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2. AB1329 COMPARATIVE EFFICACY AND SAFETY OF GLUCOCORTICOIDS, METHOTREXATE AND TOCILIZUMAB IN THE TREATMENT OF NEW-ONSET LARGE VESSEL GIANT CELL ARTERITIS

Author

Davanzo, F., primary, Iorio, L., additional, Delvino, P., additional, Fiorin, E., additional, Codirenzi, M., additional, Sorace, D., additional, Campochiaro, C., additional, Nannipieri, S., additional, Dagna, L., additional, Doria, A., additional, Monti, S., additional, Tomelleri, A., additional, and Padoan, R., additional

Published
2024
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5. 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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7. Eosinophilic granulomatosis with polyangiitis onset in severe asthma patients on monoclonal antibodies targeting type 2 inflammation: Report from the European EGPA study group

Author

Caminati, M, Fassio, A, Alberici, F, Baldini, C, Bello, F, Cameli, P, Conticini, E, Cottin, V, Crimi, C, Dagna, L, Delvino, P, Deroux, A, Duran, E, Espigol-Frigole, G, Karadag, O, Maule, M, Moiseev, S, Monti, S, Moroni, L, Padoan, R, Pugnet, G, Taille, C, Toniati, P, Vaglio, A, Emmi, G, Caminati, M, Fassio, A, Alberici, F, Baldini, C, Bello, F, Cameli, P, Conticini, E, Cottin, V, Crimi, C, Dagna, L, Delvino, P, Deroux, A, Duran, E, Espigol-Frigole, G, Karadag, O, Maule, M, Moiseev, S, Monti, S, Moroni, L, Padoan, R, Pugnet, G, Taille, C, Toniati, P, Vaglio, A, and Emmi, G

Published
2024

8. Characteristics and outcome of ANCA-associated vasculitides induced by anti-thyroid drugs: a multicentre retrospective case-control study

Author

Culerrier, J, Nguyen, Y, Karadag, O, Yasar Bilge, S, Demirci Yıldırım, T, Saygin Ögüt, T, Yazisiz, V, Bes, C, Celfe, A, Yazici, A, Sadioglu Cagdas, O, Kronbichler, A, Jayne, D, Gauckler, P, Regent, A, Teixeira, V, Marchand-Adam, S, Duffau, P, Ingen-Housz-Oro, S, Droumaguet, C, Andre, B, Luca, L, Lechtman, S, Aouba, A, Lebas, C, Servettaz, A, Dernoncourt, A, Ruivard, M, Milesi, A, Poindron, V, Jego, P, Padoan, R, Delvino, P, Vandergheynst, F, Pagnoux, C, Yacyshyn, E, Lamprecht, P, Flossmann, O, Puéchal, X, Terrier, B, Julien Culerrier, Yann Nguyen, Omer Karadag, Sule Yasar Bilge, Tuba Demirci Yıldırım, Tahir Saygin Ögüt, Veli Yazisiz, Cemal Bes, Ayse Celfe, Ayten Yazici, Oznur Sadioglu Cagdas, Andreas Kronbichler, David Jayne, Philipp Gauckler, Alexis Regent, Vitor Teixeira, Sylvain Marchand-Adam, Pierre Duffau, Saskia Ingen-Housz-Oro, Celine Droumaguet, Baptiste Andre, Luminita Luca, Sarah Lechtman, Achille Aouba, Celine Lebas, Amélie Servettaz, Amandine Dernoncourt, Marc Ruivard, Anne-Marie Milesi, Vincent Poindron, Patrick Jego, Roberto Padoan, Paolo Delvino, Frédéric Vandergheynst, christian pagnoux, Elaine Yacyshyn, Peter Lamprecht, Oliver Flossmann, Xavier Puéchal, Benjamin Terrier, Culerrier, J, Nguyen, Y, Karadag, O, Yasar Bilge, S, Demirci Yıldırım, T, Saygin Ögüt, T, Yazisiz, V, Bes, C, Celfe, A, Yazici, A, Sadioglu Cagdas, O, Kronbichler, A, Jayne, D, Gauckler, P, Regent, A, Teixeira, V, Marchand-Adam, S, Duffau, P, Ingen-Housz-Oro, S, Droumaguet, C, Andre, B, Luca, L, Lechtman, S, Aouba, A, Lebas, C, Servettaz, A, Dernoncourt, A, Ruivard, M, Milesi, A, Poindron, V, Jego, P, Padoan, R, Delvino, P, Vandergheynst, F, Pagnoux, C, Yacyshyn, E, Lamprecht, P, Flossmann, O, Puéchal, X, Terrier, B, Julien Culerrier, Yann Nguyen, Omer Karadag, Sule Yasar Bilge, Tuba Demirci Yıldırım, Tahir Saygin Ögüt, Veli Yazisiz, Cemal Bes, Ayse Celfe, Ayten Yazici, Oznur Sadioglu Cagdas, Andreas Kronbichler, David Jayne, Philipp Gauckler, Alexis Regent, Vitor Teixeira, Sylvain Marchand-Adam, Pierre Duffau, Saskia Ingen-Housz-Oro, Celine Droumaguet, Baptiste Andre, Luminita Luca, Sarah Lechtman, Achille Aouba, Celine Lebas, Amélie Servettaz, Amandine Dernoncourt, Marc Ruivard, Anne-Marie Milesi, Vincent Poindron, Patrick Jego, Roberto Padoan, Paolo Delvino, Frédéric Vandergheynst, christian pagnoux, Elaine Yacyshyn, Peter Lamprecht, Oliver Flossmann, Xavier Puéchal, and Benjamin Terrier

