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6. Risk for cancer development in familial Mediterranean fever and associated predisposing factors: an ambidirectional cohort study from the international AIDA Network registries

Author

Vitale, A, Caggiano, V, Tufan, A, Ragab, G, Batu, Ed, Portincasa, P, Aragona, E, Sota, J, Conti, G, De Paulis, A, Rigante, Donato, Olivieri, An, S ̧ahin, A, La Torre, F, Lopalco, G, Cattalini, M, Maggio, Mc, Insalaco, A, Sfikakis, Pp, Verrecchia, Elena, Yildirim, D, Kucuk, H, Kardas, Rc, Laymouna, Ah, Ghanema, M, Saad, Ma, Sener, S, Ercan Emreol, H, Ozen, S, Jaber, N, Khalil, M, Di Ciaula, A, Gaggiano, C, Malizia, G, Affronti, A, Patroniti, S, Romeo, M, Sbalchiero, J, Della Casa, F, Mormile, I, Silvaroli, Sara, Gicchino, Mf, Çelik, Nç, Tarsia, M, Karamanakos, A, Hernández-Rodríguez, J, Parronchi, P, Opris-Belinski, D, Barone, P, Recke, A, Costi, S, Sfriso, P, Giardini, Ham, Gentileschi, S, Wiesik-Szewczyk, E, Vasi, I, Loconte, R, Jahnz-Różyk, K, Martín-Nares, E, Torres-Ruiz, J, Cauli, A, Conforti, A, Emmi, G, Li Gobbi, F, Biasi, Gr, Terribili, R, Ruscitti, P, Del Giudice, E, Tharwat, S, Brucato, Al, Ogunjimi, B, Hinojosa-Azaola, A, Balistreri, A, Fabiani, C, Frediani, B, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Verrecchia E, Silvaroli S, Vitale, A, Caggiano, V, Tufan, A, Ragab, G, Batu, Ed, Portincasa, P, Aragona, E, Sota, J, Conti, G, De Paulis, A, Rigante, Donato, Olivieri, An, S ̧ahin, A, La Torre, F, Lopalco, G, Cattalini, M, Maggio, Mc, Insalaco, A, Sfikakis, Pp, Verrecchia, Elena, Yildirim, D, Kucuk, H, Kardas, Rc, Laymouna, Ah, Ghanema, M, Saad, Ma, Sener, S, Ercan Emreol, H, Ozen, S, Jaber, N, Khalil, M, Di Ciaula, A, Gaggiano, C, Malizia, G, Affronti, A, Patroniti, S, Romeo, M, Sbalchiero, J, Della Casa, F, Mormile, I, Silvaroli, Sara, Gicchino, Mf, Çelik, Nç, Tarsia, M, Karamanakos, A, Hernández-Rodríguez, J, Parronchi, P, Opris-Belinski, D, Barone, P, Recke, A, Costi, S, Sfriso, P, Giardini, Ham, Gentileschi, S, Wiesik-Szewczyk, E, Vasi, I, Loconte, R, Jahnz-Różyk, K, Martín-Nares, E, Torres-Ruiz, J, Cauli, A, Conforti, A, Emmi, G, Li Gobbi, F, Biasi, Gr, Terribili, R, Ruscitti, P, Del Giudice, E, Tharwat, S, Brucato, Al, Ogunjimi, B, Hinojosa-Azaola, A, Balistreri, A, Fabiani, C, Frediani, B, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Verrecchia E, and Silvaroli S

Abstract

Objective: Inflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies. Familial Mediterranean fever (FMF) is a severe systemic inflammatory condition and also represents the archetype of innate immunity deregulation. Therefore, the aim of this study is to investigate the risk for cancer development in FMF. Methods: The risk ratio (RR) for malignancies was separately compared between FMF patients and fibromyalgia subjects, Still’s disease patients and Behçet’s disease patients. Clinical variables associated with cancer development in FMF patients were searched through binary logistic regression. Results: 580 FMF patients and 102 fibromyalgia subjects, 1012 Behçet’s disease patients and 497 Still’s disease patients were enrolled. The RR for the occurrence of malignant neoplasms was 0.26 (95% Confidence Interval [CI.] 0.10-0.73, p=0.006) in patients with FMF compared to fibromyalgia subjects; the RR for the occurrence of malignant cancer was 0.51 (95% CI. 0.23-1.16, p=0.10) in FMF compared to Still’s disease and 0.60 (95% CI. 0.29-1.28, p=0.18) in FMF compared to Behçet’s disease. At logistic regression, the risk of occurrence of malignant neoplasms in FMF patients was associated with the age at disease onset (b1 = 0.039, 95% CI. 0.001-0.071, p=0.02), the age at the diagnosis (b1 = 0.048, 95% CI. 0.039-0.085, p=0.006), the age at the enrolment (b1 = 0.05, 95% CI. 0.007-0.068, p=0.01), the number of attacks per year (b1 = 0.011, 95% CI. 0.001- 0.019, p=0.008), the use of biotechnological agents (b1 = 1.77, 95% CI. 0.43-3.19, p=0.009), the use of anti-IL-1 agents (b1 = 2.089, 95% CI. 0.7- 3.5, p=0.002). Conclusions: The risk for cancer is reduced in Caucasic FMF patients; however, when malignant neoplasms occur, this is more frequent in FMF cases suffering from a severe disease phenotype and presenting a colchicine-resistant disease.

Published
2024

7. Preliminary data revealing efficacy of Streptococcus salivarius K12 (SSK12) in Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome: a multicenter study from the AIDA Network PFAPA syndrome registry

Author

La Torre, F, Sota, J, Insalaco, A, Conti, G, Del Giudice, E, Lubrano, R, Breda, L, Maggio, Mc, Civino, A, Mastrorilli, V, Loconte, R, Natale, Mf, Celani, C, Romeo, M, Patroniti, S, Gentile, C, Vitale, A, Caggiano, V, Gaggiano, C, Diomeda, F, Cattalini, M, Lopalco, G, Emmi, G, Parronchi, P, Gentileschi, S, Cardinale, F, Aragona, E, Shahram, F, Marino, A, Barone, P, Moscheo, C, Ozkiziltas, B, Carubbi, F, Alahmed, O, Iezzi, Ludovica, Ogunjimi, B, Mauro, A, Tarsia, M, Mahmoud, Aaa, Mayrink Giardini, Ham, Sfikakis, Pp, Laskari, K, Więsik-Szewczyk, E, Hernàndez-Rodriguez, J, Frediani, B, Gòmez-Caverzaschi, V, Tufan, A, Almaghlouth, Ia, Balistreri, A, Ragab, G, Fabiani, C, Cantarini, L, Rigante, Donato, La Torre F, Sota J, Insalaco A, Conti G, Del Giudice E, Lubrano R, Breda L, Maggio MC, Civino A, Mastrorilli V, Loconte R, Natale MF, Celani C, Romeo M, Patroniti S, Gentile C, Vitale A, Caggiano V, Gaggiano C, Diomeda F, Cattalini M, Lopalco G, Emmi G, Parronchi P, Gentileschi S, Cardinale F, Aragona E, Shahram F, Marino A, Barone P, Moscheo C, Ozkiziltas B, Carubbi F, Alahmed O, Iezzi L, Ogunjimi B, Mauro A, Tarsia M, Mahmoud AAA, Mayrink Giardini HAM, Sfikakis PP, Laskari K, Więsik-Szewczyk E, Hernàndez-Rodriguez J, Frediani B, Gòmez-Caverzaschi V, Tufan A, Almaghlouth IA, Balistreri A, Ragab G, Fabiani C, Cantarini L, Rigante D (ORCID:0000-0001-7032-7779), La Torre, F, Sota, J, Insalaco, A, Conti, G, Del Giudice, E, Lubrano, R, Breda, L, Maggio, Mc, Civino, A, Mastrorilli, V, Loconte, R, Natale, Mf, Celani, C, Romeo, M, Patroniti, S, Gentile, C, Vitale, A, Caggiano, V, Gaggiano, C, Diomeda, F, Cattalini, M, Lopalco, G, Emmi, G, Parronchi, P, Gentileschi, S, Cardinale, F, Aragona, E, Shahram, F, Marino, A, Barone, P, Moscheo, C, Ozkiziltas, B, Carubbi, F, Alahmed, O, Iezzi, Ludovica, Ogunjimi, B, Mauro, A, Tarsia, M, Mahmoud, Aaa, Mayrink Giardini, Ham, Sfikakis, Pp, Laskari, K, Więsik-Szewczyk, E, Hernàndez-Rodriguez, J, Frediani, B, Gòmez-Caverzaschi, V, Tufan, A, Almaghlouth, Ia, Balistreri, A, Ragab, G, Fabiani, C, Cantarini, L, Rigante, Donato, La Torre F, Sota J, Insalaco A, Conti G, Del Giudice E, Lubrano R, Breda L, Maggio MC, Civino A, Mastrorilli V, Loconte R, Natale MF, Celani C, Romeo M, Patroniti S, Gentile C, Vitale A, Caggiano V, Gaggiano C, Diomeda F, Cattalini M, Lopalco G, Emmi G, Parronchi P, Gentileschi S, Cardinale F, Aragona E, Shahram F, Marino A, Barone P, Moscheo C, Ozkiziltas B, Carubbi F, Alahmed O, Iezzi L, Ogunjimi B, Mauro A, Tarsia M, Mahmoud AAA, Mayrink Giardini HAM, Sfikakis PP, Laskari K, Więsik-Szewczyk E, Hernàndez-Rodriguez J, Frediani B, Gòmez-Caverzaschi V, Tufan A, Almaghlouth IA, Balistreri A, Ragab G, Fabiani C, Cantarini L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: To evaluate the potential role of Streptococcus salivarius K12 (SSK12) in controlling febrile flares in patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Further aims were to assess the impact of SSK12 on (i) flare duration, (ii) variation in the degree of the highest body temperature during flares, (iii) steroid-sparing effect, and (iv) change of PFAPA accompanying symptoms before and after SSK12 introduction. Patients and methods: The medical charts from 85 pediatric patients with PFAPA syndrome (49 males and 36 females) enrolled in the AIDA registry and treated with SSK12 for a median period of 6.00±7.00 months in the period between September 2017 and May 2022 were examined. Children recruited had a median time of disease duration of 19.00±28.00 months. Results: The number of febrile flares significantly decreased comparing the 12 months before [median (IQR), 13.00 (6.00)] and after SSK12 initiation [median (IQR), 5.50 (8.00), p<0.001]. The duration of fever was significantly reduced from 4.00 (2.00) days to 2.00 (2.00) days [p<0.001]. Similarly, the highest temperature in °C was found significantly lower in the last follow-up assessment [median (IQR), 39.00 (1.00)] compared to the period prior to SSK12 start [median (IQR), 40.00 (1.00), p<0.001]. Steroid load (mg/year) of betamethasone (or any equivalent steroid) significantly decreased between 12 months before treatment with SSK12 [median (IQR), 5.00 (8.00) mg/year] and the last follow-up visit [median (IQR), 2.00 (4.00) mg/year, p<0.001]. The number of patients experiencing symptoms including pharyngitis/tonsillitis (p<0.001), oral aphthae (p<0.001) and cervical lymphadenopathy (p<0.001) significantly decreased following SSK12. Conclusion: SSK12 prophylaxis given for at least 6.00±7.00 months was found to reduce febrile flares of PFAPA syndrome: in particular, it halved the total number per year of fever flares, shortened the

Published
2023

8. The administration of methotrexate in patients with Still’s disease, “real-life” findings from AIDA Network Still Disease Registry

