, Gerosa, Maria, Rozza, Davide, Crisafulli, Francesca, Erra, Roberta, Lini, Daniele, Trespidi, Laura, Padovan, Melissa, Ruffilli, Francesca, Serale, Francesca, Cuomo, Giovanna, Raffeiner, Bernd, Semeraro, Paolo, Tani, Chiara, Chimenti, Maria Sole, Conigliaro, Paola, Hoxha, Ariela, Nalli, Cecilia, Fredi, Micaela, Lazzaroni, Maria Grazia, Filippini, Matteo, Taglietti, Marco, Franceschini, Franco, Zatti, Sonia, Loardi, Chiara, Orabona, Rossana, Ramazzotto, Francesca, Zanardini, Cristina, Fontana, Giulia, Gozzoli, Giorgia, Barison, Claudia, Bizioli, Paola, Caporali, Roberto Felice, Carrea, Giulia, Ossola, Manuela Wally, Maranini, Beatrice, Silvagni, Ettore, Govoni, Marcello, Morano, Danila, Verteramo, Rosita, Doria, Andrea, Del Ross, Teresa, Favaro, Maria, Calligaro, Antonia, Tonello, Marta, Larosa, Maddalena, Zen, Margherita, Zambon, Alessandra, Mosca, Marta, Zucchi, Dina, Elefante, Elena, Gori, Sabrina, Iannone, Florenzo, Anelli, Maria Grazia, Lavista, Marlea, Abbruzzese, Anna, Fasano, Carlo Giuseppe, D'Angelo, Salvatore, Cutro, Maria Stefania, Picerno, Valentina, Carbone, Teresa, Padula, Angela Anna, Rovere-Querini, Patrizia, Canti, Valentina, De Lorenzo, Rebecca, Cavallo, Ludovica, Ramoni, Véronique, Montecucco, Carlomaurizio, Codullo, Veronica, Milanesi, Alessandra, Pazzola, Giulia, Comitini, Giuseppina, Marvisi, Chiara, Salvarani, Carlo, Epis, Oscar Massimiliano, Benedetti, Sara, Di Raimondo, Giuseppina, Gagliardi, Clizia, Lomater, Claudia, Crepaldi, Gloria, Bellis, Elisa, Bellisai, Francesca, Garcia Gonzalez, Estrella, Pata, Anna Paola, Zerbinati, Martina, Urban, Maria Letizia, Mattioli, Irene, Iuliano, Annamaria, Sebastiani, Giandomenico, Brucato, Antonio Luca, Bizzi, Emanuele, Cutolo, Maurizio, Santo, Leonardo, Tonetta, Sara, Landolfi, Gianpiero, Carrara, Greta, Bortoluzzi, Alessandra, Scirè, Carlo Alberto, Tincani, Angela, Andreoli, L, Gerardi, M, Gerosa, M, Rozza, D, Crisafulli, F, Erra, R, Lini, D, Trespidi, L, Padovan, M, Ruffilli, F, Serale, F, Cuomo, G, Raffeiner, B, Semeraro, P, Tani, C, Chimenti, M, Conigliaro, P, Hoxha, A, Nalli, C, Fredi, M, Lazzaroni, M, Filippini, M, Taglietti, M, Franceschini, F, Zatti, S, Loardi, C, Orabona, R, Ramazzotto, F, Zanardini, C, Fontana, G, Gozzoli, G, Barison, C, Bizioli, P, Caporali, R, Carrea, G, Ossola, M, Maranini, B, Silvagni, E, Govoni, M, Morano, D, Verteramo, R, Doria, A, Del Ross, T, Favaro, M, Calligaro, A, Tonello, M, Larosa, M, Zen, M, Zambon, A, Mosca, M, Zucchi, D, Elefante, E, Gori, S, Iannone, F, Anelli, M, Lavista, M, Abbruzzese, A, Fasano, C, D'Angelo, S, Cutro, M, Picerno, V, Carbone, T, Padula, A, Rovere-Querini, P, Canti, V, De Lorenzo, R, Cavallo, L, Ramoni, V, Montecucco, C, Codullo, V, Milanesi, A, Pazzola, G, Comitini, G, Marvisi, C, Salvarani, C, Epis, O, Benedetti, S, Di Raimondo, G, Gagliardi, C, Lomater, C, Crepaldi, G, Bellis, E, Bellisai, F, Garcia Gonzalez, E, Pata, A, Zerbinati, M, Urban, M, Mattioli, I, Iuliano, A, Sebastiani, G, Brucato, A, Bizzi, E, Cutolo, M, Santo, L, Tonetta, S, Landolfi, G, Carrara, G, Bortoluzzi, A, Scirè, C, Tincani, A, Andreoli, Laura, Gerardi, Maria Chiara, Gerosa, Maria, Rozza, Davide, Crisafulli, Francesca, Erra, Roberta, Lini, Daniele, Trespidi, Laura, Padovan, Melissa, Ruffilli, Francesca, Serale, Francesca, Cuomo, Giovanna, Raffeiner, Bernd, Semeraro, Paolo, Tani, Chiara, Chimenti, Maria Sole, Conigliaro, Paola, Hoxha, Ariela, Nalli, Cecilia, Fredi, Micaela, Lazzaroni, Maria Grazia, Filippini, Matteo, Taglietti, Marco, Franceschini, Franco, Zatti, Sonia, Loardi, Chiara, Orabona, Rossana, Ramazzotto, Francesca, Zanardini, Cristina, Fontana, Giulia, Gozzoli, Giorgia, Barison, Claudia, Bizioli, Paola, Caporali, Roberto Felice, Carrea, Giulia, Ossola, Manuela Wally, Maranini, Beatrice, Silvagni, Ettore, Govoni, Marcello, Morano, Danila, Verteramo, Rosita, Doria, Andrea, Del Ross, Teresa, Favaro, Maria, Calligaro, Antonia, Tonello, Marta, Larosa, Maddalena, Zen, Margherita, Zambon, Alessandra, Mosca, Marta, Zucchi, Dina, Elefante, Elena, Gori, Sabrina, Iannone, Florenzo, Anelli, Maria Grazia, Lavista, Marlea, Abbruzzese, Anna, Fasano, Carlo Giuseppe, D'Angelo, Salvatore, Cutro, Maria Stefania, Picerno, Valentina, Carbone, Teresa, Padula, Angela Anna, Rovere-Querini, Patrizia, Canti, Valentina, De Lorenzo, Rebecca, Cavallo, Ludovica, Ramoni, Véronique, Montecucco, Carlomaurizio, Codullo, Veronica, Milanesi, Alessandra, Pazzola, Giulia, Comitini, Giuseppina, Marvisi, Chiara, Salvarani, Carlo, Epis, Oscar Massimiliano, Benedetti, Sara, Di Raimondo, Giuseppina, Gagliardi, Clizia, Lomater, Claudia, Crepaldi, Gloria, Bellis, Elisa, Bellisai, Francesca, Garcia Gonzalez, Estrella, Pata, Anna Paola, Zerbinati, Martina, Urban, Maria Letizia, Mattioli, Irene, Iuliano, Annamaria, Sebastiani, Giandomenico, Brucato, Antonio Luca, Bizzi, Emanuele, Cutolo, Maurizio, Santo, Leonardo, Tonetta, Sara, Landolfi, Gianpiero, Carrara, Greta, Bortoluzzi, Alessandra, Scirè, Carlo Alberto, and Tincani, Angela

Abstract

Objectives To investigate pregnancy outcomes in women with autoimmune rheumatic diseases (ARD) in the Italian prospective cohort study P-RHEUM.it. Methods Pregnant women with different ARD were enrolled for up to 20 gestational weeks in 29 Rheumatology Centres for 5 years (2018-2023). Maternal and infant information were collected in a web-based database. Results We analysed 866 pregnancies in 851 patients (systemic lupus erythematosus was the most represented disease, 19.6%). Maternal disease flares were observed in 135 (15.6%) pregnancies. 53 (6.1%) pregnancies were induced by assisted reproduction techniques, 61 (7%) ended in miscarriage and 11 (1.3%) underwent elective termination. Obstetrical complications occurred in 261 (30.1%) pregnancies, including 2.3% pre-eclampsia. Two cases of congenital heart block were observed out of 157 pregnancies (1.3%) with anti-Ro/SSA. Regarding treatments, 244 (28.2%) pregnancies were treated with glucocorticoids, 388 (44.8%) with hydroxychloroquine, 85 (9.8%) with conventional synthetic disease-modifying anti-rheumatic drugs and 122 (14.1%) with biological disease-modifying anti-rheumatic drugs. Live births were 794 (91.7%), mostly at term (84.9%); four perinatal deaths (0.5%) occurred. Among 790 newborns, 31 (3.9%) were small-for-gestational-age and 169 (21.4%) had perinatal complications. Exclusive maternal breast feeding was received by 404 (46.7%) neonates. The Edinburgh Postnatal Depression Scale was compiled by 414 women (52.4%); 89 (21.5%) scored positive for emotional distress. Conclusions Multiple factors including preconception counselling and treat-to-target with pregnancy-compatible medications may have contributed to mitigate disease-related risk factors, yielding limited disease flares, good pregnancy outcomes and frequency of complications which were similar to the Italian general obstetric population. Disease-specific issues need to be further addressed to plan preventative measures.