Abstract

Objective: Data on ANCA-associated vasculitis (AAV) induced by anti-thyroid drugs (ATD) are scarce. We aimed to describe the characteristics and outcome of these patients in comparison to primary AAV. Methods: We performed a retrospective multicentre study including patients with ATD-induced AAV. We focused on ATD-induced microscopic polyangiitis (MPA) and compared them with primary MPA by matching each case with four controls by gender and year of diagnosis. Results: Forty-five patients with ATD-induced AAV of whom 24 MPA were included. ANCA were positive in 44 patients (98%), including myeloperoxidase (MPO)-ANCA in 21 (47%), proteinase 3 (PR3)-ANCA in six (13%), and double positive MPO- and PR3-ANCA in 15 (33%). Main clinical manifestations were skin involvement (64%), arthralgia (51%) and glomerulonephritis (20%). ATD was discontinued in 98% of cases, allowing vasculitis remission in seven (16%). All the remaining patients achieved remission after glucocorticoids, in combination with rituximab in 11 (30%) or cyclophosphamide in four (11%). ATD were reintroduced in seven cases (16%) without any subsequent relapse. Compared with 96 matched primary MPA, ATD-induced MPA were younger at diagnosis (48 vs 65 years, P < 0.001), had more frequent cutaneous involvement (54 vs 25%, P 1⁄4 0.007), but less frequent kidney (38 vs 73%, P 1⁄4 0.02), and a lower risk of relapse (adjusted HR 0.07; 95% CI 0.01, 0.65, P 1⁄4 0.019). Conclusion: ATD-induced AAV were mainly MPA with MPO-ANCA, but double MPO- and PR3-ANCA positivity was frequent. The most common manifestations were skin and musculoskeletal manifestations. ATD-induced MPA were less severe and showed a lower risk of relapse than primary MPA.

Published
2024

9. Risk of CFTR-related disorders and cystic fibrosis in an Italian cohort of CRMS/CFSPID subjects in preschool and school age

Author

Fevola, C., primary, Dolce, D., additional, Tosco, A., additional, Padoan, R., additional, Daccò, V., additional, Claut, L., additional, Schgor, T., additional, Sepe, A., additional, Timpano, S., additional, Fabrizzi, B., additional, Piccinini, P., additional, Taccetti, G., additional, Bonomi, P., additional, and Terlizzi, V., additional

Published
2023
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12. P287 Characterisation of CFTR mutations in people with cystic fibrosis and severe liver disease who are not eligible for CFTR modulators

Author

Colombo, C., primary, Alicandro, G., additional, Ramm, G., additional, Lindblad, A., additional, Porcaro, L., additional, Alghisi, F., additional, Asherova, I., additional, Evans, H., additional, Kashirskaya, N., additional, Kondratyeva, E., additional, de Monestrol, I., additional, Oliver, M., additional, Ooi, C.Y., additional, Padoan, R., additional, Shankar, S., additional, and Lewindon, P.J., additional

Published
2023
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13. 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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14. OP0304 BENRALIZUMAB FOR EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (EGPA): RESULTS FROM A EUROPEAN MULTICENTER STUDY ON 121 PATIENTS

Author

Bettiol, A., primary, Mattioli, I., additional, Urban, M. L., additional, Bello, F., additional, Padoan, R., additional, Groh, M., additional, Lopalco, G., additional, Egan, A., additional, Cottin, V., additional, Fraticelli, P., additional, Crimi, C., additional, Del Giacco, S., additional, Schroeder, J., additional, Moi, L., additional, Jayne, D., additional, Vaglio, A., additional, and Emmi, G., additional