Author

362., Ruscitti P, Sota, J, Vitale, A, Lopalco, G, Iannone, F, Morrone, M, Giardini, Ham, D'Agostin, Ma, Antonelli, Ipb, Almaghlouth, I, Asfina, Kn, Khalil, N, Sfikakis, Pp, Laskari, K, Tektonidou, M, Ciccia, F, Iacono, D, Riccio, F, Ragab, G, Hussein, Ma, Govoni, M, Ruffilli, F, Direskeneli, H, Alibaz-Oner, F, Giacomelli, R, Navarini, L, Bartoloni, E, Riccucci, I, Martín-Nares, E, Torres-Ruiz, J, Cipriani, P, Di Cola, I, Hernández-Rodríguez, J, Gómez-Caverzaschi, V, Dagna, L, Tomelleri, A, Makowska, J, Brzezinska, O, Iagnocco, A, Bellis, E, Caggiano, V, Gaggiano, C, Tarsia, M, Mormile, I, Emmi, G, Sfriso, P, Monti, S, Erten, Ş, Del Giudice, E, Lubrano, R, Conti, G, Olivieri, An, Lo Gullo, A, Tharwat, S, Karamanakos, A, Gidaro, A, Maggio, Mc, La Torre, F, Cardinale, F, Ogunjimi, B, Maier, A, Sebastiani, Gd, Opris-Belinski, D, Frassi, M, Viapiana, O, Bizzi, E, Carubbi, F, Fotis, L, Tufan, A, Kardas, Rc, Więsik-Szewczyk, E, Jahnz-Różyk, K, Fabiani, C, Frediani, B, Balistreri, A, Rigante, Donato, Cantarini, L, 362. Ruscitti P, Sota J, Vitale A, Lopalco G, Iannone F, Morrone M, Giardini HAM, D'Agostin MA, Antonelli IPB, Almaghlouth I, Asfina KN, Khalil N, Sfikakis PP, Laskari K, Tektonidou M, Ciccia F, Iacono D, Riccio F, Ragab G, Hussein MA, Govoni M, Ruffilli F, Direskeneli H, Alibaz-Oner F, Giacomelli R, Navarini L, Bartoloni E, Riccucci I, Martín-Nares E, Torres-Ruiz J, Cipriani P, Di Cola I, Hernández-Rodríguez J, Gómez-Caverzaschi V, Dagna L, Tomelleri A, Makowska J, Brzezinska O, Iagnocco A, Bellis E, Caggiano V, Gaggiano C, Tarsia M, Mormile I, Emmi G, Sfriso P, Monti S, Erten Ş, Del Giudice E, Lubrano R, Conti G, Olivieri AN, Lo Gullo A, Tharwat S, Karamanakos A, Gidaro A, Maggio MC, La Torre F, Cardinale F, Ogunjimi B, Maier A, Sebastiani GD, Opris-Belinski D, Frassi M, Viapiana O, Bizzi E, Carubbi F, Fotis L, Tufan A, Kardas RC, Więsik-Szewczyk E, Jahnz-Różyk K, Fabiani C, Frediani B, Balistreri A, Rigante D (ORCID:0000-0001-7032-7779), Cantarini L, 362., Ruscitti P, Sota, J, Vitale, A, Lopalco, G, Iannone, F, Morrone, M, Giardini, Ham, D'Agostin, Ma, Antonelli, Ipb, Almaghlouth, I, Asfina, Kn, Khalil, N, Sfikakis, Pp, Laskari, K, Tektonidou, M, Ciccia, F, Iacono, D, Riccio, F, Ragab, G, Hussein, Ma, Govoni, M, Ruffilli, F, Direskeneli, H, Alibaz-Oner, F, Giacomelli, R, Navarini, L, Bartoloni, E, Riccucci, I, Martín-Nares, E, Torres-Ruiz, J, Cipriani, P, Di Cola, I, Hernández-Rodríguez, J, Gómez-Caverzaschi, V, Dagna, L, Tomelleri, A, Makowska, J, Brzezinska, O, Iagnocco, A, Bellis, E, Caggiano, V, Gaggiano, C, Tarsia, M, Mormile, I, Emmi, G, Sfriso, P, Monti, S, Erten, Ş, Del Giudice, E, Lubrano, R, Conti, G, Olivieri, An, Lo Gullo, A, Tharwat, S, Karamanakos, A, Gidaro, A, Maggio, Mc, La Torre, F, Cardinale, F, Ogunjimi, B, Maier, A, Sebastiani, Gd, Opris-Belinski, D, Frassi, M, Viapiana, O, Bizzi, E, Carubbi, F, Fotis, L, Tufan, A, Kardas, Rc, Więsik-Szewczyk, E, Jahnz-Różyk, K, Fabiani, C, Frediani, B, Balistreri, A, Rigante, Donato, Cantarini, L, 362. Ruscitti P, Sota J, Vitale A, Lopalco G, Iannone F, Morrone M, Giardini HAM, D'Agostin MA, Antonelli IPB, Almaghlouth I, Asfina KN, Khalil N, Sfikakis PP, Laskari K, Tektonidou M, Ciccia F, Iacono D, Riccio F, Ragab G, Hussein MA, Govoni M, Ruffilli F, Direskeneli H, Alibaz-Oner F, Giacomelli R, Navarini L, Bartoloni E, Riccucci I, Martín-Nares E, Torres-Ruiz J, Cipriani P, Di Cola I, Hernández-Rodríguez J, Gómez-Caverzaschi V, Dagna L, Tomelleri A, Makowska J, Brzezinska O, Iagnocco A, Bellis E, Caggiano V, Gaggiano C, Tarsia M, Mormile I, Emmi G, Sfriso P, Monti S, Erten Ş, Del Giudice E, Lubrano R, Conti G, Olivieri AN, Lo Gullo A, Tharwat S, Karamanakos A, Gidaro A, Maggio MC, La Torre F, Cardinale F, Ogunjimi B, Maier A, Sebastiani GD, Opris-Belinski D, Frassi M, Viapiana O, Bizzi E, Carubbi F, Fotis L, Tufan A, Kardas RC, Więsik-Szewczyk E, Jahnz-Różyk K, Fabiani C, Frediani B, Balistreri A, Rigante D (ORCID:0000-0001-7032-7779), and Cantarini L

Abstract

Objectives: To describe clinical characteristics of patients with Still's disease treated with methotrexate (MTX) and to assess drug effectiveness evaluating change in disease activity, reduction of inflammatory markers, and glucocorticoid (GC)-sparing effect. Methods: Patients with Still's disease treated with MTX were assessed among those included in AIDA Network Still Disease Registry. Results: In this registry, 171 patients with Still's disease were treated with MTX (males 43.3%, age 37.1 ± 16.0 years). They were mainly characterised by joint features and fever without a prominent multiorgan involvement. MTX was administered with GCs in 68.4% of patients, with other conventional synthetic DMARDs in 6.4%, and with biologic DMARDs in 25.1%. A significant reduction of the modified systemic score was observed, and 38.6% patients were codified as being in clinical remission at the end of follow-up. The concomitant administration of a biologic DMARD resulted a predictor of the clinical remission. Furthermore, a reduction of inflammatory markers and ferritin levels was observed following the administration of MTX. Additionally, a marked reduction of the dosage of concomitant GCs was identified, while 36.7% discontinued such drugs. Male gender appeared as a predictor of GC discontinuation. MTX was discontinued in 12.3% of patients because of adverse effects, and in 12.3% for lack of efficacy. Conclusions: Clinical characteristics of patients with Still's disease treated with MTX were described, mainly joint features and fever without a prominent multiorgan involvement. The clinical usefulness of MTX was reported in reducing the disease activity, decreasing the inflammatory markers, and as GC-sparing agent.

Published
2023

11. The AutoInflammatory Diseases Alliance Registry of monogenic autoinflammatory diseases

Author

Gaggiano, C, Vitale, A, Tufan, A, Ragab, G, Aragona, E, Wiesik-Szewczyk, E, Ait-Idir, D, Conti, G, Iezzi, L, Maggio, Mc, Cattalini, M, La Torre, F, Lopalco, G, Verrecchia, E, de Paulis, A, Sahin, A, Insalaco, A, Sfikakis, Pp, Marino, A, Frassi, M, Ogunjimi, B, Opris-Belinski, D, Parronchi, P, Emmi, G, Shahram, F, Ciccia, F, Piga, M, Hernández-Rodríguez, J, Pereira, Rmr, Alessio, M, Naddei, R, Olivieri, An, Giudice, Ed, Sfriso, P, Ruscitti, P, Gobbi, Fl, Kucuk, H, Sota, J, Hussein, Ma, Malizia, G, Jahnz-Różyk, K, Sari-Hamidou, R, Romeo, M, Ricci, F, Cardinale, F, Iannone, F, Casa, Fd, Natale, Mf, Laskari, K, Giani, T, Franceschini, F, Sabato, V, Yildirim, D, Caggiano, V, Hegazy, Mt, Marzo, Rd, Kucharczyk, A, Khellaf, G, Tarsia, M, Almaghlouth, Ia, Laymouna, Ah, Mastrorilli, V, Dotta, L, Benacquista, L, Grosso, S, Crisafulli, F, Parretti, V, Giordano, Hf, Mahmoud, Aaa, Nuzzolese, R, Musso, M, Chighizola, Cb, Gentileschi, S, Morrone, M, Cola, Id, Spedicato, V, Giardini, Ham, Vasi, I, Renieri, A, Fabbiani, A, Mencarelli, Ma, Frediani, B, Balistreri, A, Tosi, Gm, Fabiani, C, Lidar, M, Rigante, D, Cantarini, L, Gaggiano C, Vitale A, Tufan A, Ragab G, Aragona E, Wiesik-Szewczyk E, Ait-Idir D, Iezzi L, Maggio MC, Cattalini M, La Torre F, Lopalco G, Verrecchia E, de Paulis A, Sahin A, Insalaco A, Sfikakis PP, Marino A, Frassi M, Ogunjimi B, Opris-Belinski D, Parronchi P, Emmi G, Shahram F, Ciccia F, Piga M, Hernández-Rodríguez J, Pereira RMR, Alessio M, Naddei R, Olivieri AN, Giudice ED, Sfriso P, Ruscitti P, Gobbi FL, Kucuk H, Sota J, Hussein MA, Malizia G, Jahnz-Różyk K, Sari-Hamidou R, Romeo M, Ricci F, Cardinale F, Iannone F, Casa FD, Natale MF, Laskari K, Giani T, Franceschini F, Sabato V, Yildirim D, Caggiano V, Hegazy MT, Marzo RD, Kucharczyk A, Khellaf G, Tarsia M, Almaghlouth IA, Laymouna AH, Mastrorilli V, Dotta L, Benacquista L, Grosso S, Crisafulli F, Parretti V, Giordano HF, Mahmoud AAA, Nuzzolese R, Musso M, Chighizola CB, Gentileschi S, Morrone M, Cola ID, Spedicato V, Giardini HAM, Vasi I, Renieri A, Fabbiani A, Mencarelli MA, Frediani B, Balistreri A, Tosi GM, Fabiani C, Lidar M, Rigante D (ORCID:0000-0001-7032-7779), Cantarini L, Conti G, Gaggiano, C, Vitale, A, Tufan, A, Ragab, G, Aragona, E, Wiesik-Szewczyk, E, Ait-Idir, D, Conti, G, Iezzi, L, Maggio, Mc, Cattalini, M, La Torre, F, Lopalco, G, Verrecchia, E, de Paulis, A, Sahin, A, Insalaco, A, Sfikakis, Pp, Marino, A, Frassi, M, Ogunjimi, B, Opris-Belinski, D, Parronchi, P, Emmi, G, Shahram, F, Ciccia, F, Piga, M, Hernández-Rodríguez, J, Pereira, Rmr, Alessio, M, Naddei, R, Olivieri, An, Giudice, Ed, Sfriso, P, Ruscitti, P, Gobbi, Fl, Kucuk, H, Sota, J, Hussein, Ma, Malizia, G, Jahnz-Różyk, K, Sari-Hamidou, R, Romeo, M, Ricci, F, Cardinale, F, Iannone, F, Casa, Fd, Natale, Mf, Laskari, K, Giani, T, Franceschini, F, Sabato, V, Yildirim, D, Caggiano, V, Hegazy, Mt, Marzo, Rd, Kucharczyk, A, Khellaf, G, Tarsia, M, Almaghlouth, Ia, Laymouna, Ah, Mastrorilli, V, Dotta, L, Benacquista, L, Grosso, S, Crisafulli, F, Parretti, V, Giordano, Hf, Mahmoud, Aaa, Nuzzolese, R, Musso, M, Chighizola, Cb, Gentileschi, S, Morrone, M, Cola, Id, Spedicato, V, Giardini, Ham, Vasi, I, Renieri, A, Fabbiani, A, Mencarelli, Ma, Frediani, B, Balistreri, A, Tosi, Gm, Fabiani, C, Lidar, M, Rigante, D, Cantarini, L, Gaggiano C, Vitale A, Tufan A, Ragab G, Aragona E, Wiesik-Szewczyk E, Ait-Idir D, Iezzi L, Maggio MC, Cattalini M, La Torre F, Lopalco G, Verrecchia E, de Paulis A, Sahin A, Insalaco A, Sfikakis PP, Marino A, Frassi M, Ogunjimi B, Opris-Belinski D, Parronchi P, Emmi G, Shahram F, Ciccia F, Piga M, Hernández-Rodríguez J, Pereira RMR, Alessio M, Naddei R, Olivieri AN, Giudice ED, Sfriso P, Ruscitti P, Gobbi FL, Kucuk H, Sota J, Hussein MA, Malizia G, Jahnz-Różyk K, Sari-Hamidou R, Romeo M, Ricci F, Cardinale F, Iannone F, Casa FD, Natale MF, Laskari K, Giani T, Franceschini F, Sabato V, Yildirim D, Caggiano V, Hegazy MT, Marzo RD, Kucharczyk A, Khellaf G, Tarsia M, Almaghlouth IA, Laymouna AH, Mastrorilli V, Dotta L, Benacquista L, Grosso S, Crisafulli F, Parretti V, Giordano HF, Mahmoud AAA, Nuzzolese R, Musso M, Chighizola CB, Gentileschi S, Morrone M, Cola ID, Spedicato V, Giardini HAM, Vasi I, Renieri A, Fabbiani A, Mencarelli MA, Frediani B, Balistreri A, Tosi GM, Fabiani C, Lidar M, Rigante D (ORCID:0000-0001-7032-7779), Cantarini L, and Conti G