Published
2024

10. Systemic vasculitis: one year in review 2024

Author

Treppo, E, Monti, S, Delvino, P, Marvisi, C, Ricordi, C, La Rocca, G, Moretti, M, Italiano, N, Di Cianni, F, Ferro, F, Muratore, F, Baldini, C, Talarico, R, Quartuccio, L, Salvarani, C, Treppo, Elena, Monti, Sara, Delvino, Paolo, Marvisi, Chiara, Ricordi, Caterina, La Rocca, Gaetano, Moretti, Michele, Italiano, Nazzareno, Di Cianni, Federica, Ferro, Francesco, Muratore, Francesco, Baldini, Chiara, Talarico, Rosaria, Quartuccio, Luca, Salvarani, Carlo, Treppo, E, Monti, S, Delvino, P, Marvisi, C, Ricordi, C, La Rocca, G, Moretti, M, Italiano, N, Di Cianni, F, Ferro, F, Muratore, F, Baldini, C, Talarico, R, Quartuccio, L, Salvarani, C, Treppo, Elena, Monti, Sara, Delvino, Paolo, Marvisi, Chiara, Ricordi, Caterina, La Rocca, Gaetano, Moretti, Michele, Italiano, Nazzareno, Di Cianni, Federica, Ferro, Francesco, Muratore, Francesco, Baldini, Chiara, Talarico, Rosaria, Quartuccio, Luca, and Salvarani, Carlo

Abstract

Systemic vasculitides comprise a collection of rare and heterogeneous disorders capable of impacting any organ and system, posing a considerable burden of mortality and comorbidity. As with previous annual reviews of this series, this review will offer a critical overview of the latest literature on pathogenesis, biomarkers, and treatment options in both small- and large-vessel vasculitis.

Published
2024

12. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis

Author

Emmi, G, Bettiol, A, Gelain, E, Bajema, I, Berti, A, Burns, S, Cid, M, Cohen Tervaert, J, Cottin, V, Durante, E, Holle, J, Mahr, A, Del Pero, M, Marvisi, C, Mills, J, Moiseev, S, Moosig, F, Mukhtyar, C, Neumann, T, Olivotto, I, Salvarani, C, Seeliger, B, Sinico, R, Taillé, C, Terrier, B, Venhoff, N, Bertsias, G, Guillevin, L, Jayne, D, Vaglio, A, Emmi G., Bettiol A., Gelain E., Bajema I. M., Berti A., Burns S., Cid M. C., Cohen Tervaert J. W., Cottin V., Durante E., Holle J. U., Mahr A. D., Del Pero M. M., Marvisi C., Mills J., Moiseev S., Moosig F., Mukhtyar C., Neumann T., Olivotto I., Salvarani C., Seeliger B., Sinico R. A., Taillé C., Terrier B., Venhoff N., Bertsias G., Guillevin L., Jayne D. R. W., Vaglio A., Emmi, G, Bettiol, A, Gelain, E, Bajema, I, Berti, A, Burns, S, Cid, M, Cohen Tervaert, J, Cottin, V, Durante, E, Holle, J, Mahr, A, Del Pero, M, Marvisi, C, Mills, J, Moiseev, S, Moosig, F, Mukhtyar, C, Neumann, T, Olivotto, I, Salvarani, C, Seeliger, B, Sinico, R, Taillé, C, Terrier, B, Venhoff, N, Bertsias, G, Guillevin, L, Jayne, D, Vaglio, A, Emmi G., Bettiol A., Gelain E., Bajema I. M., Berti A., Burns S., Cid M. C., Cohen Tervaert J. W., Cottin V., Durante E., Holle J. U., Mahr A. D., Del Pero M. M., Marvisi C., Mills J., Moiseev S., Moosig F., Mukhtyar C., Neumann T., Olivotto I., Salvarani C., Seeliger B., Sinico R. A., Taillé C., Terrier B., Venhoff N., Bertsias G., Guillevin L., Jayne D. R. W., and Vaglio A.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach. Current practice relies on recommendations and guidelines addressing the management of ANCA-associated vasculitis and not specifically developed for EGPA. Here, we present evidence-based, cross-discipline guidelines for the diagnosis and management of EGPA that reflect the substantial advances that have been made in the past few years in understanding the pathogenesis, clinical subphenotypes and differential diagnosis of the disease, as well as the availability of new treatment options. Developed by a panel of European experts on the basis of literature reviews and, where appropriate, expert opinion, the 16 statements and five overarching principles cover the diagnosis and staging, treatment, outcome and follow-up of EGPA. These recommendations are primarily intended to be used by healthcare professionals, pharmaceutical industries and drug regulatory authorities, to guide clinical practice and decision-making in EGPA. These guidelines are not intended to limit access to medications by healthcare agencies, nor to impose a fixed order on medication use.

Published
2023

14. AB0771 FEMALE GENDER AND STEROID TREATMENT AS MAIN INFLUENCING FACTORS IN THE PERCEPTION OF QUALITY OF LIFE IN ANCA-ASSOCIATED VASCULITIS: FINAL RESULTS FROM THE ITALIAN VERSION OF ANCA-ASSOCIATED VASCULITIS PATIENT-REPORTED OUTCOME (AAV-PRO_ITA) QUESTIONNAIRE

Author

Treppo, E., primary, Isola, M., additional, De Martino, M., additional, Padoan, R., additional, Urban, M. L., additional, Monti, S., additional, Sartorelli, S., additional, Giollo, A., additional, Argolini, L. M., additional, Marvisi, C., additional, Gattamelata, A., additional, Regola, F., additional, Ferro, F., additional, Cassone, G., additional, Motta, F., additional, Berti, A., additional, Conticini, E., additional, Guiducci, S., additional, Matucci-Cerinic, M., additional, Lo Gullo, A., additional, Manfredi, A., additional, Frediani, B., additional, Bortolotti, R., additional, Selmi, C., additional, Baldini, C., additional, Franceschini, F., additional, Conti, F., additional, Emmi, G., additional, Caporali, R., additional, Rossini, M., additional, Dagna, L., additional, Montecucco, C., additional, Schiavon, F., additional, Salvarani, C., additional, De Vita, S., additional, and Quartuccio, L., additional

Published
2023
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15. POS0722 EFFECTS ON SOLUBLE IMMUNE CHECKPOINTS INDUCED BY TOCILIZUMAB MONOTHERAPY AFTER ULTRA-SHORT-TERM GLUCOCORTICOIDS IN LARGE VESSEL – GIANT CELL ARTERITIS PATIENTS

Author

Bonacini, M., primary, Rossi, A., additional, Ferrigno, I., additional, Catellani, C., additional, Buia, V., additional, Muratore, F., additional, Galli, E., additional, Marvisi, C., additional, Cassone, G., additional, Zerbini, A., additional, Salvarani, C., additional, and Croci, S., additional

Published
2023
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16. POS1176 HOSPITALIZATION RATES, FEATURES, AND DISCHARGE DIAGNOSES OF A LARGE NATIONWIDE COHORT OF ANCA-ASSOCIATED VASCULITIS

Author

Berti, A., primary, Ottone, M., additional, Sartorelli, S., additional, Treppo, E., additional, Bettiol, A., additional, Padoan, R., additional, Regola, F., additional, Monti, S., additional, Marvisi, C., additional, Giollo, A., additional, Argolini, L. M., additional, Righini, M., additional, Gattamelata, A., additional, Cassone, G., additional, Sottini, L., additional, Maule, M., additional, Toniati, P., additional, Palermo, B. L., additional, Bello, F., additional, Guella, S., additional, Izzo, R., additional, Muratore, F., additional, Catanoso, M. G., additional, Fassio, A., additional, Cataleta, P., additional, Buscaroli, A., additional, Giorgi Rossi, P., additional, Franceschini, F., additional, Caporali, R., additional, Montecucco, C., additional, Conti, F., additional, Emmi, G., additional, Quartuccio, L., additional, Paolazzi, G., additional, Dagna, L., additional, Schiavon, F., additional, Salvarani, C., additional, and Bortolotti, R., additional

Published
2023
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18. POS0711 DEVELOPMENT OF THE TAKAYASU’S ARTERITIS INTEGRATED DISEASE ACTIVITY INDEX