Published
2023
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15. AB0789 AVACOPAN IN THE REAL-LIFE PRACTICE: EVALUATION OF EFFICACY, SAFETY, AND IMPACT ON QUALITY OF LIFE IN THE EARLY STAGES OF TREATMENT

Author

Treppo, E., primary, Bello, F., additional, Galli, E., additional, Padoan, R., additional, Monti, S., additional, Moroni, L., additional, Tombetti, E., additional, Affatato, S., additional, Conticini, E., additional, Frediani, B., additional, Alberici, F., additional, Dagna, L., additional, Montecucco, C., additional, Schiavon, F., additional, Emmi, G., additional, Salvarani, C., additional, and Quartuccio, L., 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. POS0708 VALIDATION OF THE REVISED 2022 AMERICAN COLLEGE OF RHEUMATOLOGY/ EULAR CLASSIFICATION CRITERIA FOR TAKAYASU ARTERITIS

Author

Tomelleri, A., primary, Padoan, R., additional, Kavadichanda, C., additional, Jose, A., additional, Singh, K., additional, Iorio, L., additional, Rathore, U., additional, Rinaldi, E., additional, Baldissera, E., additional, Agarwal, V., additional, Dagna, L., additional, Campochiaro, C., additional, and Misra, D. P., additional

Published
2023
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20. 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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21. Anti-neutrophil cytoplasmic antibody specificity determines a different clinical subset in granulomatosis with polyangiitis

Author

Monti, S, Felicetti, M, Delvino, P, Padoan, R, Berti, A, Paolazzi, G, Brunori, G, Schiavon, F, Caporali, R, Montecucco, C, Monti S., Felicetti M., Delvino P., Padoan R., Berti A., Paolazzi G., Brunori G., Schiavon F., Caporali R., Montecucco C., Monti, S, Felicetti, M, Delvino, P, Padoan, R, Berti, A, Paolazzi, G, Brunori, G, Schiavon, F, Caporali, R, Montecucco, C, Monti S., Felicetti M., Delvino P., Padoan R., Berti A., Paolazzi G., Brunori G., Schiavon F., Caporali R., and Montecucco C.

Abstract

Objective. It has been suggested that anti-neutrophil cytoplasmic antibody (ANCA) specificity, rather than clinical diagnosis influences the phenotype and course of ANCA-associated vasculitis (AAV). However, preliminary evidence suggests that further combined levels of categorisation might be of clinical relevance. The aim of this study was to investigate differences in clinical presentation at disease onset and outcomes based on clinical diagnosis and ANCA specificity. Methods. Newly diagnosed patients with GPA or MPA assessed in three referral centres between 2000 and 2016 were included. Patients were grouped as MPO-ANCA-positive granulomatosis with polyangiitis (MPO-GPA), PR3-ANCA-positive-GPA (PR3-GPA), and MPO-ANCA-positive microscopic polyangiitis (MPO-MPA). Results. Of the 143 AAV patients included (female 52%), 87 were categorised as PR3-GPA, 23 as MPO-GPA, and 33 as MPO-MPA. Patients with MPO-GPA were significantly younger than MPA patients (age 49±15 versus 63±10; p<0.001). MPO-GPA had significantly more frequent subglottic stenosis compared to PR3-GPA. Ear, nose, throat involvement was significantly more frequent in both GPA groups compared to MPA. Type of pulmonary involvement differed between both GPA groups and MPA with diffuse pulmonary haemorrhage being significantly more frequent in the latter (7% in PR3-GPA, 0% in MPO-GPA, 27% in MPO-MPA; p<0.001). Renal involvement was more frequent in MPO-MPA compared to both MPO-GPA and PR3-GPA (impaired renal function in 84%, 39%, and 36%, respectively; p<0.001). PR3-GPA relapsed significantly more than the other two groups. After adjusting for age, MPO-GPA was a significant risk factor for mortality [HR 4.44 (95%CI 1.46-13.52), p=0.009]. Conclusion. ANCA specificity identifies specific subsets of disease characterised by different clinical presentation and outcome within the clinical diagnosis of GPA.

Published
2021

22. Anti-neutrophil cytoplasmic antibody specificity determines a different clinical subset in granulomatosis with polyangiitis

Author

Monti, S., Felicetti, M., Paolo Delvino, Padoan, R., Berti, A., Paolazzi, G., Brunori, G., Schiavon, F., Caporali, R., and Montecucco, C.