Abstract

Objective: The present manuscript aims to describe an international, electronic-based, user-friendly and interoperable patient registry for monogenic autoinflammatory diseases (mAIDs), developed in the contest of the Autoinflammatory Diseases Alliance (AIDA) Network. Methods: This is an electronic platform, based on the Research Electronic Data Capture (REDCap) tool, used for real-world data collection of demographics, clinical, laboratory, instrumental and socioeconomic data of mAIDs patients. The instrument has flexibility, may change over time based on new scientific acquisitions, and communicate potentially with other similar registries; security, data quality and data governance are corner stones of the platform. Results: AIDA project will share knowledge and expertise on mAIDs. Since its start, 118 centers from 24 countries and 4 continents have joined the AIDA project. Fifty-nine centers have already obtained the approval from their local Ethics Committees. Currently, the platform counts 337 users (122 Principal Investigators, 210 Site Investigators, 2 Lead Investigators, and 3 data managers). The Registry collects baseline and follow-up data using 3,748 fields organized into 21 instruments, which include demographics, patient history, symptoms, trigger/risk factors, therapies, and healthcare information for mAIDs patients. Conclusions: The AIDA mAIDs Registry, acts both as a research tool for future collaborative real-life studies on mAIDs and as a service to connect all the figures called to participate. On this basis, the registry is expected to play a pivotal role in generating new scientific evidence on this group of rare diseases, substantially improving the management of patients, and optimizing the impact on the healthcare system. NCT 05200715 available at https://clinicaltrials.gov

Published
2022

12. 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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13. AB0164 SERUM LEVELS OF INTERLEUKIN-36 Α TO DISTINGUISH BEHÇET’S SYNDROME AND PSORIATIC ARTHRITIS

Author

Bettiol, A., primary, Fagni, F., additional, Mattioli, I., additional, Bagni, G., additional, Vitiello, G., additional, Grassi, A., additional, Della Bella, C., additional, Benagiano, M., additional, Troilo, A., additional, Holownia, K. S., additional, Simon, D., additional, Argento, F. R., additional, Sota, J., additional, Fabiani, C., additional, Becatti, M., additional, Fiorillo, C., additional, Schett, G., additional, Lopalco, G., additional, Cantarini, L., additional, Prisco, D., additional, Silvestri, E., additional, Emmi, G., additional, and D’elios, M. M., additional

Published
2023
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15. POS1172 GEOGRAPHICAL VARIABILITY OF ORGAN DAMAGE BURDEN IN A LARGE INTERNATIONAL MULTICENTER COHORT OF PATIENTS WITH BEHÇET’S SYNDROME: PRELIMINARY RESULTS OF THE PROBE STUDY

Author

Floris, A., primary, Chadli, S., additional, Lamouna, A., additional, Hegazy, M. T., additional, Yagiz Ozogul, Y., additional, Ozguler, Y., additional, Serpa Pinto, L., additional, Alikhani, M., additional, Oliveira, L., additional, Sota, J., additional, Lopalco, G., additional, Poddighe, D., additional, Kougkas, N., additional, Khabbazi Oskouei, A., additional, Laconi, R., additional, Wagner Silva de Souza, A., additional, Lo Monaco, A., additional, Espinosa, G., additional, Butendieck, R., additional, Govoni, M., additional, Issayeva, B., additional, Iannone, F., additional, Fabiani, C. M., additional, Cantarini, L., additional, Vasconcelos, C., additional, Shahram, F., additional, Correia, J., additional, Tazi Mezalek, Z., additional, Ragab, G., additional, Arnaud, L., additional, Cauli, A., additional, Hatemi, G., additional, and Piga, M., additional

Published
2023
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16. AB0431 CANAKINUMAB AS FIRST-LINE BIOLOGICAL THERAPY IN STILL’S DISEASE AND DIFFERENCES BETWEEN THE SYSTEMIC AND THE CHRONIC-ARTICULAR COURSES: REAL-LIFE EXPERIENCE FROM THE INTERNATIONAL AIDA REGISTRY

Author

Vitale, A., primary, Caggiano, V., additional, Maggio, M. C., additional, Lopalco, G., additional, Emmi, G., additional, Sota, J., additional, La Torre, F., additional, Ruscitti, P., additional, Bartoloni, E., additional, Conti, G., additional, Fabiani, C., additional, Mattioli, I., additional, Gaggiano, C., additional, Cardinale, F., additional, Dagna, L., additional, Campochiaro, C., additional, Giacomelli, R., additional, Balistreri, A., additional, Laskari, K., additional, Tufan, A., additional, Ragab, G., additional, Almaghlouth, I., additional, Więsik-Szewczyk, E., additional, Pereira, R. M., additional, Frediani, B., additional, Iannone, F., additional, Sfikakis, P., additional, and Cantarini, L., additional

Published
2023
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17. Axial spondyloarthritis in patients with recurrent fever attacks: data from the AIDA network registry for undifferentiated autoinflammatory diseases (USAIDs)

Author

Vitale, A, Caggiano, V, Silva, I, Oliveira, Dg, Ruscitti, P, Ciccia, F, Vasi, I, Tufan, A, Lopalco, G, Almaghlouth, Ia, Sota, J, Wiesik-Szewczyk, E, Gaggiano, C, Giardini, Ham, Spedicato, V, Ragab, G, Iannone, F, Balistreri, A, Frassi, M, Hernández-Rodríguez, J, Fabiani, C, Falsetti, P, Di Meglio, N, Frediani, B, Mazzei, Ma, Rigante, Donato, Faria, R, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Caggiano, V, Silva, I, Oliveira, Dg, Ruscitti, P, Ciccia, F, Vasi, I, Tufan, A, Lopalco, G, Almaghlouth, Ia, Sota, J, Wiesik-Szewczyk, E, Gaggiano, C, Giardini, Ham, Spedicato, V, Ragab, G, Iannone, F, Balistreri, A, Frassi, M, Hernández-Rodríguez, J, Fabiani, C, Falsetti, P, Di Meglio, N, Frediani, B, Mazzei, Ma, Rigante, Donato, Faria, R, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Background: Despite the recent advances in the field of autoinflammatory diseases, most patients with recurrent fever episodes do not have any defined diagnosis. The present study aims at describing a cohort of patients suffering from apparently unexplained recurrent fever, in whom non-radiographic axial spondylarthritis (SpA) represented the unique diagnosis identified after a complete clinical and radiologic assessment. Materials and methods: Patients' data were obtained from the international registry on Undifferentiated Systemic AutoInflammatory Diseases (USAIDs) developed by the AutoInflammatory Disease Alliance (AIDA) network. Results: A total of 54 patients with recurrent fever episodes were also affected by non-radiographic axial SpA according to the international classification criteria. SpA was diagnosed after the start of fever episodes in all cases; the mean age at the diagnosis of axial SpA was 39.9 ± 14.8 years with a diagnostic delay of 9.3 years. The highest body temperature reached during flares was 42°C, with a mean temperature of 38.8 ± 1.1°C. The most frequent manifestations associated to fever were: arthralgia in 33 (61.1%) cases, myalgia in 24 (44.4%) cases, arthritis in 22 (40.7%) cases, headache in 15 (27.8%) cases, diarrhea in 14 (25.9%) cases, abdominal pain in 13 (24.1%) cases, and skin rash in 12 (22.1%) cases. Twenty-four (44.4%) patients have taken daily or on-demand non-steroidal anti-inflammatory drugs (NSAIDs) and 31 (57.4%) patients have been treated with daily or on demand oral glucocorticoids. Colchicine was used in 28 (51.8%) patients, while other conventional disease modifying anti-rheumatic drugs (cDMARDs) were employed in 28 (51.8%) patients. Forty (74.1%) patients underwent anti-tumor necrosis factor (TNF) agents and 11 (20.4%) were treated with interleukin (IL)-1 inhibitors. The response to TNF inhibitors on recurrent fever episodes appeared more effective than that observed with anti-IL-1 agents; colchicine and other cDMARD