Author

Marvisi, C., primary, Bolek, E. C., additional, Ahlman, M., additional, Alessi, H., additional, Galli, E., additional, Ricordi, C., additional, Muratore, F., additional, Kaymaz Tahra, S., additional, Alibaz-Oner, F., additional, Özgüven, S., additional, Merkel, P. A., additional, Salvarani, C., additional, Direskeneli, H., additional, Quinn, K. A., additional, and Grayson, P., additional

Published
2023
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19. POS0728 EFFECTIVENESS AND SAFETY OF A 26 WEEK TAPER REGIMEN OF GLUCOCORTICOID IN NEWLY-DIAGNOSED GCA PATIENTS: A REAL LIFE EXPERIENCE

Author

Marvisi, C., primary, Castrignanò, P., additional, Muratore, F., additional, Galli, E., additional, Croci, S., additional, Bonacini, M., additional, Boiardi, L., additional, Besutti, G., additional, Spaggiari, L., additional, Versari, A., additional, Casali, M., additional, Pipitone, N., additional, and Salvarani, C., additional

Published
2023
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20. AB0723 TAKAYASU ARTERITIS AND LARGE-VESSEL GIANT CELL ARTERITIS IN ITALIAN POPULATION. A RETROSPECTIVE COHORT STUDY

Author

Boiardi, L., primary, Macchioni, P., additional, Galli, E., additional, Muratore, F., additional, Csaali, M., additional, Besutti, G., additional, Spaggiari, L., additional, Versari, A., additional, Croci, S., additional, Mancuso, P., additional, Giorgi Rossi, P., additional, Marvisi, C., additional, Gene, G., additional, and Salvarani, C., additional

Published
2023
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21. POS0696 TREATMENT OF GIANT CELL ARTERITIS PATIENTS WITH ULTRA-SHORT GLUCOCORTICOIDS AND TOCILIZUMAB: ROLE OF IMAGING IN A PROSPECTIVE STUDY

Author

Muratore, F., primary, Cassone, G., additional, Marvisi, C., additional, Galli, E., additional, Boiardi, L., additional, Besutti, G., additional, Spaggiari, L., additional, Casali, M., additional, Mancuso, P., additional, Versari, A., additional, Giorgi Rossi, P., additional, and Salvarani, C., additional

Published
2023
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22. Mepolizumab for Eosinophilic Granulomatosis With Polyangiitis: A European Multicenter Observational Study

Author

Bettiol, A., Urban, M. L., 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. A., Quartuccio, L., Lunardi, C., Parronchi, P., Moosig, F., Espigol-Frigole, G., Schroeder, J., Kernder, A. L., Monti, S., Silvagni, E., Crimi, C., Cinetto, F., Fraticelli, P., Roccatello, D., Vacca, A., Mohammad, A. J., Hellmich, B., Samson, M., Bargagli, E., Cohen Tervaert, J. W., Ribi, C., Fiori, D., Bello, F., Fagni, F., Moroni, L., Ramirez, G. A., 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., Ahmad, K., Beccalli, M., Bonnotte, B., Bortolotti, R., Cariddi, A., Caminati, M., Cid, M. C., Deidda, M., Delvino, P., Scala, G. D., Felicetti, M., Ferro, F., Furini, F., Gelain, E., Ghirelli, G., Holle, J., Losappio, L. M., Mahr, A., Malandrino, D., Marhhold, J., Mattioli, I., Moi, L., Moiseev, S., Muratore, F., Nolasco, S., Olivieri, B., Palermo, A., Regola, F., Sander, O., Scarpa, R., Sciascia, S., Silvestri, E., Susca, N., Terrier, B., Treppo, E., Trezzi, B., Uzzo, M., Vitiello, G., Yacyshyn, E., RS: MHeNs - R3 - Neuroscience, Faculteit FHML Centraal, 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, Jayne, David [0000-0002-1712-0637], and Apollo - University of Cambridge Repository

Subjects
Male, Epidemiology, Birmingham Vasculitis Activity Score, law.invention, Glucocorticoid, Randomized controlled trial, Prednisone, law, Eosinophilic, Monoclonal, Immunology and Allergy, Humanized, PLACEBO, Middle Aged, egpa mepolizumab, Treatment Outcome, SAFETY, Female, ANCA-associated Vasculitis, Biologicals, Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss), Glucocorticoids, Granulomatosis with polyangiitis, ANCA-associated Vasculiti, medicine.drug, Keywords: ANCA-associated Vasculitis, Adult, medicine.medical_specialty, Immunology, Antibodies, Monoclonal, Humanized, Antibodies, Drug Administration Schedule, Eosinophilia, Granulomatosis with Polyangiitis, Humans, Retrospective Studies, Rheumatology, Internal medicine, medicine, Adverse effect, Asthma, business.industry, medicine.disease, Biological, EGPA, European EGPA Study Group, business, FOLLOW-UP, Mepolizumab
Abstract

OBJECTIVE: Mepolizumab proved to be an efficacious treatment for eosinophilic granulomatosis with polyangiitis (EGPA) at a dose of 300 mg every 4 weeks in the randomized, controlled MIRRA trial. In a few recently reported studies, successful real-life experiences with the approved dose for treating severe eosinophilic asthma (100 mg every 4 weeks) were observed. We undertook this study to assess the effectiveness and safety of mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks in a large European EGPA cohort. METHODS: We included all patients with EGPA treated with mepolizumab at the recruiting centers in 2015-2020. Treatment response was evaluated from 3 months to 24 months after initiation of mepolizumab. Complete response to treatment was defined as no disease activity (Birmingham Vasculitis Activity Score [BVAS] = 0) and a prednisolone or prednisone dose (or equivalent) of ���4 mg/day. Respiratory outcomes included asthma and ear, nose, and throat (ENT) exacerbations. RESULTS: Two hundred three patients, of whom 191 received a stable dose of mepolizumab (158 received 100 mg every 4 weeks and 33 received 300 mg every 4 weeks) were included. Twenty-five patients (12.3%) had a complete response to treatment at 3 months. Complete response rates increased to 30.4% and 35.7% at 12 months and 24 months, respectively, and rates were comparable between mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks. Mepolizumab led to a significant reduction in BVAS score, prednisone dose, and eosinophil counts from 3 months to 24 months, with no significant differences observed between 100 mg every 4 weeks and 300 mg every 4 weeks. Eighty-two patients (40.4%) experienced asthma exacerbations (57 of 158 [36%] who received 100 mg every 4 weeks; 17 of 33 [52%] who received 300 mg every 4 weeks), and 31 patients (15.3%) experienced ENT exacerbations. Forty-four patients (21.7%) experienced adverse events (AEs), most of which were nonserious AEs (38 of 44). CONCLUSION: Mepolizumab at both 100 mg every 4 weeks and 300 mg every 4 weeks is effective for the treatment of EGPA. The 2 doses should be compared in the setting of a controlled trial.

Published
2022
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23. Émulation d’un essai clinique comparant les corticoïdes versus les corticoïdes plus le cyclophosphamide au cours de la granulomatose éosinophilique avec polyangéite sévère

Author

Sorin, B., Papo, M., Sinico, R.A., Teixeira, V., Venhoff, N., Urban, M.L., Mahrhold, J., Locatelli, F., Cassone, G., Schiavon, F., Seeliger, B., Neumann, T., Kroegel, C., Groh, M., Marvisi, C., Samson, M., Barba, T., Jayne, D., Troilo, A., Thiel, J., Hellmich, B., Monti, S., Montecucco, C., Salvarani, C., Kahn, J.E., Durel, C.A., Mouthon, L., Guillevin, L., Giacomo, E., Vaglio, A., Porcher, R., and Terrier, B.

Published
2024
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24. 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

25. AB0627 Evaluation of internal consistency, feasibility, and reliability of the Italian version of ANCA-associated vasculitis patient-reported outcome (AAV-PRO_ita) questionnaire: preliminary results from a multicenter study on a large cohort of Italian patients

Author

Treppo, E., primary, Isola, M., additional, De Martino, M., additional, Padoan, R., additional, Urban, M. L., additional, Monti, S., additional, Sartorelli, S., additional, Giollo, A., additional, Argolini, L. M., additional, Marvisi, C., additional, Ferro, F., additional, Cassone, G., additional, Motta, F., additional, Berti, A., additional, Conticini, E., additional, Manfredi, A., additional, Frediani, B., additional, Bortolotti, R., additional, Selmi, C., additional, Baldini, C., additional, Emmi, G., additional, Caporali, R., additional, Rossini, M., additional, Dagna, L., additional, Montecucco, C., additional, Schiavon, F., additional, Salvarani, C., additional, De Vita, S., additional, and Quartuccio, L., additional

Published
2022
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26. EVALUATION OF INTERNAL CONSISTENCY, FEASIBILITY, AND RELIABILITY OF THE ITALIAN VERSION OF ANCA-ASSOCIATED VASCULITIS PATIENT-REPORTED OUTCOME (AAV-PRO_ITA) QUESTIONNAIRE: PRELIMINARY RESULTS FROM A MULTICENTER STUDY ON A LARGE COHORT OF ITALIAN PATIENTS