Subjects
Adult, Myeloblastin, Immunology, Antineutrophil Cytoplasmic, Granulomatosis with Polyangiitis, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Middle Aged, Antibodies, Antibodies, Antineutrophil Cytoplasmic, Rheumatology, Humans, Immunology and Allergy, Female, Peroxidase, Retrospective Studies
Abstract

It has been suggested that anti-neutrophil cytoplasmic antibody (ANCA) specificity, rather than clinical diagnosis influences the phenotype and course of ANCA-associated vasculitis (AAV). However, preliminary evidence suggests that further combined levels of categorisation might be of clinical relevance. The aim of this study was to investigate differences in clinical presentation at disease onset and outcomes based on clinical diagnosis and ANCA specificity.Newly diagnosed patients with GPA or MPA assessed in three referral centres between 2000 and 2016 were included. Patients were grouped as MPO-ANCA-positive granulomatosis with polyangiitis (MPO-GPA), PR3-ANCA-positive-GPA (PR3-GPA), and MPO-ANCA-positive microscopic polyangiitis (MPO-MPA).Of the 143 AAV patients included (female 52%), 87 were categorised as PR3-GPA, 23 as MPO-GPA, and 33 as MPO-MPA. Patients with MPO-GPA were significantly younger than MPA patients (age 49±15 versus 63±10; p0.001). MPO-GPA had significantly more frequent subglottic stenosis compared to PR3-GPA. Ear, nose, throat involvement was significantly more frequent in both GPA groups compared to MPA. Type of pulmonary involvement differed between both GPA groups and MPA with diffuse pulmonary haemorrhage being significantly more frequent in the latter (7% in PR3-GPA, 0% in MPO-GPA, 27% in MPOMPA; p0.001). Renal involvement was more frequent in MPO-MPA compared to both MPO-GPA and PR3-GPA (impaired renal function in 84%, 39%, and 36%, respectively; p0.001). PR3-GPA relapsed significantly more than the other two groups. After adjusting for age, MPO-GPA was a significant risk factor for mortality [HR 4.44 (95%CI 1.46-13.52), p=0.009].ANCA specificity identifies specific subsets of disease characterised by different clinical presentation and outcome within the clinical diagnosis of GPA.

Published
2021

23. Sécurité d’utilisation et efficacité du dupilumab au cours de la granulomatose éosinophilique avec polyangéite (GEPA) réfractaire

Author

Molina, B., primary, Urban, M.L., additional, Padoan, R., additional, Novikov, P., additional, Bouillet, L., additional, Caminati, M., additional, Taillé, C., additional, Hamidou, M., additional, Fraticeli, P., additional, Schleinitz, N., additional, Christides, C., additional, Moi, L., additional, Godeau, B., additional, Knight, A., additional, Schroder, J.W., additional, Marchand-Adam, S., additional, Gil, H., additional, Cottin, V., additional, Durel, C.A., additional, and Terrier, B., additional

Published
2022
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24. Association between age at disease onset of anti-neutrophil cytoplasmic antibody-associated vasculitis and clinical presentation and short-term outcomes