Published
2023

18. A patient-driven registry on Behçet's disease: the AIDA for patients pilot project

Author

Gaggiano, C, Del Bianco, A, Sota, J, Gentileschi, S, Ruscitti, P, Giacomelli, R, Piga, M, Crisafulli, F, Monti, S, Emmi, G, De Paulis, A, Vitale, A, Tarsia, M, Caggiano, V, Nuzzolese, R, Parretti, V, Fabiani, C, Lopalco, G, Maier, A, Cattalini, M, Rigante, Donato, Govoni, M, Li Gobbi, F, Guiducci, S, Parronchi, P, Marino, A, Ciccia, F, Maggio, Mc, Aragona, E, Bartoloni, E, Iagnocco, A, Viapiana, O, Sebastiani, Gd, Guerriero, S, Insalaco, A, Del Giudice, E, Conti, G, Barone, P, Olivieri, An, Brucato, A, Carubbi, F, Triggianese, P, Mauro, A, Tosi, Gm, Fonollosa, A, Giardini, Ham, Ragab, G, Tharwat, S, Hernández-Rodríguez, J, Sfikakis, Pp, Laskari, K, Karamanakos, A, Espinosa, G, Shahram, F, Direskeneli, H, Hinojosa-Azaola, A, Opris-Belinski, D, Almaghlouth, Ia, Hatemi, G, Eksin, Ma, Önen, F, Więsik-Szewczyk, E, Akkoç, N, Tufan, A, Şahin, A, Erten, Ş, Ozen, S, Batu, Ed, Frediani, B, Balistreri, A, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Gaggiano, C, Del Bianco, A, Sota, J, Gentileschi, S, Ruscitti, P, Giacomelli, R, Piga, M, Crisafulli, F, Monti, S, Emmi, G, De Paulis, A, Vitale, A, Tarsia, M, Caggiano, V, Nuzzolese, R, Parretti, V, Fabiani, C, Lopalco, G, Maier, A, Cattalini, M, Rigante, Donato, Govoni, M, Li Gobbi, F, Guiducci, S, Parronchi, P, Marino, A, Ciccia, F, Maggio, Mc, Aragona, E, Bartoloni, E, Iagnocco, A, Viapiana, O, Sebastiani, Gd, Guerriero, S, Insalaco, A, Del Giudice, E, Conti, G, Barone, P, Olivieri, An, Brucato, A, Carubbi, F, Triggianese, P, Mauro, A, Tosi, Gm, Fonollosa, A, Giardini, Ham, Ragab, G, Tharwat, S, Hernández-Rodríguez, J, Sfikakis, Pp, Laskari, K, Karamanakos, A, Espinosa, G, Shahram, F, Direskeneli, H, Hinojosa-Azaola, A, Opris-Belinski, D, Almaghlouth, Ia, Hatemi, G, Eksin, Ma, Önen, F, Więsik-Szewczyk, E, Akkoç, N, Tufan, A, Şahin, A, Erten, Ş, Ozen, S, Batu, Ed, Frediani, B, Balistreri, A, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Introduction: This paper describes the creation and preliminary results of a patient-driven registry for the collection of patient-reported outcomes (PROs) and patient-reported experiences (PREs) in Behçet’s disease (BD). Methods: The project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behçet), in the context of the AIDA (AutoInflammatory Diseases Alliance) Network programme. Quality of life, fatigue, socioeconomic impact of the disease and therapeutic adherence were selected as core domains to include in the registry. Results: Respondents were reached via SIMBA communication channels in 167 cases (83.5%) and the AIDA Network affiliated clinical centers in 33 cases (16.5%). The median value of the Behçet’s Disease Quality of Life (BDQoL) score was 14 (IQR 11, range 0–30), indicating a medium quality of life, and the median Global Fatigue Index (GFI) was 38.7 (IQR 10.9, range 1–50), expressing a significant level of fatigue. The mean Beliefs about Medicines Questionnaire (BMQ) necessity-concern differential was 0.9 ± 1.1 (range – 1.8–4), showing that the registry participants prioritized necessity belief over concerns to a limited extent. As for the socioeconomic impact of BD, in 104 out of 187 cases (55.6%), patients had to pay from their own pocket for medical exams required to reach the diagnosis. The low family socioeconomic status (p < 0.001), the presence of any major organ involvement (p < 0.031), the presence of gastro-intestinal (p < 0.001), neurological (p = 0.012) and musculoskeletal (p = 0.022) symptoms, recurrent fever (p = 0.002), and headache (p < 0.001) were associated to a higher number of accesses to the healthcare system. Multiple linear regression showed that the BDQoL score could significantly predict the global socioeconomic impact of BD (F = 14.519, OR 1.162 [CI 0.557–1.766], p < 0.001). Discussion: Preliminary results from the AID

Published
2023

19. Musculoskeletal manifestations in children with Behçet's syndrome: data from the AIDA Network Behçet's Syndrome Registry

Author

Gaggiano, C, Maselli, A, Sfikakis, Pp, Laskari, K, Ragab, G, Hegazy, Mt, Laymouna, Ah, Lopalco, G, Almaghlouth, Ia, Asfina, Kn, Alahmed, O, Giardini Mayrink, Ha, Parente de Brito Antonelli, I, Cattalini, M, Piga, M, Sota, J, Gentileschi, S, Maggio, Mc, Opris-Belinski, D, Hatemi, G, Insalaco, A, Olivieri, An, Tufan, A, Karadeniz, H, Kardaş, Rc, La Torre, F, Cardinale, F, Marino, A, Guerriero, S, Ruscitti, P, Tarsia, M, Vitale, A, Caggiano, V, Telesca, S, Iannone, F, Parretti, V, Frassi, M, Aragona, E, Ciccia, F, Wiesik-Szewczyk, E, Ionescu, R, Şahin, A, Akkoç, N, Hinojosa-Azaola, A, Tharwat, S, Hernández-Rodríguez, J, Espinosa, G, Conti, G, Del Giudice, E, Govoni, M, Emmi, G, Fabiani, C, Balistreri, A, Frediani, B, Rigante, Donato, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Gaggiano, C, Maselli, A, Sfikakis, Pp, Laskari, K, Ragab, G, Hegazy, Mt, Laymouna, Ah, Lopalco, G, Almaghlouth, Ia, Asfina, Kn, Alahmed, O, Giardini Mayrink, Ha, Parente de Brito Antonelli, I, Cattalini, M, Piga, M, Sota, J, Gentileschi, S, Maggio, Mc, Opris-Belinski, D, Hatemi, G, Insalaco, A, Olivieri, An, Tufan, A, Karadeniz, H, Kardaş, Rc, La Torre, F, Cardinale, F, Marino, A, Guerriero, S, Ruscitti, P, Tarsia, M, Vitale, A, Caggiano, V, Telesca, S, Iannone, F, Parretti, V, Frassi, M, Aragona, E, Ciccia, F, Wiesik-Szewczyk, E, Ionescu, R, Şahin, A, Akkoç, N, Hinojosa-Azaola, A, Tharwat, S, Hernández-Rodríguez, J, Espinosa, G, Conti, G, Del Giudice, E, Govoni, M, Emmi, G, Fabiani, C, Balistreri, A, Frediani, B, Rigante, Donato, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

This study aims to describe musculoskeletal manifestations (MSM) in children with Behçet’s syndrome (BS), their association with other disease manifestations, response to therapy, and long-term prognosis. Data were retrieved from the AIDA Network Behçet’s Syndrome Registry. Out of a total of 141 patients with juvenile BS, 37 had MSM at disease onset (26.2%). The median age at onset was 10.0 years (IQR 7.7). The median follow-up duration was 21.8 years (IQR 23.3). Recurrent oral (100%) and genital ulcers (67.6%) and pseudofolliculitis (56.8%) were the most common symptoms associated with MSM. At disease onset, 31 subjects had arthritis (83.8%), 33 arthralgia (89.2%), and 14 myalgia (37.8%). Arthritis was monoarticular in 9/31 cases (29%), oligoarticular in 10 (32.3%), polyarticular in 5 (16.1%), axial in 7 (22.6%). Over time, arthritis became chronic-recurrent in 67.7% of cases and 7/31 patients had joint erosions (22.6%). The median Behçet's Syndrome Overall Damage Index was 0 (range 0–4). Colchicine was inefcacious for MSM in 4/14 cases (28.6%), independently from the type of MSM (p=0.46) or the concomitant therapy (p=0.30 for cDMARDs, p=1.00 for glucocorticoids); cDMARDs and bDMARDs were inefcacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefcacy (p=0.014). To conclude, MSM in children with BS are frequently associated with recurrent ulcers and pseudofolliculitis. Arthritis is mostly mono- or oligoarticular, but sacroiliitis is not unusual. Prognosis of this subset of BS is overall favorable, though the presence of myalgia negatively afects response to biologic therapies.

Published
2023

20. Efficacy and Safety of Adalimumab in Pediatric Non-infectious Non-anterior Uveitis: Real-life Experience From the International AIDA Network Uveitis Registry

Author

Vitale, A., Casa, F. D., Guerriero, S., Ragab, G., Mauro, A., Caggiano, V., Cattalini, M., Del Giudice, E., Favale, R., Gaggiano, C., Bellicini, I., Paroli, M. P., Hegazy, M. T., Sota, J., Tufan, A., Balistreri, A., Almaghlouth, I., La Torre, F., Więsik-Szewczyk, E., Tarsia, M., Hinojosa-Azaola, A., Martín-Nares, E., Frediani, B., Tosi, G. M., Fonollosa, A., Hernández-Rodríguez, J., Amin, R. H., Lopalco, G., Rigante, Donato, Cantarini, L., Fabiani, C., Rigante D. (ORCID:0000-0001-7032-7779), Vitale, A., Casa, F. D., Guerriero, S., Ragab, G., Mauro, A., Caggiano, V., Cattalini, M., Del Giudice, E., Favale, R., Gaggiano, C., Bellicini, I., Paroli, M. P., Hegazy, M. T., Sota, J., Tufan, A., Balistreri, A., Almaghlouth, I., La Torre, F., Więsik-Szewczyk, E., Tarsia, M., Hinojosa-Azaola, A., Martín-Nares, E., Frediani, B., Tosi, G. M., Fonollosa, A., Hernández-Rodríguez, J., Amin, R. H., Lopalco, G., Rigante, Donato, Cantarini, L., Fabiani, C., and Rigante D. (ORCID:0000-0001-7032-7779)

Abstract

Introduction: Scientific evidence of the effectiveness of the tumor necrosis factor inhibitor adalimumab (ADA) in pediatric patients with non-infectious non-anterior uveitis is still limited. The aim of this study is to investigate the therapeutic role of ADA in a cohort of pediatric patients with non-anterior uveitis. MethodsThis is an international multicenter study analyzing real-life data referred to pediatric patients treated with ADA for intermediate uveitis/pars planitis, posterior uveitis and panuveitis. Data were drawn from the AutoInflammatory Disease Alliance (AIDA) registry for patients with uveitis. ResultsTwenty-one patients (36 affected eyes) were enrolled, and all patients benefited from ADA administration. In detail, 11 patients (19 affected eyes) did not experience further ocular inflammation after ADA introduction; 10 cases (17 affected eyes) showed a significant clinical improvement consisting of a decrease in severity and/or frequency of ocular relapses. The number of ocular flares dropped from 3.91 to 1.1 events/patient/year after ADA introduction (p = 0.0009); macular edema and retinal vasculitis were respectively observed in 18 eyes and 20 eyes at the start of ADA and in 4 eyes and 2 eyes at the last assessment. The mean daily glucocorticoid dosage significantly decreased from 26.8 +/- 16.8 mg/day at the start of ADA to 6.25 +/- 6.35 mg/day at the last assessment (p = 0.002). Intermediate uveitis/pars planitis (p = 0.01) and posterior uveitis (p = 0.03) were more frequently observed in patients with full response to ADA; panuveitis (p = 0.001) was significantly more frequent among patients continuing to experience uveitic flares. This could be related to a higher use of systemic glucocorticoids (p = 0.002) and conventional immunosuppressants (p = 0.007) at the start of ADA when treating intermediate uveitis/pars planitis. Regarding the safety profile, only one adverse event was reported during ADA treatment, consisting of the development of g

Published
2023

21. 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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22. Development and implementation of the AIDA International Registry for patients with undifferentiated systemic autoinflammatory diseases