Author

Treppo, E, Isola, M, De Martino, M, Padoan, R, Urban, Ml, Monti, S, Sartorelli, S, Giollo, A, Argolini, Lm, Marvisi, C, Ferro, F, Cassone, G, Motta, F, Berti, A, Conticini, E, Manfredi, A, Frediani, B, Bortolotti, R, Selmi, C, Baldini, C, Emmi, G, Caporali, R, Rossini, M, Dagna, L, Montecucco, C, Schiavon, F, Salvarani, C, De Vita, S, and Quartuccio, L

Published
2022

27. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease

Author

Wallace, Zachary S, Naden, Ray P, Chari, Suresh, Choi, Hyon K, Della-Torre, Emanuel, Dicaire, Jean-Francois, Hart, Phillip A, Inoue, Dai, Kawano, Mitsuhiro, Khosroshahi, Arezou, Lanzillotta, Marco, Okazaki, Kazuichi, Perugino, Cory A, Sharma, Amita, Saeki, Takako, Schleinitz, Nicolas, Takahashi, Naoki, Umehara, Hisanori, Zen, Yoh, Stone, Collaborators: Akamizu T, John H., Akiyama, M, Barra, L, Bateman, A, Blockmans, D, Brito-Zeron, P, Campochiaro, C, Carruthers, M, Chiba, T, Cornell, L, Culver, E, Darabian, S, Deshpande, V, Dong, L, Ebbo, M, Fernández-Codina, A, Ferry, Ja, Fragkoulis, G, Frost, F, Frulloni, Luca, Hernandez-Molina, G, Ji, H, Keat, K, Kamisawa, T, Kawa, S, Kobayashi, H, Kodama, Y, Kubo, S, Kubota, K, Leng, H, Lerch, Mm, Liu, Y, Liu, Z, Löhr, M, Martin-Nares, E, Martinez-Valle, F, Marvisi, C, Masaki, Y, Matsui, S, Mizushima, I, Nakamura, S, Nordeide, J, Notohara, K, Paira, S, Popovic, J, Ramos-Casals, M, Rosenbaum, J, Ryu, J, Sato, Y, Sekiguchi, H, Sokol, Ev, Stone, Jr, Sun, W, Takahashi, H, Takahira, M, Tanaka, Y, Vaglio, A, Villamil, A, Wada, Y, Webster, G, Yamada, K, Yamamoto, M, Yi, J, Yi, Y, Zamboni, G, Zhang, W., Wallace, Z, Naden, Rp, Chari, S, Choi, Hk, DELLA TORRE, E, Dicaire, Jf, Hart, Pa, Inoue, D, Kawano, M, Khosroshahi, A, Lanzillotta, M, Okazaki, K, Perugino, Ca, Sharma, A, Saeki, T, Schleinitz, N, Takahashi, N, Umehara, H, Zen, Y, Stone, Jh, and Members of the ACR/EULAR IgG4-RD Classification Criteria Working, Group.

Subjects
rheumatoid arthritis, Adult, Male, 0301 basic medicine, medicine.medical_specialty, Immunology, Sensitivity and Specificity, General Biochemistry, Genetics and Molecular Biology, Diagnosis, Differential, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal medicine, parasitic diseases, Epidemiology, Humans, Immunology and Allergy, Medicine, Pathological, Sjøgren's syndrome, Aged, 030203 arthritis & rheumatology, business.industry, Middle Aged, inflammation, medicine.disease, Test (assessment), 030104 developmental biology, Rheumatoid arthritis, Radiological weapon, Female, Immunoglobulin G4-Related Disease, business, Rheumatism, Decision analysis
Abstract

IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serological, radiological and pathological data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises; existing literature; derivation and validation cohorts of 1879 subjects (1086 cases, 793 mimickers); and multicriterion decision analysis to identify, weight and test potential classification criteria. Two independent validation cohorts were included. A three-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least one of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serological, radiological and pathological items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, eight weighted inclusion criteria domains, addressing clinical findings, serological results, radiological assessments and pathological interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% CI 97.2% to 99.8%) and a sensitivity of 85.5% (95% CI 81.9% to 88.5%). In the second, the specificity was 97.8% (95% CI 93.7% to 99.2%) and the sensitivity was 82.0% (95% CI 77.0% to 86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiological and basic science investigations.

Published
2019

28. Risk of acute arterial and venous thromboembolic events in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Author

Bettiol, A., Sinico, R. A., Schiavon, F., Monti, S., Bozzolo, E. P., Franceschini, F., Govoni, M., Lunardi, C., Guida, G., Lopalco, G., Paolazzi, G., Vacca, A., Gregorini, G., Leccese, P., Piga, M., Conti, F., Fraticelli, P., Quartuccio, L., Alberici, F., Salvarani, C., Bettio, S., Negrini, S., Selmi, C., Sciascia, S., Moroni, G., Colla, L., Manno, C., Urban, M. L., Vannacci, A., Pozzi, M. R., Fabbrini, P., Polti, S., Felicetti, M., Marchi, M. R., Padoan, R., Delvino, P., Caporali, R., Montecucco, C., Dagna, L., Cariddi, A., Toniati, P., Tamanini, S., Furini, F., Bortoluzzi, A., Tinazzi, E., Delfino, L., Badiu, I., Rolla, G., Venerito, V., Iannone, F., Berti, A., Bortolotti, R., Racanelli, V., Jeannin, G., Padula, A., Cauli, A., Priori, R., Gabrielli, A., Bond, M., Tedesco, M., Pazzola, G., Tomietto, P., Pellecchio, M., Marvisi, C., Maritati, F., Palmisano, A., Dejaco, C., Willeit, J., Kiechl, S., Olivotto, I., Willeit, P., Prisco, D., Vaglio, A., Emmi, G., Bargagli, E., Becatti, M., Beccalli, M., Bello, F., Bozzao, F., Canti, V., Cassia, M. A., Cassone, G., Catanoso, M., Chieco-Bianchi, F., Clari, R., Coladonato, L., De Santis, M., Di Scala, G., Fagni, F., Fenaroli, P., Fiorillo, C., Floris, A., Fornaro, M., Galli, E., Generali, E., Giliberti, M., Lascaro, N., Leccese, I., Mattioli, I., Olivieri, B., Osti, N., Peyronel, F., Radin, M., Righetti, G., Salvati, S., Silvestri, E., Susca, N., Tamburini, C., Taurisano, G., Trezzi, B., Trivioli, G., Bettiol, A, Sinico, R, Schiavon, F, Monti, S, Bozzolo, E, Franceschini, F, Govoni, M, Lunardi, C, Guida, G, Lopalco, G, Paolazzi, G, Vacca, A, Gregorini, G, Leccese, P, Piga, M, Conti, F, Fraticelli, P, Quartuccio, L, Alberici, F, Salvarani, C, Bettio, S, Negrini, S, Selmi, C, Sciascia, S, Moroni, G, Colla, L, Manno, C, Urban, M, Vannacci, A, Pozzi, M, Fabbrini, P, Polti, S, Felicetti, M, Marchi, M, Padoan, R, Delvino, P, Caporali, R, Montecucco, C, Dagna, L, Cariddi, A, Toniati, P, Tamanini, S, Furini, F, Bortoluzzi, A, Tinazzi, E, Delfino, L, Badiu, I, Rolla, G, Venerito, V, Iannone, F, Berti, A, Bortolotti, R, Racanelli, V, Jeannin, G, Padula, A, Cauli, A, Priori, R, Gabrielli, A, Bond, M, Tedesco, M, Pazzola, G, Tomietto, P, Pellecchio, M, Marvisi, C, Maritati, F, Palmisano, A, Dejaco, C, Willeit, J, Kiechl, S, Olivotto, I, Willeit, P, Prisco, D, Vaglio, A, and Emmi, G

Subjects
Pulmonary and Respiratory Medicine, Burden of disease, Humans, Churg-Strauss Syndrome, Granulomatosis with Polyangiitis, Venous Thromboembolism, Venous Thrombosis, Churg-strauss syndrome, Criminology, NO, 03 medical and health sciences, 0302 clinical medicine, Medicine, 030212 general & internal medicine, Vascular inflammation, business.industry, Conflict of interest, Cytoplasmic antibody, medicine.disease, 030228 respiratory system, Wegener granulomatosis, arterial and venous thromboembolic events, Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome), Organ involvement, business, Production team
Abstract