Author

Monti, S., Craven, A., Klersy, C., Montecucco, C., Caporali, R., Watts, R., Merkel, P. A., Luqmani, R., Achilleos, K., Adler, M., Alba, M. A., Albert, D. A., Alibaz-Oner, F., Allcoat, P., Amano, K., Amarasuriya, M., Amudala, N. A., Andrews, J., Archer, A. M., Arimura, Y., Atukorala, I., Azevedo, E., Bajad, S., Baldwin, C., Barra, L. J., Baslund, B., Basu, N., Baykal, M., Berger, C., Berglin, E., Besada, E., Bhardwaj, M., Bischof, A., Blockmans, D., Blood, J., Draibe, J. B., Brand, S., Brandao, M., Bruce, I. N., Butler, A., Calabrese, L. H., Ferrer, D. C., Carette, S., Carmona, D., Ceunen, H., Chakravarty, K., Chapman, P. T., Chocova, Z., Chung, S. A., Ci, W., Cid, M. C., Clark, T. M., Clarkson, M. R., De Jesus Contreras-Rodriguez, F., Conway, R., Cooke, K., Viros, X. C., Cordeiro, A., Costa, A., Culfear, K., Daikeler, T., Danda, D., Das, S. K., Dasgupta, B., De Castro, A. M., Dehghan, N., Devassy, R., Dhindsa, N., Diamantopoulos, A. P., Direskeneli, H., Dobashi, H., Juan, D., Durrani, M., Edelsten, C., Eifert, J., Elhayek, S., Elsideeg, S., Endo, T., Erden, A., Erer, B., Eriksson, P., Erturk, Z., Espigol-Frigole, G., Felicetti, M., Ferraro, A., Ferro, J. M., Fifi-Mah, A., Flores-Suarez, L. F., Flossmann, O., Flynn, D., Fonseca, J. E., Foot, J., Foote, M., Forbess, L., Fujimoto, S., Fukuoka, K., Furtado, C., Furuta, S., Gaffo, A. L., Gallagher, P., Gao, N., Gatenby, P., Gendi, N., Geraldes, R., Gerits, A., Gioffredi, A., Gomples, L., Goncalves, M. J., Gondo, P., Graham, A., Grainger, R., Gray, D. T., Grayson, P. C., Griffiths, L., Guo, Y., Gupta, R., Gylling, M., Hajj-Ali, R. A., Hammam, N., Harigai, M., Hartley, L., Haslett, J., Hassan, A., Hatemi, G., Hellmich, B., Henckaerts, L., Henes, J. C., Hepburn, J., Herd, V., Hess, C., Hill, C., Hinojosa-Azaola, A., Hirahashi, J., Hirano, F., Hocevar, A., Holle, J., Hollinger, N., Homma, S., Howard, T., Hoyles, R. K., Hruskova, Z., Hutcheon, G., Ignacak, M., Igney-Oertel, A., Ikeda, K., Ikegaya, N., Jagadeesh, S., Jaquith, J., Jayne, D. R. W., Jewell, T., Jones, C., Joshi, A., Kalyoncu, U., Kamall, S., Kamath, S., Lai, K. S., Kaname, S., Kanchinadham, S., Karadag, O., Karube, M., Kaszuba, M., Kaur, R., Kawakami, T., Kawashima, S., Khalidi, N., Khan, A., Kikuchi, M., Kilic, L., Kimura, M., King, M. J., Klapa, S., Klocke, R., Kobayashi, T., Kobayashi, S., Komagata, Y., Kronbichler, A., Kuczia, P., Kumar, M. S., Kurosawa, M., Lamprecht, P., Langford, C. A., Lanyon, P., Laversuch, C., Lee, S. J., Leoni, S., Li, J., Liang, K., Liang, P., Liao, H., Lee, L. A., Luqmani, R. A., Lyle, A., Macdonald, M., Mackie, S. L., Madden, L., Magliano, M., Makino, H., Makol, A., Malaiya, R., Malaviya, A., Manthri, R., Maritati, F., Da Silva, A. M., Mason, J. C., Matara, C., Matsui, K., Matteson, E. L., Mcbride, D., Mccullough, K., Mcgeoch, L., Mclaren, J., Mcmillian, C., Mendiratta, N., Menon, A., Merinopoulos, D., Merkel, P., Messier, S., Micheletti, R. G., Mills, K., Milman, N., Minoda, M., Minz, R. W., Mock, C., Mohammad, A. J., Moiseev, S., Moitinho, M., Molloy, E., Monach, P. A., Montgomery, M., Moosig, F., Moradizadeh, M., Morgan, M., Morgan, A. W., Morgan, A. -M., Muir, A., Mukhtyar, C., Muller, A., Muratore, F., Muso, E., Nada, R., Nakajima, H., Nakajima, T., Nakano, H., Nandagudi, A., Neumann, T., Y. F., Ng, K. H., Ng, Nogueira, E. L., Nolkha, N., Nordstrom, D., Novikov, P., Nugaliyadde, A., O'Donnell, J. L., O'Donoghue, J., O'Neill, L., O'Riordan, E., Oatley, M., Okubo, K., Oliva, E., Oshikawa, H., Ota, Y., Padoan, R., Pagnoux, C., Pan, L., Panaritis, K., Park, J. K., Patel, S., Patil, P., Pazzola, G., Peall, A., Pearce, F., Pehlevan, S., Pereira, L., Pettersson, T., Pineau, C. A., Pirila, L., Poglodek, B., Ponte, C., Prieto-Gonzalez, S., Priya, S. R., Purewal, B., Purschke, S., Putaala, J., Quickert, S., Quincey, V., Raghuvanshi, S., Rajasekhar, L., Ranganathan, D., Rathi, M., Rees, D., Rees, F., Renken, U., Restuccia, G., Rhee, R. L., Rice, B., Robins, D., Robson, J., Rodrigues, M., Romao, V. C., Rotar, C., Ruediger, C., Rutgers, A., A. C., Sa, Saavedra, M. J., Sada, K. -E., Sahbudin, I., Salvarani, C., Sandhu, N., Santos, E., Sato, Y., Schafer, V. S., Schiavon, F., Schmidt, W. A., Segelmark, M., Shahin, A., Sharma, A., Shotton, J., Silva, C., Singer, O. G., Sivasuthan, G., Smolen, S., Solanich-Moreno, X., Boixader, L. S., Song, Y. W., Springer, J., Sreih, A. G., Srivastava, R., Stamp, L. K., Stevens, R., Strbian, D., Sugino, K., Sunderkotter, C., Suppiah, R., Suzuki, K., Szekanecz, Z., Sznajd, J., Taimen, K., Tak, P. P., Takeuchi, T., Takizawa, N., Tames, L., Tan, B. E., Tanaka, M., Tang, M. W., Tatlisumak, T., Tesar, V., Thomas, A., Tian, X., Tokunaga, K., Tombetti, E., Tomsic, M., Toz, B., Tsukamoto, T., Uchida, S., Unal, A. U., Urban, M. L., Usui, J., Vaglio, A., Venkatachalam, S., Vermaak, E., Viswanath, V., Wada, T., Wagh, S., Wallace, D. J., Walters, G., Walz, B., Wan, J., Wang, T., Wang, G., Warrington, K. J., Watts, R. A., Wawrzycka-Adamczyk, K., Weeratunga, P., Weisman, M. H., Wickramasinghe, S., Williams, M., Wojcik, K., Woodruff, L., Xenitidis, T., Yamada, H., Yamagata, K., Yee, C. -S., Yoon, M., Yoshida, K., Yoshifuji, H., Ytterberg, S. R., Yumura, W., Zayed, H., Zeng, X., Zhao, M. -H., Zugaj, A., Zuk, J., İç Hastalıkları, Clinical Haematology, and Translational Immunology Groningen (TRIGR)