Author

Della Casa, F, Vitale, A, Lopalco, G, Ruscitti, P, Ciccia, F, Emmi, G, Cattalini, M, Wiesik-Szewczyk, E, Maggio, Mc, Ogunjimi, B, Sfikakis, Pp, Tufan, A, Al-Mayouf, Sm, Del Giudice, E, Aragona, E, La Torre, F, Sota, J, Colella, S, Di Cola, I, Iacono, D, Mattioli, I, Jahnz-Rózyk, K, Joos, R, Laskari, K, Gaggiano, C, Abbruzzese, A, Cipriani, P, Rozza, G, Alsaleem, A, Yildirim, D, Tarsia, M, Ragab, G, Ricci, F, Cardinale, F, Korzeniowska, M, Frassi, M, Caggiano, V, Saad, Ma, Pereira, Rm, Berlengiero, V, Gentileschi, S, Guerriero, S, Giani, T, Gelardi, V, Iannone, F, Giardini, Ham, Almaghlouth, Ia, Kardas, Rc, Ait-Idir, D, Frediani, B, Balistreri, A, Fabiani, C, Rigante, Donato, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Della Casa, F, Vitale, A, Lopalco, G, Ruscitti, P, Ciccia, F, Emmi, G, Cattalini, M, Wiesik-Szewczyk, E, Maggio, Mc, Ogunjimi, B, Sfikakis, Pp, Tufan, A, Al-Mayouf, Sm, Del Giudice, E, Aragona, E, La Torre, F, Sota, J, Colella, S, Di Cola, I, Iacono, D, Mattioli, I, Jahnz-Rózyk, K, Joos, R, Laskari, K, Gaggiano, C, Abbruzzese, A, Cipriani, P, Rozza, G, Alsaleem, A, Yildirim, D, Tarsia, M, Ragab, G, Ricci, F, Cardinale, F, Korzeniowska, M, Frassi, M, Caggiano, V, Saad, Ma, Pereira, Rm, Berlengiero, V, Gentileschi, S, Guerriero, S, Giani, T, Gelardi, V, Iannone, F, Giardini, Ham, Almaghlouth, Ia, Kardas, Rc, Ait-Idir, D, Frediani, B, Balistreri, A, Fabiani, C, Rigante, Donato, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: This paper points out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients affected with Undifferentiated Systemic AutoInflammatory Diseases (USAIDs). Methods: This is a physician-driven, population- and electronic-based registry employed for real-world data collection about demographics, clinical, laboratory, instrumental and socioeconomic data of USAIDs patients. Data recruitment, based on the Research Electronic Data Capture (REDCap) tool, is thought to gain standardised information for real-life research and has been developed to change over time according to the scientific acquisitions and potentially communicate with other similar instruments; this platform ensures security, data quality and data governance. Results: The focus of the AIDA project is connecting physicians and researchers from all over the world to shed a new light on heterogeneous rare diseases. Since its birth, 99 centres from 20 countries and 4 continents have joined the AIDA project. Forty-eight centres have already obtained the approval from their local Ethics Committees. Currently, the platform counts 265 users (99 Principal Investigators, 162 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry is collecting baseline and follow-up data using 3357 fields organised into 23 instruments, which include demographics, history, symptoms, trigger/risk factors, therapies, and healthcare access for USAIDs patients. Conclusions: The development of the AIDA International Registry for USAIDs patients will facilitate the on-line collection of standardised data, connecting a worldwide group of researchers: the Registry constitutes an international multicentre observational groundwork aimed at increasing the study cohort of patients with USAIDs in order to improve our knowledge of this peculiar cluster of autoinflammatory diseases.

Published
2022

23. Development and implementation of the AIDA International Registry for patients with Behçet's disease

Author

Vitale, A, Della Casa, F, Ragab, G, Almaghlouth, Ia, Lopalco, G, Pereira, Rm, Guerriero, S, Govoni, M, Sfikakis, Pp, Giacomelli, R, Ciccia, F, Monti, S, Ruscitti, P, Piga, M, Lomater, C, Tufan, A, Opris-Belinski, D, Emmi, G, Hernández-Rodríguez, J, Şahin, A, Sebastiani, Gd, Bartoloni, E, Akkoç, N, Gündüz, Ö, Cattalini, M, Conti, Giorgio, Hatemi, G, Maier, A, Parronchi, P, Del Giudice, E, Erten, S, Insalaco, A, Li Gobbi, F, Maggio, Mc, Shahram, F, Caggiano, V, Hegazy, Mt, Asfina, Kn, Morrone, M, Prado, Ll, Dammacco, R, Ruffilli, F, Arida, A, Navarini, L, Pantano, I, Cavagna, L, Conforti, A, Cauli, A, Marucco, Em, Kucuk, H, Ionescu, R, Mattioli, I, Espinosa, G, Araújo, O, Karkaş, B, Canofari, C, Sota, J, Laymouna, Ah, Bedaiwi, Aa, Colella, S, Giardini, Ham, Albano, V, Lo Monaco, A, Fragoulis, Ge, Kardas, Rc, Berlengiero, V, Hussein, Ma, Ricci, F, La Torre, F, Rigante, Donato, Więsik-Szewczyk, E, Frassi, M, Gentileschi, S, Tosi, Gm, Dagostin, Ma, Mahmoud, Aaa, Tarsia, M, Alessio, G, Cimaz, R, Giani, T, Gaggiano, C, Iannone, F, Cipriani, P, Mourabi, M, Spedicato, V, Barneschi, S, Aragona, E, Balistreri, A, Frediani, B, Fabiani, C, Cantarini, L &amp, Autoinflammatory Diseases Alliance (AIDA), Network, Conti G (ORCID:0000-0002-8566-9365), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Della Casa, F, Ragab, G, Almaghlouth, Ia, Lopalco, G, Pereira, Rm, Guerriero, S, Govoni, M, Sfikakis, Pp, Giacomelli, R, Ciccia, F, Monti, S, Ruscitti, P, Piga, M, Lomater, C, Tufan, A, Opris-Belinski, D, Emmi, G, Hernández-Rodríguez, J, Şahin, A, Sebastiani, Gd, Bartoloni, E, Akkoç, N, Gündüz, Ö, Cattalini, M, Conti, Giorgio, Hatemi, G, Maier, A, Parronchi, P, Del Giudice, E, Erten, S, Insalaco, A, Li Gobbi, F, Maggio, Mc, Shahram, F, Caggiano, V, Hegazy, Mt, Asfina, Kn, Morrone, M, Prado, Ll, Dammacco, R, Ruffilli, F, Arida, A, Navarini, L, Pantano, I, Cavagna, L, Conforti, A, Cauli, A, Marucco, Em, Kucuk, H, Ionescu, R, Mattioli, I, Espinosa, G, Araújo, O, Karkaş, B, Canofari, C, Sota, J, Laymouna, Ah, Bedaiwi, Aa, Colella, S, Giardini, Ham, Albano, V, Lo Monaco, A, Fragoulis, Ge, Kardas, Rc, Berlengiero, V, Hussein, Ma, Ricci, F, La Torre, F, Rigante, Donato, Więsik-Szewczyk, E, Frassi, M, Gentileschi, S, Tosi, Gm, Dagostin, Ma, Mahmoud, Aaa, Tarsia, M, Alessio, G, Cimaz, R, Giani, T, Gaggiano, C, Iannone, F, Cipriani, P, Mourabi, M, Spedicato, V, Barneschi, S, Aragona, E, Balistreri, A, Frediani, B, Fabiani, C, Cantarini, L &amp, Autoinflammatory Diseases Alliance (AIDA), Network, Conti G (ORCID:0000-0002-8566-9365), and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: Purpose of the present paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients with Behçet’s disease (BD). Methods: The Registry is a clinical physician-driven population- and electronic-based instrument implemented for the retrospective and prospective collection of real-life data about demographics, clinical, therapeutic, laboratory, instrumental and socioeconomic information from BD patients; the Registry is based on the Research Electronic Data Capture (REDCap) tool, which is thought to collect standardised information for clinical real-life research, and has been realised to change over time according to future scientific acquisitions and potentially communicate with other existing and future Registries dedicated to BD. Results: Starting from January 31st to November 23rd, 2021, 99 centres from 20 countries in 4 continents have been involved. Forty-eight of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 265 users (99 Principal Investigators, 162 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 5474 fields organised into 15 instruments, including patient’s demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. Conclusions: The development of the AIDA International Registry for BD patients will facilitate the collection of standardised data leading to real-world evidence, enabling international multicentre collaborative research through data sharing, international consultation, dissemination of knowledge, inclusion of patients and families, and ultimately optimisation of scientific efforts and implementation of standardised care.

Published
2022

24. Development and implementation of the AIDA International Registry for patients with VEXAS syndrome

Author

Vitale, A, Caggiano, V, Della Casa, F, Hernández-Rodríguez, J, Frassi, M, Monti, S, Tufan, A, Telesca, S, Conticini, E, Ragab, G, Lopalco, G, Almaghlouth, I, Pereira, Rmr, Yildirim, D, Cattalini, M, Marino, A, Giani, T, La Torre, F, Ruscitti, P, Aragona, E, Wiesik-Szewczyk, E, Del Giudice, E, Sfikakis, Pp, Govoni, M, Emmi, G, Maggio, Mc, Giacomelli, R, Ciccia, F, Conti, Giorgio, Ait-Idir, D, Lomater, C, Sabato, V, Piga, M, Sahin, A, Opris-Belinski, D, Ionescu, R, Bartoloni, E, Franceschini, F, Parronchi, P, de Paulis, A, Espinosa, G, Maier, A, Sebastiani, Gd, Insalaco, A, Shahram, F, Sfriso, P, Minoia, F, Alessio, M, Makowska, J, Hatemi, G, Akkoç, N, Li Gobbi, F, Gidaro, A, Olivieri, An, Al-Mayouf, Sm, Erten, S, Gentileschi, S, Vasi, I, Tarsia, M, Mahmoud, Aaa, Frediani, B, Fares Alzahrani, M, Laymouna, Ah, Ricci, F, Cardinale, F, Jahnz-Rózyk, K, Tosi, Gm, Crisafulli, F, Balistreri, A, Dagostin, Ma, Ghanema, M, Gaggiano, C, Sota, J, Di Cola, I, Fabiani, C, Giardini, Ham, Renieri, A, Fabbiani, A, Carrer, A, Bocchia, M, Caroni, F, Rigante, Donato, Cantarini, L, Conti G (ORCID:0000-0002-8566-9365), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Caggiano, V, Della Casa, F, Hernández-Rodríguez, J, Frassi, M, Monti, S, Tufan, A, Telesca, S, Conticini, E, Ragab, G, Lopalco, G, Almaghlouth, I, Pereira, Rmr, Yildirim, D, Cattalini, M, Marino, A, Giani, T, La Torre, F, Ruscitti, P, Aragona, E, Wiesik-Szewczyk, E, Del Giudice, E, Sfikakis, Pp, Govoni, M, Emmi, G, Maggio, Mc, Giacomelli, R, Ciccia, F, Conti, Giorgio, Ait-Idir, D, Lomater, C, Sabato, V, Piga, M, Sahin, A, Opris-Belinski, D, Ionescu, R, Bartoloni, E, Franceschini, F, Parronchi, P, de Paulis, A, Espinosa, G, Maier, A, Sebastiani, Gd, Insalaco, A, Shahram, F, Sfriso, P, Minoia, F, Alessio, M, Makowska, J, Hatemi, G, Akkoç, N, Li Gobbi, F, Gidaro, A, Olivieri, An, Al-Mayouf, Sm, Erten, S, Gentileschi, S, Vasi, I, Tarsia, M, Mahmoud, Aaa, Frediani, B, Fares Alzahrani, M, Laymouna, Ah, Ricci, F, Cardinale, F, Jahnz-Rózyk, K, Tosi, Gm, Crisafulli, F, Balistreri, A, Dagostin, Ma, Ghanema, M, Gaggiano, C, Sota, J, Di Cola, I, Fabiani, C, Giardini, Ham, Renieri, A, Fabbiani, A, Carrer, A, Bocchia, M, Caroni, F, Rigante, Donato, Cantarini, L, Conti G (ORCID:0000-0002-8566-9365), and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: The aim of this paper is to present the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome, describing its design, construction, and modalities of dissemination. Methods: The present registry is a clinical, physician-driven, population- and electronic-based instrument designed for the retrospective and prospective collection of real-life data. Data gathering is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain real-world evidence for daily patients’ management; the registry may potentially communicate with other on-line tools dedicated to VEXAS syndrome, enhancing international collaboration and data sharing for research purposes. The registry is handy enough to be easily modified to meet future needs regarding VEXAS syndrome. Results: To date (April 2022), 105 Centers from 23 Countries in 4 continents have been involved; 287 users (106 Principal Investigators, 177 Site Investigators, 2 Lead Investigators, and 2 data managers) may already enter the registry for data collection and sharing. The registry includes 4950 fields organised into 18 instruments designed to fully describe patient’s details about demographics, clinical manifestations, symptoms, histologic details about skin and bone marrow biopsies and aspirate, laboratory features, complications, comorbidities, therapies, and healthcare access. Conclusions: This international Registry for patients with VEXAS syndrome will allow the achievement of a comprehensive knowledge about this new disease in a relatively short time with the final goal to obtain real-world evidence data for daily clinical practice. This project can be found on https://clinicaltrials.gov NCT 05200715