Eosinophilic Granulomatosis with Polyangiitis (EGPA, Churg-Strauss syndrome) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) characterised by respiratory manifestations and systemic organ involvement [1]. Particularly, cardiac manifestations occur in 40–60% of patients, representing the leading cause of mortality [2]. Recent reports suggest that venous thromboembolic events might also represent a consistent burden of disease [3, 4], as already known for the other AAVs [5–7], possibly due to eosinophil-mediated vascular inflammation [5]. Nevertheless, the occurrence of arterial and venous thrombotic events (AVTE) has never been systematically explored in EGPA. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Alessandra Bettiol Conflict of interest: Renato Alberto Sinico Conflict of interest: Franco Schiavon Conflict of interest: Sara Monti Conflict of interest: Enrica Paola Bozzolo Conflict of interest: Franco Franceschini Conflict of interest: Marcello Govoni Conflict of interest: Claudio Lunardi Conflict of interest: Giuseppe Guida Conflict of interest: Giuseppe Lopalco Conflict of interest: Giuseppe Paolazzi Conflict of interest: Angelo Vacca Conflict of interest: Gina Gregorini Conflict of interest: Pietro Leccese Conflict of interest: Matteo Piga Conflict of interest: Fabrizio Conti Conflict of interest: Paolo Fraticelli Conflict of interest: Luca Quartuccio Conflict of interest: Federico Alberici Conflict of interest: Carlo Salvarani Conflict of interest: Silvano Bettio Conflict of interest: Simone Negrini Conflict of interest: Carlo Selmi Conflict of interest: Savino Sciascia Conflict of interest: Gabriella Moroni Conflict of interest: Loredana Colla Conflict of interest: Carlo Manno Conflict of interest: Maria Letizia Urban Conflict of interest: Alfredo Vannacci Conflict of interest: Maria Rosa Pozzi Conflict of interest: Paolo Fabbrini Conflict of interest: Stefano Polti Conflict of interest: Mara Felicetti Conflict of interest: Maria Rita Marchi Conflict of interest: Roberto Padoan Conflict of interest: Paolo Delvino Conflict of interest: Roberto Caporali Conflict of interest: Carlomaurizio Montecucco Conflict of interest: Lorenzo Dagna Conflict of interest: Adriana Cariddi Conflict of interest: Paola Toniati Conflict of interest: Dr. Tamanini reports other from Glaxo Smith Kline, outside the submitted work. Conflict of interest: Federica Furini Conflict of interest: Alessandra Bortoluzzi Conflict of interest: Elisa Tinazzi Conflict of interest: Lorenzo Delfino Conflict of interest: Iuliana Badiu Conflict of interest: Giovanni Rolla Conflict of interest: Vincenzo Venerito Conflict of interest: Florenzo Iannone Conflict of interest: Alvise Berti Conflict of interest: Roberto Bortolotti Conflict of interest: Vito Racanelli Conflict of interest: Guido Jeannin Conflict of interest: Angela Padula Conflict of interest: Alberto Cauli Conflict of interest: Roberta Priori Conflict of interest: Armando Gabrielli Conflict of interest: Milena Bond Conflict of interest: Martina Tedesco Conflict of interest: Giulia Pazzola Conflict of interest: Paola Tomietto Conflict of interest: Marco Pellecchio Conflict of interest: Chiara Marvisi Conflict of interest: Federica Maritati Conflict of interest: Alessandra Palmisano Conflict of interest: Christian Dejaco Conflict of interest: Johann Willeit Conflict of interest: Stefan Kiechl Conflict of interest: Iacopo Olivotto Conflict of interest: Peter Willeit Conflict of interest: Domenico Prisco Conflict of interest: Augusto Vaglio Conflict of interest: Giacomo Emmi

Published
2020

29. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, P. A., Peters, J. E., Alberici, F., Liley, J., Coulson, R. M. R., Astle, W., Baldini, C., Bonatti, F., Cid, María Cinta, Elding, H., Emmi, G., Epplen, J., Guillevin, L., Jayne, D. R. W., Jiang, T., Gunnarsson, I., Lamprecht, P., Leslie, S., Little, M. A., Martorana, D., Moosig, F., Neumann, T., Ohlsson, S., Quickert, S., Ramirez, G. A., Rewerska, B., Schett, George, Sinico, R. A., Szczeklik, W., Tesar, Vladimir, Vukcevic, D., Akil, M., Barratt, J., Basu, N., Butterworth, A. S., Bruce, I., Clarkson, M., Conlon, N., DasGupta, B., Doulton, T. W. R., Espígol-Frigolé, Georgina, Flossmann, O., Gabrielli, A., Gasior, J., Gregorini, G., Guida, G., Hernández Rodríguez, José, Hruskova, Z., Hudson, A., Knight, A., Lanyon, P., Luqmani, R., Magliano, M., Manfredi, A. A., Marguerie, C., Maritati, F., Marvisi, C., McHugh, N. J., Molloy, E., Motyer, A., Mukhtyar, C., Padyukov, Leonid, Pesci, Alberto, Prieto-Gonzalez, S., Ramentol-Sintas, Marc, Reis, P., Roccatello, D., Rovere-Querini, P., Salvarani, C., Santarsia, F., Solans, Roser, Soranzo, N., Taylor, J., Wessels, J., Zwerina, J., Terrier, B., Watts, R. A., Vaglio, A., Holle, J. U., Wallace, C., Smith, K. G. C., Universitat Autònoma de Barcelona, Lyons, P. A., Peters, J. E., Alberici, F., Liley, J., Coulson, R. M. R., Astle, W., Baldini, C., Bonatti, F., Cid, M. C., Elding, H., Emmi, G., Epplen, J., Guillevin, L., Jayne, D. R. W., Jiang, T., Gunnarsson, I., Lamprecht, P., Leslie, S., Little, M. A., Martorana, D., Moosig, F., Neumann, T., Ohlsson, S., Quickert, S., Ramirez, G. A., Rewerska, B., Schett, G., Sinico, R. A., Szczeklik, W., Tesar, V., Vukcevic, D., Akil, M., Barratt, J., Basu, N., Butterworth, A. S., Bruce, I., Clarkson, M., Conlon, N., Dasgupta, B., Doulton, T. W. R., Espigol-Frigole, G., Flossmann, O., Gabrielli, A., Gasior, J., Gregorini, G., Guida, G., Hernandez-Rodriguez, J., Hruskova, Z., Hudson, A., Knight, A., Lanyon, P., Luqmani, R., Magliano, M., Manfredi, A. A., Marguerie, C., Maritati, F., Marvisi, C., Mchugh, N. J., Molloy, E., Motyer, A., Mukhtyar, C., Padyukov, L., Pesci, A., Prieto-Gonzalez, S., Ramentol-Sintas, M., Reis, P., Roccatello, D., Rovere-Querini, P., Salvarani, C., Santarsia, F., Solans-Laque, R., Soranzo, N., Taylor, J., Wessels, J., Zwerina, J., Terrier, B., Watts, R. A., Vaglio, A., Holle, J. U., Wallace, C., Smith, K. G. C., Lyons, P, Peters, J, Alberici, F, Liley, J, Coulson, R, Astle, W, Baldini, C, Bonatti, F, Cid, M, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, D, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, M, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramierez, G, Rewerska, B, Schett, G, Sinico, R, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, A, Bruce, I, Clarkson, M, Conlon, N, Dasgupta, B, Doulton, T, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, A, Marguerie, C, Maritati, F, Marvisi, C, Mchugh, N, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, R, Vaglio, A, Holle, J, Wallace, C, Smith, K, and United Kingdom Research and Innovation

Subjects
0301 basic medicine, Candidate gene, Antineutrophil Cytoplasmic, General Physics and Astronomy, Genome-wide association study, Autoimmunity, Genome-wide association studies, 0302 clinical medicine, Rheumatic diseases, immune system diseases, Eosinophilic, Eosinophilia, lcsh:Science, education.field_of_study, Multidisciplinary, Genome-wide association, eosinophilic granulomatosis with polyangiitis, ANCA status, 3. Good health, medicine.symptom, Vasculitis, Granulomatosis with polyangiitis, Antibodies, Antineutrophil Cytoplasmic, Eosinophils, Genetic Association Studies, Granulomatosis with Polyangiitis, Humans, Mendelian Randomization Analysis, Genetic Loci, Genetic Predisposition to Disease, Genome-Wide Association Study, European Vasculitis Genetics Consortium, Science, Population, General Biochemistry, Genetics and Molecular Biology, Article, Antibodies, 03 medical and health sciences, medicine, Immunogenetics, education, Rheumatology and Autoimmunity, Anti-neutrophil cytoplasmic antibody, 030203 arthritis & rheumatology, Reumatologi och inflammation, business.industry, General Chemistry, medicine.disease, respiratory tract diseases, 030104 developmental biology, Immunology, EGPA, HLA, ANCA, genetics, lcsh:Q, business
Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA., Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disorder characterised by asthma, eosinophilia and vasculitis. Here, the authors describe a genome-wide association study of EGPA that reveals clinical and genetic differences between subgroups stratified by autoantibody status (ANCA).