Subjects
Male, Outcome, Antineutrophil Cytoplasmic, 030232 urology & nephrology, 0302 clinical medicine, Risk Factors, 80 and over, Pharmacology (medical), Age of Onset, Young adult, Aged, 80 and over, education.field_of_study, age, anti-neutrophil cytoplasmic antibody-associated vasculitis, outcome, Adolescent, Adult, Aged, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Antibodies, Antineutrophil Cytoplasmic, Female, Humans, Middle Aged, Morbidity, Prognosis, Retrospective Studies, Risk Assessment, Survival Rate, United Kingdom, Young Adult, Vasculitis, Systemic vasculitis, medicine.medical_specialty, Population, anti-neutrophil cytoplasmic antibody–associated vasculitis, Antibodies, 03 medical and health sciences, Rheumatology, Internal medicine, medicine, education, Anti-neutrophil cytoplasmic antibody–associated vasculitis, Survival rate, Anti-neutrophil cytoplasmic antibody, 030203 arthritis & rheumatology, business.industry, Retrospective cohort study, medicine.disease, Age of onset, business
Abstract

Objectives ANCA-associated vasculitis (AAV) can affect all age groups. We aimed to show that differences in disease presentation and 6 month outcome between younger- and older-onset patients are still incompletely understood. Methods We included patients enrolled in the Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS) study between October 2010 and January 2017 with a diagnosis of AAV. We divided the population according to age at diagnosis: Results A total of 1338 patients with AAV were included: 66% had disease onset at Conclusion Within 6 months of diagnosis of AAV, patients >65 years of age display a different pattern of organ involvement and an increased risk of significant damage and mortality compared with younger patients.

Published
2021

25. 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

27. POS0246 SEQUENTIAL RITUXIMAB AND MEPOLIZUMAB IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

Author

Bettiol, A., primary, Urban, M. L., additional, Bello, F., additional, Fiori, D., additional, Mattioli, I., additional, Lopalco, G., additional, Iannone, F., additional, Egan, A., additional, Moroni, L., additional, Dagna, L., additional, Caminati, M., additional, Negrini, S., additional, Cameli, P., additional, Folci, M., additional, Toniati, P., additional, Padoan, R., additional, Flossmann, O., additional, Solans-Laqué, R., additional, Losappio, L., additional, Schroeder, J., additional, André, M., additional, Moi, L., additional, Parronchi, P., additional, Conti, F., additional, Sciascia, S., additional, Jayne, D., additional, Vaglio, A., additional, and Emmi, G., additional

Published
2022
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31. POS1214 COVID-19 VACCINATION RATE AND SAFETY PROFILE IN PATIENTS AFFECTED BY MIXED CRYOGLOBULINEMIC VASCULITIS.