Published
2022

25. Development and implementation of the AIDA International Registry for patients with Schnitzler’s syndrome

Author

Sota, J, Vitale, A, Więsik-Szewczyk, E, Frassi, M, Lopalco, G, Emmi, G, Govoni, M, de Paulis, A, Marino, A, Gidaro, A, Monti, S, Opris-Belinski, D, Pereira, Rmr, Jahnz-Rózyk, K, Gaggiano, C, Crisafulli, F, Iannone, F, Mattioli, I, Ruffilli, F, Mormile, I, Rybak, K, Caggiano, V, Airò, P, Tufan, A, Gentileschi, S, Ragab, G, Almaghlouth, Ia, Aboul-Fotouh Khalil, A, Cattalini, M, La Torre, F, Tarsia, M, Giardini, Ham, Ali Saad, M, Bocchia, M, Caroni, F, Giani, T, Cinotti, E, Ruscitti, P, Rubegni, P, Dagostin, Ma, Frediani, B, Guler, Aa, Della Casa, F, Maggio, Mc, Recke, A, von Bubnoff, D, Krause, K, Balistreri, A, Fabiani, C, Rigante, Donato, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Sota, J, Vitale, A, Więsik-Szewczyk, E, Frassi, M, Lopalco, G, Emmi, G, Govoni, M, de Paulis, A, Marino, A, Gidaro, A, Monti, S, Opris-Belinski, D, Pereira, Rmr, Jahnz-Rózyk, K, Gaggiano, C, Crisafulli, F, Iannone, F, Mattioli, I, Ruffilli, F, Mormile, I, Rybak, K, Caggiano, V, Airò, P, Tufan, A, Gentileschi, S, Ragab, G, Almaghlouth, Ia, Aboul-Fotouh Khalil, A, Cattalini, M, La Torre, F, Tarsia, M, Giardini, Ham, Ali Saad, M, Bocchia, M, Caroni, F, Giani, T, Cinotti, E, Ruscitti, P, Rubegni, P, Dagostin, Ma, Frediani, B, Guler, Aa, Della Casa, F, Maggio, Mc, Recke, A, von Bubnoff, D, Krause, K, Balistreri, A, Fabiani, C, Rigante, Donato, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: The present paper describes the design, development, and implementation of the AutoInflammatory Disease Alliance (AIDA) International Registry specifically dedicated to patients with Schnitzler syndrome. Methods: This is a clinical physician-driven, population- and electronic-based registry implemented for the retrospective and prospective collection of real-life data from patients with Schnitzler’s syndrome; the registry is based on the Research Electronic Data Capture (REDCap) tool, which is thought to collect standardised information for clinical research, and has been realised to change over time according to future scientific acquisitions and potentially communicate with other existing or future similar registries. Results: Since its launch, 112 centres from 23 countries in 4 continents have been involved. Fifty-four have already obtained the approval from their local Ethics Committees. The platform counts 303 users (113 Principal Investigators, 186 Site Investigators, 2 Lead Investigators, and 2 data managers) at current (April 12th 2022). The registry collects baseline and follow-up data using 3922 fields organised into 25 instruments, including patient’s demographics, history, clinical manifestations and symptoms, trigger/risk factors, laboratory, instrumental exams, therapies, socioeconomic information, and healthcare access. Conclusions: This International Registry for patients with Schnitzler syndrome facilitates standardised data collection, enabling international collaborative projects through data sharing and dissemination of knowledge; in turn, it will shed light into many blind spots characterizing this complex autoinflammatory disorder

Published
2022

26. Efficacy and safety of tocilizumab in adult-onset Still's disease: Real-life experience from the international AIDA registry

Author

Sota, J, Vitale, A, Lopalco, G, Pereira, Rmr, Giordano, Hf, Antonelli, Ipb, Makowska, J, Brzezińska, O, Lewandowska-Polak, A, Ruscitti, P, Cipriani, P, Cola, Id, Govoni, M, Ruffili, F, Sfikakis, Pp, Laskari, K, Ragab, G, Hussein, Ma, Gentileschi, S, Gaggiano, C, La Torre, F, Maier, A, Emmi, G, Marino, A, Ciccia, F, Sfriso, P, Maggio, Mc, Bartoloni, E, Lomater, C, Hegazy, Mt, Tektonidou, M, Dagostin, Ma, Opinc, A, Sebastiani, Gd, Giacomelli, R, Giudice, Ed, Olivieri, An, Tufan, A, Kardas, Rk, Nuzzolese, R, Cardinale, F, Więsik-Szewczyk, E, Veronica, P, Tarsia, M, Iannone, F, Della Casa, F, Fabiani, C, Frediani, B, Balistreri, A, Rigante, Donato, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Sota, J, Vitale, A, Lopalco, G, Pereira, Rmr, Giordano, Hf, Antonelli, Ipb, Makowska, J, Brzezińska, O, Lewandowska-Polak, A, Ruscitti, P, Cipriani, P, Cola, Id, Govoni, M, Ruffili, F, Sfikakis, Pp, Laskari, K, Ragab, G, Hussein, Ma, Gentileschi, S, Gaggiano, C, La Torre, F, Maier, A, Emmi, G, Marino, A, Ciccia, F, Sfriso, P, Maggio, Mc, Bartoloni, E, Lomater, C, Hegazy, Mt, Tektonidou, M, Dagostin, Ma, Opinc, A, Sebastiani, Gd, Giacomelli, R, Giudice, Ed, Olivieri, An, Tufan, A, Kardas, Rk, Nuzzolese, R, Cardinale, F, Więsik-Szewczyk, E, Veronica, P, Tarsia, M, Iannone, F, Della Casa, F, Fabiani, C, Frediani, B, Balistreri, A, Rigante, Donato, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Background/objectives: Long-term efficacy and safety of tocilizumab (TCZ) in adult-onset Still’s disease (AOSD) mostly derive from small case series. Herein we report a registry-based study investigating TCZ efficacy and safety in a cohort of patients with AOSD evaluated by clinical and serum inflammatory markers as well as drug retention rate analysis. Methods: This is an international multicentre study analyzing data from patients with AOSD regularly enrolled in the AIDA registry. TCZ efficacy was evaluated between baseline and last follow-up assessment in terms of changes in the Pouchot score and laboratory findings. Drug-retention rate was estimated by the Kaplan-Meier method, while Cox-regression analysis was employed to detect potential predictive factors of treatment withdrawal. Results: Data from 31 patients (15 men, 16 women) refractory to the conventional therapies and treated with TCZ were extracted from the AIDA registry. Mean ± SD time of treatment duration with TCZ was 24.32 ± 20.57 months. Pouchot score significantly decreased from baseline 2.00 (4.00) to the last follow-up assessment 00.00 (00.00), p=0.003). Similarly, laboratory parameters significantly decreased from baseline to the last follow-up visit. With regard to drug survival, cumulative TCZ retention rate at 12-, 24-, and 36-month follow-up visit were 83.1%, 71.7% and 63.7%, respectively, without significant differences between biologic naïve patients and those previously treated with other biologics (p=0.329). Likewise, no significant differences were observed between chronic articular course of AOSD and other types of disease course (p=0.938) or between patients co-administered with conventional immunosuppressants and patients receiving TCZ as monotherapy (p=0.778). Cox-regression analysis identified no variable associated with a higher hazard of treatment withdrawal. Treatment was discontinued in 9 patients due to long-term remission (n=4), adverse events (n=2), loss of efficacy (n=1)

Published
2022

27. Development and implementation of the AIDA International Registry for patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis syndrome

Author

Della Casa, F, Vitale, A, Cattalini, M, La Torre, F, Capozio, G, Del Giudice, E, Maggio, Mc, Conti, Giorgio, Alessio, M, Ogunjimi, B, Ragab, G, Emmi, G, Aragona, E, Giani, T, Lopalco, G, Parronchi, P, Shahram, F, Verrecchia, Elena, Ricci, F, Cardinale, F, Di Noi, Silvia, Nuzzolese, R, Lubrano, R, Patroniti, S, Naddei, R, Sabato, V, Hussein, Ma, Dotta, L, Mastrorilli, V, Gentileschi, S, Tufan, A, Caggiano, V, Hegazy, Mt, Sota, J, Almaghlouth, Ia, Ibrahim, A, Wiȩsik-Szewczyk, E, Ozkiziltas, B, Grosso, S, Frassi, M, Tarsia, M, Pereira, Rmr, Taymour, M, Gaggiano, C, Colella, S, Fabiani, C, Morrone, M, Ruscitti, P, Frediani, B, Spedicato, V, Giardini, Ham, Balistreri, A, Rigante, Donato, Cantarini, L, Conti G (ORCID:0000-0002-8566-9365), Verrecchia E, Di Noi S, Rigante D (ORCID:0000-0001-7032-7779), Della Casa, F, Vitale, A, Cattalini, M, La Torre, F, Capozio, G, Del Giudice, E, Maggio, Mc, Conti, Giorgio, Alessio, M, Ogunjimi, B, Ragab, G, Emmi, G, Aragona, E, Giani, T, Lopalco, G, Parronchi, P, Shahram, F, Verrecchia, Elena, Ricci, F, Cardinale, F, Di Noi, Silvia, Nuzzolese, R, Lubrano, R, Patroniti, S, Naddei, R, Sabato, V, Hussein, Ma, Dotta, L, Mastrorilli, V, Gentileschi, S, Tufan, A, Caggiano, V, Hegazy, Mt, Sota, J, Almaghlouth, Ia, Ibrahim, A, Wiȩsik-Szewczyk, E, Ozkiziltas, B, Grosso, S, Frassi, M, Tarsia, M, Pereira, Rmr, Taymour, M, Gaggiano, C, Colella, S, Fabiani, C, Morrone, M, Ruscitti, P, Frediani, B, Spedicato, V, Giardini, Ham, Balistreri, A, Rigante, Donato, Cantarini, L, Conti G (ORCID:0000-0002-8566-9365), Verrecchia E, Di Noi S, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: Aim of this paper is to illustrate the methodology, design, and development of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to patients with the Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Methods: This is a physician-driven, population- and electronic-based registry proposed to gather real-world demographics, clinical, laboratory, instrumental and socioeconomic data from patients suffering from PFAPA syndrome. Data recruitment is realized through the on-line Research Electronic Data Capture (REDCap) tool. This registry is thought to collect standardized information for clinical research leading to solid real-life evidence. The international scope and the flexibility of the registry will facilitate the realization of cutting-edge study projects through the constant updating of variables - as required by future scientific acquisitions - and the possible merging and transfer of data between current and future registries devoted to this disease. Results: One hundred and twelve centers have already been involved from 23 countries and 4 continents starting from August 24th, 2021, to March 21st, 2022. Fifty-five out of 112 have already obtained the formal approval from their local Ethics Committees. At current, the platform counts 287 users (108 principal investigators, 179 site investigators, 2 lead investigators, and 2 data managers). The registry collects retrospective and prospective data using 3845 fields organized into 24 instruments, including PFAPA patient’s demographics, medical histories, symptoms, triggers/risk factors, therapies, and impact on the healthcare systems. Conclusions: The development of the AIDA International Registry for patients with PFAPA syndrome will enable the on-line collection of standardized data prompting real-life studies through the connection of worldwide groups of physicians and researchers. This project can be found on https://clinicaltrials.gov