Published
2019

30. Risk of acute arterial and venous thromboembolic events in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)

Author

Bettiol, A, Sinico, R, Schiavon, F, Monti, S, Bozzolo, E, Franceschini, F, Govoni, M, Lunardi, C, Guida, G, Lopalco, G, Paolazzi, G, Vacca, A, Gregorini, G, Leccese, P, Piga, M, Conti, F, Fraticelli, P, Quartuccio, L, Alberici, F, Salvarani, C, Bettio, S, Negrini, S, Selmi, C, Sciascia, S, Moroni, G, Colla, L, Manno, C, Urban, M, Vannacci, A, Pozzi, M, Fabbrini, P, Polti, S, Felicetti, M, Marchi, M, Padoan, R, Delvino, P, Caporali, R, Montecucco, C, Dagna, L, Cariddi, A, Toniati, P, Tamanini, S, Furini, F, Bortoluzzi, A, Tinazzi, E, Delfino, L, Badiu, I, Rolla, G, Venerito, V, Iannone, F, Berti, A, Bortolotti, R, Racanelli, V, Jeannin, G, Padula, A, Cauli, A, Priori, R, Gabrielli, A, Bond, M, Tedesco, M, Pazzola, G, Tomietto, P, Pellecchio, M, Marvisi, C, Maritati, F, Palmisano, A, Dejaco, C, Willeit, J, Kiechl, S, Olivotto, I, Willeit, P, Prisco, D, Vaglio, A, Emmi, G, Bettiol, Alessandra, Sinico, Renato Alberto, Schiavon, Franco, Monti, Sara, Bozzolo, Enrica Paola, Franceschini, Franco, Govoni, Marcello, Lunardi, Claudio, Guida, Giuseppe, Lopalco, Giuseppe, Paolazzi, Giuseppe, Vacca, Angelo, Gregorini, Gina, Leccese, Pietro, Piga, Matteo, Conti, Fabrizio, Fraticelli, Paolo, Quartuccio, Luca, Alberici, Federico, Salvarani, Carlo, Bettio, Silvano, Negrini, Simone, Selmi, Carlo, Sciascia, Savino, Moroni, Gabriella, Colla, Loredana, Manno, Carlo, Urban, Maria Letizia, Vannacci, Alfredo, Pozzi, Maria Rosa, Fabbrini, Paolo, Polti, Stefano, Felicetti, Mara, Marchi, Maria Rita, Padoan, Roberto, Delvino, Paolo, Caporali, Roberto, Montecucco, Carlomaurizio, Dagna, Lorenzo, Cariddi, Adriana, Toniati, Paola, Tamanini, Silvia, Furini, Federica, Bortoluzzi, Alessandra, Tinazzi, Elisa, Delfino, Lorenzo, Badiu, Iuliana, Rolla, Giovanni, Venerito, Vincenzo, Iannone, Florenzo, Berti, Alvise, Bortolotti, Roberto, Racanelli, Vito, Jeannin, Guido, Padula, Angela, Cauli, Alberto, Priori, Roberta, Gabrielli, Armando, Bond, Milena, Tedesco, Martina, Pazzola, Giulia, Tomietto, Paola, Pellecchio, Marco, Marvisi, Chiara, Maritati, Federica, Palmisano, Alessandra, Dejaco, Christian, Willeit, Johann, Kiechl, Stefan, Olivotto, Iacopo, Willeit, Peter, Prisco, Domenico, Vaglio, Augusto, Emmi, Giacomo, Bettiol, A, Sinico, R, Schiavon, F, Monti, S, Bozzolo, E, Franceschini, F, Govoni, M, Lunardi, C, Guida, G, Lopalco, G, Paolazzi, G, Vacca, A, Gregorini, G, Leccese, P, Piga, M, Conti, F, Fraticelli, P, Quartuccio, L, Alberici, F, Salvarani, C, Bettio, S, Negrini, S, Selmi, C, Sciascia, S, Moroni, G, Colla, L, Manno, C, Urban, M, Vannacci, A, Pozzi, M, Fabbrini, P, Polti, S, Felicetti, M, Marchi, M, Padoan, R, Delvino, P, Caporali, R, Montecucco, C, Dagna, L, Cariddi, A, Toniati, P, Tamanini, S, Furini, F, Bortoluzzi, A, Tinazzi, E, Delfino, L, Badiu, I, Rolla, G, Venerito, V, Iannone, F, Berti, A, Bortolotti, R, Racanelli, V, Jeannin, G, Padula, A, Cauli, A, Priori, R, Gabrielli, A, Bond, M, Tedesco, M, Pazzola, G, Tomietto, P, Pellecchio, M, Marvisi, C, Maritati, F, Palmisano, A, Dejaco, C, Willeit, J, Kiechl, S, Olivotto, I, Willeit, P, Prisco, D, Vaglio, A, Emmi, G, Bettiol, Alessandra, Sinico, Renato Alberto, Schiavon, Franco, Monti, Sara, Bozzolo, Enrica Paola, Franceschini, Franco, Govoni, Marcello, Lunardi, Claudio, Guida, Giuseppe, Lopalco, Giuseppe, Paolazzi, Giuseppe, Vacca, Angelo, Gregorini, Gina, Leccese, Pietro, Piga, Matteo, Conti, Fabrizio, Fraticelli, Paolo, Quartuccio, Luca, Alberici, Federico, Salvarani, Carlo, Bettio, Silvano, Negrini, Simone, Selmi, Carlo, Sciascia, Savino, Moroni, Gabriella, Colla, Loredana, Manno, Carlo, Urban, Maria Letizia, Vannacci, Alfredo, Pozzi, Maria Rosa, Fabbrini, Paolo, Polti, Stefano, Felicetti, Mara, Marchi, Maria Rita, Padoan, Roberto, Delvino, Paolo, Caporali, Roberto, Montecucco, Carlomaurizio, Dagna, Lorenzo, Cariddi, Adriana, Toniati, Paola, Tamanini, Silvia, Furini, Federica, Bortoluzzi, Alessandra, Tinazzi, Elisa, Delfino, Lorenzo, Badiu, Iuliana, Rolla, Giovanni, Venerito, Vincenzo, Iannone, Florenzo, Berti, Alvise, Bortolotti, Roberto, Racanelli, Vito, Jeannin, Guido, Padula, Angela, Cauli, Alberto, Priori, Roberta, Gabrielli, Armando, Bond, Milena, Tedesco, Martina, Pazzola, Giulia, Tomietto, Paola, Pellecchio, Marco, Marvisi, Chiara, Maritati, Federica, Palmisano, Alessandra, Dejaco, Christian, Willeit, Johann, Kiechl, Stefan, Olivotto, Iacopo, Willeit, Peter, Prisco, Domenico, Vaglio, Augusto, and Emmi, Giacomo

Published
2021

31. Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Author

Papo, M, Sinico, R, Teixeira, V, Venhoff, N, Urban, M, Iudici, M, Mahrhold, J, Locatelli, F, Cassone, G, Schiavon, F, Seeliger, B, Neumann, T, Kroegel, C, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Troilo, A, Thiel, J, Hellmich, B, Monti, S, Montecucco, C, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, Matthias, Sinico, Renato A, Teixeira, Vítor, Venhoff, Nils, Urban, Maria-Letizia, Iudici, Michele, Mahrhold, Juliane, Locatelli, Francesco, Cassone, Giulia, Schiavon, Franco, Seeliger, Benjamin, Neumann, Thomas, Kroegel, Claus, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Troilo, Arianna, Thiel, Jens, Hellmich, Bernhard, Monti, Sara, Montecucco, Carlomaurizio, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, Terrier, Benjamin, Papo, M, Sinico, R, Teixeira, V, Venhoff, N, Urban, M, Iudici, M, Mahrhold, J, Locatelli, F, Cassone, G, Schiavon, F, Seeliger, B, Neumann, T, Kroegel, C, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Troilo, A, Thiel, J, Hellmich, B, Monti, S, Montecucco, C, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, Matthias, Sinico, Renato A, Teixeira, Vítor, Venhoff, Nils, Urban, Maria-Letizia, Iudici, Michele, Mahrhold, Juliane, Locatelli, Francesco, Cassone, Giulia, Schiavon, Franco, Seeliger, Benjamin, Neumann, Thomas, Kroegel, Claus, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Troilo, Arianna, Thiel, Jens, Hellmich, Bernhard, Monti, Sara, Montecucco, Carlomaurizio, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, and Terrier, Benjamin

Abstract

OBJECTIVES: Only a third of patients with eosinophilic granulomatosis with polyangiitis (EGPA) are ANCA-positive, mainly directed against MPO. ANCA directed against PR3 are rarely found in EGPA. We aimed to examine the significance of PR3-ANCA in EGPA. METHODS: We set up a retrospective European multicentre cohort including 845 patients. Baseline characteristics and outcomes were analysed and compared according to ANCA status. RESULTS: ANCA status was available for 734 patients: 508 (69.2%) ANCA-negative, 210 (28.6%) MPO-ANCA and 16 (2.2%) PR3-ANCA. At baseline, PR3-ANCA patients, compared with those with MPO-ANCA and ANCA-negative, less frequently had active asthma (69% vs 91% and 93%, P = 0.003, respectively) and peripheral neuropathy (31% vs 71% and 47%, P < 0.0001), more frequently had cutaneous manifestations (63% vs 38% and 34%, P = 0.03) and pulmonary nodules (25% vs 10% and 8%, P = 0.046), and lower median eosinophil count (1450 vs 5400 and 3224/mm3, P < 0.0001). Vasculitis relapse-free survival was shorter for PR3-ANCA (hazard ratio 6.05, P = 0.005) and MPO-ANCA patients (hazard ratio 1.88, P = 0.0002) compared with ANCA-negative patients. CONCLUSION: PR3-ANCA EGPA patients differ from those with MPO-ANCA and negative ANCA, and share clinical features with granulomatosis with polyangiitis. This suggests that PR3-ANCA EGPA could be a particular form of PR3-ANCA-associated vasculitis.