Author

Vacchi, C., primary, Testoni, S., additional, Visentini, M., additional, Zani, R., additional, Lauletta, G., additional, Gragnani, L., additional, Filippini, D. A., additional, Mazzaro, C., additional, Fraticelli, P., additional, Quartuccio, L., additional, Padoan, R., additional, Castelnovo, L., additional, Zignego, A. L., additional, Ferri, C., additional, Hoxha, A., additional, Salvarani, C., additional, Monti, G., additional, Galli, M., additional, and Sebastiani, M., additional

Published
2022
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32. 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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33. 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

36. [Italian Cystic Fibrosis Registry (ICFR). Report 2019-2020]

Author

Campagna, G., Amato, A., Majo, F., Ferrari, G., Quattrucci, S., Padoan, R., Floridia, G., Salvatore, D., Carnovale, V., Puppo Fornaro, G., Angiolillo, A., Badolato, R., Battistini, F., Bernardi, M. A., Bertasi, S., Bignamin, I., Caloiero, M., Cannata, L., Carnicella, A., Castellani, C., Ciciretti, M. A., Cimino, G., Cipolli, M., Cirilli, N., Collura, M., Colombo, C., De Venuto, D., Di Sabatino, M, Donati, V., Fabrizi, B., Ficili, F., Francalanci, M., Giordano, P., Iansa, P., Laezza, C., Leonardi, S., Lucanto, M. C., Lucidi, V., Macchiaroli, A. M., Manca, A., Maschio, M., Mascotto, D., Mencarini, V., Messore, B., Moretti, P., Negri, A., Pantano, S., Palladino, N., Pintani, E., Pisano, G., Pisi, G., Pizzamiglio, G., Pradal, U., Raia, V., Redemagni, A., Ripani, P., Ros, M., Rotolo, N., Rottigni, S., Salvatore, M., Scarlata, A., Serio, L., Spaggiari, C., Taccetti, G., Taruscio, D., and Vitullo, P

Subjects
Adult, Male, CF referral centre, Adolescent, Cystic Fibrosis, [delta]F508, COVID-19, FEV, registry, BMI, Young Adult, CF support centre, Italy, cystic fibrosis, Child, Child, Preschool, Female, Humans, Pandemics, Registries, Preschool
Published
2022

37. The Italian Society of Rheumatology clinical practice guidelines for the management of large vessel vasculitis

Author

Ughi, N., primary, Padoan, R., additional, Crotti, C., additional, Sciascia, S., additional, Carrara, G., additional, Zanetti, A., additional, Rozza, D., additional, Monti, S., additional, Camellino, D., additional, Muratore, F., additional, Emmi, G., additional, Quartuccio, L., additional, Morbelli, S., additional, El Aoufy, K., additional, Tonolo, S., additional, Caporali, R., additional, De Vita, S., additional, Salvarani, C., additional, and Cimmino, M.A., additional

Published
2022
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38. 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

39. Italian cystic fibrosis registry (ICFR): Report 2017-2018

Author

Campagna, G., Amato, A., Majo, F., Ferrari, G., Quattrucci, S., Padoan, R., Floridia, G., Salvatore, D., Carnovale, V., Fornaro, G. P., Taruscio, D., Salvatore, M., Angiolillo, A., Baldo, E., Battistini, F., Bernardi, M. A., Bertasi, S., Bignamini, E., Bisogno, A., Braggion, C., Caloiero, M., Cannata, L., Carnicella, A., Cellini, C., Ciciretti, M. A., Cimino, G., Cipolli, M., Cirilli, N., Collura, M., Colombo, C., Sabatino, M. D., Donati, V., Fabrizi, B., Ficili, F., Francalanci, M., Iansa, P., Laezza, C., Leonardi, S., Lucanto, M. C., Lucidi, V., Macchiaroli, A. M., Manca, A., Maschio, M., Mascotto, D., Mencarini, V., Messore, B., Minicucci, L., Moretti, P., Negri, A., Pantano, S., Palladino, N., Pintani, E., Pisano, G., Pisi, G., Pizzamiglio, G., Quattromano, E., Raia, V., Redemagni, A., Ros, M., Rotolo, N., Rottigni, S., Scarlata, A., Spaggiari, C., Taccetti, G., Vassanelli, C., and Vitullo, P.

Subjects
Adult, Male, CF referral centre, Registry, Adolescent, Cystic Fibrosis, [delta]F508, Cystic Fibrosis Transmembrane Conductance Regulator, FEV, BMI, Young Adult, CF support centre, Italy, Cystic fibrosis, Child, Female, Humans, Registries, Lung Transplantation
Published
2021

40. Quantitative assessment of impact and sensitivity of imaging spectroscopy for monitoring of ageing of archival documents

Author

Padoan, R. (author), Klein, Marvin E. (author), Groves, R.M. (author), de Bruin, Gerrit (author), Steemers, Ted A.G. (author), Strlič, Matija (author), Padoan, R. (author), Klein, Marvin E. (author), Groves, R.M. (author), de Bruin, Gerrit (author), Steemers, Ted A.G. (author), and Strlič, Matija (author)