Published
2022

28. Development and implementation of the AIDA International Registry for patients with Still’s disease

Author

Vitale, A, Della Casa, F, Lopalco, G, Pereira, Rm, Ruscitti, P, Giacomelli, R, Ragab, G, La Torre, F, Bartoloni, E, Del Giudice, E, Lomater, C, Emmi, G, Govoni, M, Maggio, Mc, Maier, A, Makowska, J, Ogunjimi, B, Sfikakis, Pp, Sfriso, P, Gaggiano, C, Iannone, F, Dagostin, Ma, Di Cola, I, Navarini, L, Ahmed Mahmoud, Aa, Cardinale, F, Riccucci, I, Paroli, Mp, Marucco, Em, Mattioli, I, Sota, J, Abbruzzese, A, Antonelli, Ipb, Cipriani, P, Tufan, A, Fabiani, C, Ramadan, Mm, Cattalini, M, Kardas, Rc, Sebastiani, Gd, Giardini, Ham, Hernández-Rodríguez, J, Mastrorilli, V, Więsik-Szewczyk, E, Frassi, M, Caggiano, V, Telesca, S, Giordano, Hf, Guadalupi, E, Balistreri, A, Rigante, Donato, Cantarini, L., Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Della Casa, F, Lopalco, G, Pereira, Rm, Ruscitti, P, Giacomelli, R, Ragab, G, La Torre, F, Bartoloni, E, Del Giudice, E, Lomater, C, Emmi, G, Govoni, M, Maggio, Mc, Maier, A, Makowska, J, Ogunjimi, B, Sfikakis, Pp, Sfriso, P, Gaggiano, C, Iannone, F, Dagostin, Ma, Di Cola, I, Navarini, L, Ahmed Mahmoud, Aa, Cardinale, F, Riccucci, I, Paroli, Mp, Marucco, Em, Mattioli, I, Sota, J, Abbruzzese, A, Antonelli, Ipb, Cipriani, P, Tufan, A, Fabiani, C, Ramadan, Mm, Cattalini, M, Kardas, Rc, Sebastiani, Gd, Giardini, Ham, Hernández-Rodríguez, J, Mastrorilli, V, Więsik-Szewczyk, E, Frassi, M, Caggiano, V, Telesca, S, Giordano, Hf, Guadalupi, E, Balistreri, A, Rigante, Donato, Cantarini, L., and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: Aim of this paper is to present the design, construction, and modalities of dissemination of the AutoInflammatory Disease Alliance (AIDA) International Registry for patients with systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD), which are the pediatric and adult forms of the same autoinflammatory disorder. Methods: This Registry is a clinical, physician-driven, population- and electronic-based instrument implemented for the retrospective and prospective collection of real-world data. The collection of data is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain evidence drawn from routine patients’ management. The collection of standardized data is thought to bring knowledge about real-life clinical research and potentially communicate with other existing and future Registries dedicated to Still’s disease. Moreover, it has been conceived to be flexible enough to easily change according to future scientific acquisitions. Results: Starting from June 30th to February 7th, 2022, 110 Centers from 23 Countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 4449 fields organized into 14 instruments, including patient’s demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. Conclusions: This international Registry for patients with Still’s disease will allow a robust clinical research through collection of standardized data, international consultation, dissemination of knowledge, and implementation of observational studies based on wide cohorts of patients followed-up for very long periods. Solid evidence drawn from “real-life” data represents the ultimate goal of t

Published
2022

29. Development and implementation of the AIDA International Registry for patients with non-infectious uveitis

Author

Della Casa, F, Vitale, A, Guerriero, S, Sota, J, Cimaz, R, Ragab, G, Ruscitti, P, Pereira, Rmr, Minoia, F, Del Giudice, E, Emmi, G, Lomater, C, Monti, S, Canofari, C, Gaggiano, C, Alessio, G, Miserocchi, E, Conforti, A, Dagostin, Ma, Mapelli, C, Paroli, Mp, Parretti, V, Albano, V, Favale, R, Marelli, L, Hegazy, Mt, Cipriani, P, Antonelli, Ipb, Caggiano, V, Aragona, E, Laymouna, Ah, Tosi, Gm, Tarsia, M, Cattalini, M, La Torre, F, Lopalco, G, Więsik-Szewczyk, E, Frassi, M, Gentileschi, S, Giordano, Hf, Frediani, B, Shinjo, Sk, Rigante, Donato, Sfikakis, Pp, Balistreri, A, Hussein, Ma, Amin, Rh, Cantarini, L, Fabiani, C, Rigante D (ORCID:0000-0001-7032-7779), Della Casa, F, Vitale, A, Guerriero, S, Sota, J, Cimaz, R, Ragab, G, Ruscitti, P, Pereira, Rmr, Minoia, F, Del Giudice, E, Emmi, G, Lomater, C, Monti, S, Canofari, C, Gaggiano, C, Alessio, G, Miserocchi, E, Conforti, A, Dagostin, Ma, Mapelli, C, Paroli, Mp, Parretti, V, Albano, V, Favale, R, Marelli, L, Hegazy, Mt, Cipriani, P, Antonelli, Ipb, Caggiano, V, Aragona, E, Laymouna, Ah, Tosi, Gm, Tarsia, M, Cattalini, M, La Torre, F, Lopalco, G, Więsik-Szewczyk, E, Frassi, M, Gentileschi, S, Giordano, Hf, Frediani, B, Shinjo, Sk, Rigante, Donato, Sfikakis, Pp, Balistreri, A, Hussein, Ma, Amin, Rh, Cantarini, L, Fabiani, C, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Introduction: The aim of this paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance(AIDA) International Registry for paediatric and adult patients with non-infectious uveitis(NIU). Methods: This is a physician-driven, population- and electronic-based registry implemented for both retrospective and prospective collection of real-world demographics, clinical, laboratory, instrumental and socioeconomic data of patients with uveitis and other non-infectious inflammatory ocular diseases recruited through the AIDA Network. Data recruitment, based on the Research Electronic Data Capture (REDCap) tool, is thought to collect standardised information for real-life research and has been developed to change over time according to future scientific acquisitions and potentially communicate with other similar instruments. Security, data quality and data governance are cornerstones of this platform. Results: Ninety-five centres have been involved from 19 countries and four continents from 24 March to 16 November 2021. Forty-eight out of 95 have already obtained the approval from their local ethics committees. At present, the platform counts 259 users (95 principal investigators, 160 site investigators, 2 lead investigators, and 2 data managers). The AIDA Registry collects baseline and follow-up data using 3943 fields organised into 13 instruments, including patient’s demographics, history, symptoms,trigger/risk factors, therapies and health care utilization for patients with NIU. Conclusions: The development of the AIDA Registry for patients with NIU will facilitate the collection of standardised data leading to real-world evidence and enabling international multicentre collaborative research through inclusion of patients and their families worldwide.

Published
2022

30. Development and implementation of the AIDA International Registry for patients with non-infectious scleritis

Author

Della Casa, F, Vitale, A, Pereira, Rm, Guerriero, S, Ragab, G, Lopalco, G, Cattalini, M, Mattioli, I, Parronchi, P, Paroli, Mp, Del Giudice, E, Gaggiano, C, Dagostin, Ma, Albano, V, Soliman, Mm, Colella, S, Nascimbeni, G, Sota, J, Antonelli, Ipb, Alessio, G, Caggiano, V, Tufan, A, Amin, Rh, Tarsia, M, Ghanema, M, Iannone, F, Ricci, F, La Torre, F, Więsik-Szewczyk, E, Conticini, E, Gentileschi, S, Dammacco, R, Cimaz, R, Frediani, B, Abbruzzese, A, Ruscitti, P, Tosi, Gm, Giordano, Hf, Conforti, A, Balistreri, A, Rigante, Donato, Cantarini, L, Fabiani, C, Rigante D (ORCID:0000-0001-7032-7779), Della Casa, F, Vitale, A, Pereira, Rm, Guerriero, S, Ragab, G, Lopalco, G, Cattalini, M, Mattioli, I, Parronchi, P, Paroli, Mp, Del Giudice, E, Gaggiano, C, Dagostin, Ma, Albano, V, Soliman, Mm, Colella, S, Nascimbeni, G, Sota, J, Antonelli, Ipb, Alessio, G, Caggiano, V, Tufan, A, Amin, Rh, Tarsia, M, Ghanema, M, Iannone, F, Ricci, F, La Torre, F, Więsik-Szewczyk, E, Conticini, E, Gentileschi, S, Dammacco, R, Cimaz, R, Frediani, B, Abbruzzese, A, Ruscitti, P, Tosi, Gm, Giordano, Hf, Conforti, A, Balistreri, A, Rigante, Donato, Cantarini, L, Fabiani, C, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Introduction: This article points out the design, methods, development and deployment of the international registry promoted by the AutoInflammatory Disease Alliance (AIDA) Network with the aim to define and assess paediatric and adult patients with immune-mediated scleritis. Methods: This registry collects both retrospec-tive and prospective real-world data from patients with non-infectious scleritis through the Research Electronic Data Capture (REDCap) tool and aims to promote knowledge and real-life evidence from patients enrolled worldwide; the registry also allows the collection of standardised data, ensuring the highest levels of security and anonymity of patients’ data and flexibility to change according to scientific acquisitions over time. The communication with other similar registries has been also ensured in order to pursue the sustainability of the project with respect to the adaptation of collected data to the most diverse research projects. Results: Since the launch of the registry, 99 centres have been involved from 20 countries and four continents. Forty-eight of the centres have already obtained a formal approval from their local ethics committees. At present, the platform counts 259 users (95 principal investigators, 160 site investigators, 2 lead investigators, and 2 data managers); the platform collects baseline and follow-up data using 3683 fields organised into 13 instruments, including patient’s demographics, history, symptoms, trigger or risk factors, therapies and health care utilization. Conclusions: The development of the AIDA International Registry for patients with non-infectious scleritis will allow solid research on this rare condition. Real-world evidence result-ing from standardised real-life data will lead to the optimisation of routine clinical and therapeutic management, which are currently limited by the rarity of this ocular inflammatory condition.

Published
2022

31. 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

32. AB0636 Relationship between organ damage and impairment of health-related quality of life in patients with Behçet’s Syndrome: results from a longitudinal extension of the BODI Project.

Author

Floris, A., primary, Laconi, R., additional, Espinosa, G., additional, Lopalco, G., additional, Serpa Pinto, L., additional, Kougkas, N., additional, Sota, J., additional, Lo Monaco, A., additional, Govoni, M., additional, Cantarini, L., additional, Bertsias, G., additional, Correia, J., additional, Iannone, F., additional, Cervera, R., additional, Vasconcelos, C., additional, Mathieu, A., additional, Cauli, A., additional, and Piga, M., additional

Published
2022
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33. 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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34. AB0630 Assessment of organ damage accrual in Behçet's Syndrome over 2-year follow-up: results from the BODI Project longitudinal extension.

Author

Floris, A., primary, Laconi, R., additional, Espinosa, G., additional, Lopalco, G., additional, Serpa Pinto, L., additional, Kougkas, N., additional, Sota, J., additional, Lo Monaco, A., additional, Govoni, M., additional, Cantarini, L., additional, Bertsias, G., additional, Correia, J., additional, Iannone, F., additional, Cervera, R., additional, Vasconcelos, C., additional, Mathieu, A., additional, Cauli, A., additional, and Piga, M., additional

Published
2022
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38. AB0631 Impact of Behçet’s Syndrome on work activity and productivity: results from a sub-analysis of the BODI Project cohort.