Published
2021

33. FCGR3B polymorphism predicts relapse risk in eosinophilic granulomatosis with polyangiitis

Author

Alberici, F, Bonatti, F, Adorni, A, Daminelli, G, Sinico, R, Gregorini, G, Marvisi, C, Fenaroli, P, Peyronel, F, Maritati, F, Palmisano, A, Urban, M, Percesepe, A, Emmi, G, Martorana, D, Vaglio, A, Alberici, Federico, Bonatti, Francesco, Adorni, Alessia, Daminelli, Giulia, Sinico, Renato A, Gregorini, Gina, Marvisi, Chiara, Fenaroli, Paride, Peyronel, Francesco, Maritati, Federica, Palmisano, Alessandra, Urban, Maria Letizia, Percesepe, Antonio, Emmi, Giacomo, Martorana, Davide, Vaglio, Augusto, Alberici, F, Bonatti, F, Adorni, A, Daminelli, G, Sinico, R, Gregorini, G, Marvisi, C, Fenaroli, P, Peyronel, F, Maritati, F, Palmisano, A, Urban, M, Percesepe, A, Emmi, G, Martorana, D, Vaglio, A, Alberici, Federico, Bonatti, Francesco, Adorni, Alessia, Daminelli, Giulia, Sinico, Renato A, Gregorini, Gina, Marvisi, Chiara, Fenaroli, Paride, Peyronel, Francesco, Maritati, Federica, Palmisano, Alessandra, Urban, Maria Letizia, Percesepe, Antonio, Emmi, Giacomo, Martorana, Davide, and Vaglio, Augusto

Published
2020

34. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, P.A., Peters, J.E., Alberici, F., Liley, J., Coulson, R.M.R., Astle, W., Baldini, C., Bonatti, F., Cid, M.C., Elding, H., Emmi, G., Epplen, J., Guillevin, L., Jayne, D.R.W., Jiang, T., Gunnarsson, I., Lamprecht, P., Leslie, S., Little, M.A., Martorana, D., Moosig, F., Neumann, T., Ohlsson, S., Quickert, S., Ramirez, G.A., Rewerska, B., Schett, G., Sinico, R.A., Szczeklik, W., Tesar, V., Vukcevic, D., Akil, M., Barratt, J., Basu, N., Butterworth, A.S., Bruce, I., Clarkson, M., Conlon, N., DasGupta, B., Doulton, T.W.R., Espígol-Frigolé, Georgina, Flossmann, O., Gabrielli, A., Gasior, J., Gregorini, G., Guida, G., Hernández Rodríguez, José, Hruskova, Z., Hudson, A., Knight, A., Lanyon, P., Luqmani, R., Magliano, M., Manfredi, A.A., Marguerie, C., Maritati, F., Marvisi, C., McHugh, N.J., Molloy, E., Motyer, A., Mukhtyar, C., Padyukov, L., Pesci, A., Prieto-Gonzalez, S., Ramentol-Sintas, Marc, Reis, P., Roccatello, D., Rovere-Querini, P., Salvarani, C., Santarsia, F., Solans-Laque, R., Soranzo, N., Taylor, J., Wessels, J., Zwerina, J., Terrier, B., Watts, R.A., Vaglio, A., Holle, J.U., Wallace, C., Smith, K.G.C., and Universitat Autònoma de Barcelona

Subjects
Eosinophils, Genetic Loci, Granulomatosis with Polyangiitis, Humans, Genetic Predisposition to Disease, Mendelian Randomization Analysis, Genetic Association Studies, Antibodies, Antineutrophil Cytoplasmic, Genome-Wide Association Study
Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA.

Published
2019

35. PREDICTION OF LONG-TERM EVOLUTIONARY PROFILES IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS) BASED ON BASELINE AND FOLLOW-UP CHARACTERISTICS

Author

Papo, M, Sinico, Ra, Teixeira, V, Urban, Ml, Mahrhold, J, Monti, S, Cassone, G, Schiavon, F, Seeliger, B, Neumann, T, Kroegel, C, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Montecucco, C, Salvarani, C, Kahn, Je, Bonnotte, B, Durel, Ca, Mouthon, L, Puechal, X, Guillevin, L, Emmi, G, Vaglio, A, and Terrier, B

Published
2019

36. EFFICACY OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS TREATMENTS ACCORDING TO THE TYPE OF MANIFESTATIONS BASED ON ANALYSIS OF 636 PATIENTS

Author

Papo, M, Sinico, Ra, Teixeira, V, Urban, Ml, Mahrhold, J, Monti, S, Cassone, G, Schiavon, F, Seeliger, B, Neumann, T, Kroegel, C, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Montecucco, C, Salvarani, C, Kahn, Je, Bonnotte, B, Durel, Ca, Puechal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, and Terrier, B

Published
2019

37. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, P, Peters, J, Alberici, F, Liley, J, Coulson, R, Astle, W, Baldini, C, Bonatti, F, Cid, M, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, D, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, M, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramierez, G, Rewerska, B, Schett, G, Sinico, R, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, A, Bruce, I, Clarkson, M, Conlon, N, Dasgupta, B, Doulton, T, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, A, Marguerie, C, Maritati, F, Marvisi, C, Mchugh, N, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, R, Vaglio, A, Holle, J, Wallace, C, Smith, K, Lyons, PA, Peters, JE, Cid, MC, Little, MA, Ramierez, GA, Butterworth, AS, DasGupta, B, Doulton, TWR, Manfredi, AA, McHugh, NJ, Watts, RA, Holle, JU, Smith, KGC, Lyons, P, Peters, J, Alberici, F, Liley, J, Coulson, R, Astle, W, Baldini, C, Bonatti, F, Cid, M, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, D, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, M, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramierez, G, Rewerska, B, Schett, G, Sinico, R, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, A, Bruce, I, Clarkson, M, Conlon, N, Dasgupta, B, Doulton, T, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, A, Marguerie, C, Maritati, F, Marvisi, C, Mchugh, N, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, R, Vaglio, A, Holle, J, Wallace, C, Smith, K, Lyons, PA, Peters, JE, Cid, MC, Little, MA, Ramierez, GA, Butterworth, AS, DasGupta, B, Doulton, TWR, Manfredi, AA, McHugh, NJ, Watts, RA, Holle, JU, and Smith, KGC

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA.

Published
2019

38. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, PA, Peters, JE, Alberici, F, Liley, J, Coulson, RMR, Astle, W, Baldini, C, Bonatti, F, Cid, MC, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, DRW, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, MA, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramirez, GA, Rewerska, B, Schett, G, Sinico, RA, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, AS, Bruce, I, Clarkson, M, Conlon, N, DasGupta, B, Doulton, TWR, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, AA, Marguerie, C, Maritati, F, Marvisi, C, McHugh, NJ, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, RA, Vaglio, A, Holle, JU, Wallace, C, Smith, KGC, Lyons, PA, Peters, JE, Alberici, F, Liley, J, Coulson, RMR, Astle, W, Baldini, C, Bonatti, F, Cid, MC, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, DRW, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, MA, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramirez, GA, Rewerska, B, Schett, G, Sinico, RA, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, AS, Bruce, I, Clarkson, M, Conlon, N, DasGupta, B, Doulton, TWR, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, AA, Marguerie, C, Maritati, F, Marvisi, C, McHugh, NJ, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, RA, Vaglio, A, Holle, JU, Wallace, C, and Smith, KGC

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA.