Abstract

Ageing of historical documents often results in changes in the optical properties of the constituent materials. Imaging spectroscopy (IS) can be a valuable tool for monitoring of such changes, if the method fulfils two important conditions. Firstly, compared to natural ageing, the accumulated light dose from repeated measurements of the monitored document must not induce any significant degradation. Secondly, the monitoring instrumentation and procedures should be sensitive enough to detect changes in the materials before they become visible. We present experimental methods to evaluate the suitability of IS instrumentation for monitoring purposes. In the first set of experiments, the impact of repeated monitoring measurements was determined using a set of Blue Wool Standard materials. In the second set of experiments, the capability of the instrument to detect spectral changes was tested using ISO standard materials and several documents representative of European archive collections. It is concluded that the tested hyperspectral instrument is suitable for monitoring of the colour change of documents during display. The described experimental approach can be recommended to test the suitability of other imaging spectroscopy instruments for monitoring applications., Structural Integrity & Composites

Published
2021
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41. 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

43. Italian Cystic Fibrosis Registry (ICFR). Report 2015-2016

Author

Giordani, B., Amato, A., Majo, F., Ferrari, G., Quattrucci, S., Minicucci, L., Padoan, R., Floridia, G., Salvatore, D., Carnovale, V., Puppo Fornaro, G., Taruscio, D., Salvatore, M., Albera, C, Amato, A, Angiolillo, A, Baldo, E, Battistini, F, Bena, C, Bernardi, Mg, Bertasi, S, Bignamini, E, Bisogno, A, Braggion, C, Cannata, L, Carnicella, A, Carnovale, V, Ciciretti, Ma, Cipolli, M, Cirilli, N, Colombo, C, Cucchiara, S, Di Sabatino, M, Di Stefano, E, Fabrizi, B, Ferrari, G, Ficili, F, Floridia, G, Francalanci, M, Gagliardini, R, Giordani, B, Iansa, P, Laezza, C, Leonardi, S, Lucanto, Mc, Lucidi, V, Macchiaroli, Am, Magazzù, G, Majo, F, Manca, A, Maschio, M, Mascotto, D, Mencarini, V, Minicucci, L, Moretti, P, Negri, A, Padoan, R, Palladino, N, Pintani, E, Pisano, G, Pisi, G, Pizzamiglio, G, Quattrucci, S, Raia, V, Ratclif, L, Ros, M, Rotolo, N, Salvatore, D, Salvatore, M, Spaggiari, C, Taruscio, D, Tonelli, T, Tuccio, G, Vassanelli, C, Vitullo, P, and Zavataro L, .

Published
2019

47. P083 Clinical progression of SARS-CoV-2 infection in people with cystic fibrosis: a global observational study

Author

McClenaghan, E., primary, Cosgriff, R., additional, Brownlee, K., additional, Ahern, S., additional, Burgel, P.-R., additional, Byrnes, C.A., additional, Colombo, C., additional, Corvol, H., additional, Cheng, S.Y., additional, Daneau, G., additional, Elbert, A., additional, Faro, A., additional, Goss, C.H., additional, Gulmans, V., additional, Gutierrez, H., additional, de Monestrol, I., additional, Jung, A., additional, Nährlich, L., additional, Kashirskaya, N., additional, Marshall, B.C., additional, McKone, E., additional, Middleton, P.G., additional, Mondejar-Lopez, P., additional, Pastor-Vivero, M.D., additional, Padoan, R., additional, Rizvi, S., additional, Ruseckaite, R., additional, Salvatore, M., additional, Stephenson, A.L., additional, da Silva Filho, L.V.R., additional, Melo, J., additional, Zampoli, M., additional, Abdrakhmanov, O., additional, Harutyunyan, S., additional, and Carr, S.B., additional

Published
2021
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50. SARS-CoV-2 infection in patients with autoimmune rheumatic diseases in northeast Italy: A cross-sectional study on 916 patients

Author

Zen, M., primary, Fuzzi, E., additional, Astorri, D., additional, Saccon, F., additional, Padoan, R., additional, Ienna, L., additional, Cozzi, G., additional, Depascale, R., additional, Zanatta, E., additional, Gasparotto, M., additional, Benvenuti, F., additional, Bindoli, S., additional, Gatto, M., additional, Felicetti, M., additional, Ortolan, A., additional, Campaniello, D., additional, Larosa, M., additional, Lorenzin, M., additional, Ramonda, R., additional, Sfriso, P., additional, Schiavon, F., additional, Iaccarino, L., additional, and Doria, A., additional

Published
2020
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