Published
2019

39. New perspectives in eosinophilic granulomatosis with polyangiitis (EGPA): report of the first meeting of the European EGPA Study Group

Author

Marvisi, C, Sinico, R, Salvarani, C, Jayne, D, Prisco, D, Terrier, B, Emmi, G, Vaglio, A, Marvisi, Chiara, Sinico, Renato Alberto, Salvarani, Carlo, Jayne, David, Prisco, Domenico, Terrier, Benjamin, Emmi, Giacomo, Vaglio, Augusto, Marvisi, C, Sinico, R, Salvarani, C, Jayne, D, Prisco, D, Terrier, B, Emmi, G, Vaglio, A, Marvisi, Chiara, Sinico, Renato Alberto, Salvarani, Carlo, Jayne, David, Prisco, Domenico, Terrier, Benjamin, Emmi, Giacomo, and Vaglio, Augusto

Abstract

The European Eosinophilic Granulomatosis with Polyangiitis (EGPA) study group first gathered in Firenze in December 2018. The discussion was centred around the clinical and therapeutic needs in EGPA which still remain unmet. Indeed, EGPA is a puzzling and rare disease which shares clinical features with other anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAVs) and hypereosinophilic syndromes (HESs). Some of the recommendations published in 2015 are based on data derived from EGPA-related diseases, rather than from EGPA itself, and therefore need to be updated. Thus, the aim of the meeting was to stimulate ongoing research, to promote collaborative European studies and to define the main issues on which future studies should be focused. Current fields of research on EGPA include potential serological biomarkers of disease activity and of specific organ involvement, possible links between different genetic variants and clinical phenotypes, and new therapeutic perspectives. Herein, we give an overview of the meeting with the goal to stimulate an international collaboration and new points of discussion.

Published
2019

40. 117. Prediction of Long-Term Evolutionary Profiles in Eosinophilic Granulomatosis with Polyangiitis (Churg– Strauss) Based on Baseline and Follow-Up Characteristics

Author

Papo, M, Sinico, R, Teixeira, V, Urban, M, Mahrhold, J, Cassone, G, Schiavon, F, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Cohen, P, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, Matthias, Sinico, Renato, Teixeira, Vítor, Urban, Maria Letizia, Mahrhold, Juliane, Cassone, Giulia, Schiavon, Franco, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Hellmich, Bernhard, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Cohen, Pascal, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, Terrier, Benjamin, Papo, M, Sinico, R, Teixeira, V, Urban, M, Mahrhold, J, Cassone, G, Schiavon, F, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Cohen, P, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, Matthias, Sinico, Renato, Teixeira, Vítor, Urban, Maria Letizia, Mahrhold, Juliane, Cassone, Giulia, Schiavon, Franco, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Hellmich, Bernhard, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Cohen, Pascal, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, and Terrier, Benjamin

Published
2019

41. 354. EFFICACY OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS TREATMENTS ACCORDING TO THE TYPE OF MANIFESTATIONS BASED ON ANALYSIS OF 636 PATIENTS

Author

Papo, M, Sinico, A, Teixeira, V, Urban, M, Mahrhold, J, Cassone, G, Schiavon, F, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Cohen, P, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, Matthias, Sinico, Alberto, Teixeira, Vítor, Urban, Maria Letizia, Mahrhold, Juliane, Cassone, Giulia, Schiavon, Franco, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Hellmich, Bernhard, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Cohen, Pascal, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, Terrier, Benjamin, Papo, M, Sinico, A, Teixeira, V, Urban, M, Mahrhold, J, Cassone, G, Schiavon, F, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Salvarani, C, Kahn, J, Bonnotte, B, Durel, C, Cohen, P, Puéchal, X, Mouthon, L, Guillevin, L, Emmi, G, Vaglio, A, Terrier, B, Papo, Matthias, Sinico, Alberto, Teixeira, Vítor, Urban, Maria Letizia, Mahrhold, Juliane, Cassone, Giulia, Schiavon, Franco, Groh, Matthieu, Marvisi, Chiara, Samson, Maxime, Barba, Thomas, Jayne, David, Hellmich, Bernhard, Salvarani, Carlo, Kahn, Jean-Emmanuel, Bonnotte, Bernard, Durel, Cécile-Audrey, Cohen, Pascal, Puéchal, Xavier, Mouthon, Luc, Guillevin, Loïc, Emmi, Giacomo, Vaglio, Augusto, and Terrier, Benjamin

Published
2019

42. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease

Author

Wallace, Zachary S, Naden, Ray P, Chari, Suresh, Choi, Hyon, Della-Torre, Emanuel, Dicaire, Jean-Francois, Hart, Phil A, Inoue, Dai, Kawano, Mitsuhiro, Khosroshahi, Arezou, Kubota, Kensuke, Lanzillotta, Marco, Okazaki, Kazuichi, Perugino, Cory A, Sharma, Amita, Saeki, Takako, Sekiguchi, Hiroshi, Schleinitz, Nicolas, Stone, James R, Takahashi, Naoki, Umehara, Hisanori, Webster, George, Zen, Yoh, Stone, John H, Akamizu, T, Akiyama, M, Barra, L, Bateman, A, Blockmans, D, Brito-Zeron, P, Campochiaro, C, Carruthers, M, Chari, S, Chiba, T, Choi, H, Cornell, L, Culver, E, Darabian, S, Torre, Ed, Deshpande, V, Dong, L, Ebbo, M, Fernández-Codina, A, Ferry, Ja, Fragkoulis, G, Frost, F, Frulloni, L, Hart, Pa, Hernandez-Molina, G, Inoue, D, Ji, H, Keat, K, Kamisawa, T, Kawa, S, Kawano, M, Khosroshahi, A, Kobayashi, H, Kodama, Y, Kubo, S, Kubota, K, Lanzillotta, M, Leng, H, Lerch, M, Liu, Y, Liu, Z, Löhr, M, Martin-Nares, E, Martinez-Valle, F, Marvisi, C, Masaki, Y, Matsui, S, Mizushima, I, Naden, Rp, Nakamura, S, Nordeide, J, Notohara, K, Okazaki, K, Paira, S, Perugino, Ca, Popovic, J, Ramos-Casals, M, Rosenbaum, J, Ryu, J, Saeki, T, Sato, Y, Schleinitz, N, Sekiguchi, H, Sharma, A, Sokol, Ev, Stone, Jr, Stone, Jh, Sun, W, Takahashi, H, Takahashi, N, Takahira, M, Tanaka, Y, Umehara, H, Vaglio, A, Villamil, A, Wada, Y, Wallace, Zs, Webster, G, Yamada, K, Yamamoto, M, Yi, J, Yi, Y, Zamboni, G, Zen, Y, Zhang, W., Wallace, Z, Naden, Rp, Chari, S, Choi, H, DELLA TORRE, E, Dicaire, Jf, Hart, Pa, Inoue, D, Kawano, M, Khosroshahi, A, Kubota, K, Lanzillotta, M, Okazaki, K, Perugino, Ca, Sharma, A, Saeki, T, Sekiguchi, H, Schleinitz, N, Stone, Jr, Takahashi, N, Umehara, H, Webster, G, Zen, Y, Stone, Jh, and American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working, Group.

Subjects
Adult, Male, medicine.medical_specialty, Consensus, Aged, Diagnosis, Differential, Europe, Female, Humans, Immunoglobulin G4-Related Disease, Middle Aged, Reproducibility of Results, Rheumatology, Societies, Medical, United States, Immunology, MEDLINE, Disease, Internal medicine, Medical, parasitic diseases, Diagnosis, Immunology and Allergy, Medicine, business.industry, medicine.disease, Confidence interval, Test (assessment), Differential, IgG4-related disease, business, Societies, Rheumatism, Decision analysis
Abstract

Objective IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. Methods An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises, existing literature, derivation and validation cohorts of 1,879 subjects (1,086 cases, 793 mimickers), and multicriterion decision analysis to identify, weight, and test potential classification criteria. Two independent validation cohorts were included. Results A 3-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic, and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, 8 weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments, and pathology interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% confidence interval [95% CI] 97.2-99.8%) and a sensitivity of 85.5% (95% CI 81.9-88.5%). In the second, the specificity was 97.8% (95% CI 93.7-99.2%) and the sensitivity was 82.0% (95% CI 77.0-86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. Conclusion ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiologic, and basic science investigations.

Published
2018

43. FEMALE GENDER AND STEROID TREATMENT AS MAIN INFLUENCING FACTORS IN THE PERCEPTION OF QUALITY OF LIFE IN ANCA-ASSOCIATED VASCULITIS: FINAL RESULTS FROM THE ITALIAN VERSION OF ANCA-ASSOCIATED VASCULITIS PATIENT-REPORTED OUTCOME (AAV-PRO_ITA) QUESTIONNAIRE

Author

Treppo, E., Isola, M., De Martino, M., Padoan, R., Urban, M. L., Monti, S., Sartorelli, S., Giollo, A., Argolini, L. M., Marvisi, C., Gattamelata, A., Regola, F., Ferro, F., Cassone, G., Motta, F., Berti, A., Conticini, E., Guiducci, S., Matucci-Cerinic, M., and Lo Gullo, A.

Published
2023
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