Author

Laconi, R., primary, Floris, A., additional, Espinosa, G., additional, Lopalco, G., additional, Serpa Pinto, L., additional, Kougkas, N., additional, Sota, J., additional, Lo Monaco, A., additional, Govoni, M., additional, Cantarini, L., additional, Bertsias, G., additional, Correia, J., additional, Iannone, F., additional, Cervera, R., additional, Vasconcelos, C., additional, Mathieu, A., additional, Cauli, A., additional, and Piga, M., additional

Published
2022
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39. POS1218 RELAPSES OF IDIOPATHIC INFLAMMATORY MYOPATHIES AFTER VACCINATION AGAINST COVID19: A REAL-LIFE ITALIAN STUDY

Author

Conticini, E., primary, D’alessandro, M., additional, Grazzini, S., additional, Fornaro, M., additional, Sabella, D., additional, Lopalco, G., additional, Iannone, F., additional, Gattamelata, A., additional, Colafrancesco, S., additional, Giardina, F., additional, Priori, R., additional, Rizzo, C., additional, Guggino, G., additional, Cameli, P., additional, Bennett, D., additional, Bargagli, E., additional, Cantarini, L., additional, and Frediani, B., additional

Published
2022
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41. Development and Implementation of the AIDA International Registry for Patients With Still's Disease

Author

Vitale, A. Della Casa, F. Lopalco, G. Pereira, R.M. Ruscitti, P. Giacomelli, R. Ragab, G. La Torre, F. Bartoloni, E. Del Giudice, E. Lomater, C. Emmi, G. Govoni, M. Maggio, M.C. Maier, A. Makowska, J. Ogunjimi, B. Sfikakis, P.P. Sfriso, P. Gaggiano, C. Iannone, F. Dagostin, M.A. Di Cola, I. Navarini, L. Ahmed Mahmoud, A.A. Cardinale, F. Riccucci, I. Paroli, M.P. Marucco, E.M. Mattioli, I. Sota, J. Abbruzzese, A. Antonelli, I.P.B. Cipriani, P. Tufan, A. Fabiani, C. Ramadan, M.M. Cattalini, M. Kardas, R.C. Sebastiani, G.D. Giardini, H.A.M. Hernández-Rodríguez, J. Mastrorilli, V. Więsik-Szewczyk, E. Frassi, M. Caggiano, V. Telesca, S. Giordano, H.F. Guadalupi, E. Giani, T. Renieri, A. Colella, S. Cataldi, G. Gentile, M. Fabbiani, A. Al-Maghlouth, I.A. Frediani, B. Balistreri, A. Rigante, D. Cantarini, L. The Autoinflammatory Diseases Alliance (AIDA) Network

Abstract

Objective: Aim of this paper is to present the design, construction, and modalities of dissemination of the AutoInflammatory Disease Alliance (AIDA) International Registry for patients with systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), which are the pediatric and adult forms of the same autoinflammatory disorder. Methods: This Registry is a clinical, physician-driven, population- and electronic-based instrument implemented for the retrospective and prospective collection of real-world data. The collection of data is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain evidence drawn from routine patients' management. The collection of standardized data is thought to bring knowledge about real-life clinical research and potentially communicate with other existing and future Registries dedicated to Still's disease. Moreover, it has been conceived to be flexible enough to easily change according to future scientific acquisitions. Results: Starting from June 30th to February 7th, 2022, 110 Centers from 23 Countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 4449 fields organized into 14 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. Conclusions: This international Registry for patients with Still's disease will allow a robust clinical research through collection of standardized data, international consultation, dissemination of knowledge, and implementation of observational studies based on wide cohorts of patients followed-up for very long periods. Solid evidence drawn from “real-life” data represents the ultimate goal of this Registry, which has been implemented to significantly improve the overall management of patients with Still's disease. NCT 05200715 available at https://clinicaltrials.gov/. Copyright © 2022 Vitale, Della Casa, Lopalco, Pereira, Ruscitti, Giacomelli, Ragab, La Torre, Bartoloni, Del Giudice, Lomater, Emmi, Govoni, Maggio, Maier, Makowska, Ogunjimi, Sfikakis, Sfriso, Gaggiano, Iannone, Dagostin, Di Cola, Navarini, Ahmed Mahmoud, Cardinale, Riccucci, Paroli, Marucco, Mattioli, Sota, Abbruzzese, Antonelli, Cipriani, Tufan, Fabiani, Ramadan, Cattalini, Kardas, Sebastiani, Giardini, Hernández-Rodríguez, Mastrorilli, Więsik-Szewczyk, Frassi, Caggiano, Telesca, Giordano, Guadalupi, Giani, Renieri, Colella, Cataldi, Gentile, Fabbiani, Al-Maghlouth, Frediani, Balistreri, Rigante and Cantarini.

Published
2022

42. 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

43. Erratum: Management of adult-onset Still's disease with interleukin-1 inhibitors: Evidence- And consensus-based statements by a panel of Italian experts (Arthritis Res Ther (2019) 21:275 DOI: 10.1186/s13075-019-2021-9)

Author

Colafrancesco, S., Manara, M., Bortoluzzi, A., Serban, T., Bianchi, G., Cantarini, L., Ciccia, F., Dagna, L., Govoni, M., Montecucco, C., Priori, R., Ravelli, A., Sfriso, P., Sinigaglia, L., Alivernini, S., Baldissera, E., Bartoloni, E., Berti, A., Bugatti, S., Camellino, D., Cammelli, D., Caporali, R., Caso, F., Cavallaro, E., Cavalli, G., Colaci, M., Costa, L., Di Scala, G., Emmi, G., Frassi, M., Gerli, R., Giacomelli, R., Gremese, E., Iannone, F., Lapadula, G., Leveghi, L., Lopalco, G., Manna, R., Marotto, D., Mathieu, A., Neri, R., Patisso, I., Piga, M., Punzi, L., Romano, M., Ruscitti, P., Salvarani, C., Scarpa, R., Scrivo, R., Talarico, R., Verrecchia, E., Viapiana, O., Vitale, A., Vitiello, G., Colafrancesco, S., Manara, M., Bortoluzzi, A., Serban, T., Bianchi, G., Cantarini, L., Ciccia, F., Dagna, L., Govoni, M., Montecucco, C., Priori, R., Ravelli, A., Sfriso, P., Sinigaglia, L., Alivernini, S., Baldissera, E., Bartoloni, E., Berti, A., Bugatti, S., Camellino, D., Cammelli, D., Caporali, R., Caso, F., Cavallaro, E., Cavalli, G., Colaci, M., Costa, L., Di Scala, G., Emmi, G., Frassi, M., Gerli, R., Giacomelli, R., Gremese, E., Iannone, F., Lapadula, G., Leveghi, L., Lopalco, G., Manna, R., Marotto, D., Mathieu, A., Neri, R., Patisso, I., Piga, M., Punzi, L., Romano, M., Ruscitti, P., Salvarani, C., Scarpa, R., Scrivo, R., Talarico, R., Verrecchia, E., Viapiana, O., Vitale, A., and Vitiello, G.

Abstract

Following publication of the original article [1], it was brought to our attention that the AOSD Consensus Group was incorrectly tagged and therefore not searchable. The publishers apologize for this error.

Published
2020

45. Golimumab effectiveness in biologic inadequate responding patients with rheumatoid arthritis, psoriatic arthritis and spondyloarthritis in real-life from the Italian registry GISEA

Author

Iannone, F., Favalli, E. G., Caporali, R., D'Angelo, S., Cantatore, F. P., Sarzi-Puttini, P., Foti, Roberta, Conti, Francesco, Carletto, A., Gremese, Elisa, Cauli, A., Ramonda, R., Palermo, A., Epis, O., Priora, M., Bergossi, F., Frediani, B., Salaffi, F., Lopalco, G., Cacciapaglia, F., Marchesoni, A., Biggioggiero, M., Bugatti, S., Balduzzi, S., Carriero, Alessandro, Corrado, A., Bongiovanni, S., Benenati, A., Miranda, F., Fracassi, E., Perra, D., Ferraccioli, Gianfranco, Lapadula, G., Foti R., Conti F., Gremese E. (ORCID:0000-0002-2248-1058), Carriero A., Ferraccioli G. (ORCID:0000-0001-6246-2428), Iannone, F., Favalli, E. G., Caporali, R., D'Angelo, S., Cantatore, F. P., Sarzi-Puttini, P., Foti, Roberta, Conti, Francesco, Carletto, A., Gremese, Elisa, Cauli, A., Ramonda, R., Palermo, A., Epis, O., Priora, M., Bergossi, F., Frediani, B., Salaffi, F., Lopalco, G., Cacciapaglia, F., Marchesoni, A., Biggioggiero, M., Bugatti, S., Balduzzi, S., Carriero, Alessandro, Corrado, A., Bongiovanni, S., Benenati, A., Miranda, F., Fracassi, E., Perra, D., Ferraccioli, Gianfranco, Lapadula, G., Foti R., Conti F., Gremese E. (ORCID:0000-0002-2248-1058), Carriero A., and Ferraccioli G. (ORCID:0000-0001-6246-2428)

Abstract

Objective: To evaluate the clinical effectiveness of golimumab in biologic inadequate responder (IR) patients with Rheumatoid arthritis (RA), Spondyloarthritis (SpA), and Psoriatic arthritis (PsA). Methods: We analyzed 1424 patients on golimumab from the GISEA registry. Drug survival was estimated by Kaplan-Meier analysis in biologic-naïve, 1-biologic IR, ≥ 2-biologics IR patients. Hazard ratios (HRs) of discontinuing golimumab at 2 years were assessed by multivariate Cox regression. Patients achieving CDAI based low disease activity (LDA) or BASDAI < 4 were calculated at 6 and 12 months. Results: In RA (n.370), the 2-years survival on golimumab was 61.4% in 1-biologic IR, 51.9% in ≥ 2-biologics IR, and 73.1% in biologic-naive patients (P = 0.002 vs ≥ 2-biologics IR). In SpA (n.502), the survival was similar among 1-biologic IR (80%), ≥ 2-biologics IR (76.5%), and biologic-naive (74.6%) patients (P > 0.05). In PsA (n.552) the survival was 72% in 1-biologic IR, 72.5% in ≥ 2-biologics IR, and 71.8% in naïve-biologic (P > 0.05). Predictors of golimumab discontinuation were monotherapy (HR 1.65) for RA, female gender for SpA (HR 2.48) and PsA (HR 1.57). In RA, patients on CDAI-LDA were lower in 1-biologic IR (40%) or ≥ 2 biologics IR (40%) than in biologic-naïve (60%) group at 6 months (P = 0.02), but no difference was observed at 12 months. In PsA and SpA, the percentage of patients on CDAI-LDA or BASDAI < 4 at 6 months was almost identical across the subgroups. Conclusions: Golimumab had similar effectiveness in biologic-failure and biologic-naïve SpA and PsA, but seems to be less effective in multi-failure RA patients, especially as monotherapy. The best outcomes were seen in male patients.

Published
2021

46. 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

47. Drug survival of anakinra and canakinumab in monogenic autoinflammatory diseases: observational study from the International AIDA Registry

Author

Sota, J, Rigante, D, Cimaz, R, Cattalini, M, Frassi, M, Manna, R, Sicignano, Ll, Verrecchia, E, Aragona, E, Maggio, Mc, Lopalco, G, Emmi, G, Parronchi, P, Cauli, A, Wiesik-Szewczyk, E, Hernández-Rodríguez, J, Gaggiano, C, Tarsia, M, Mourabi, M, Ragab, G, Vitale, A, Fabiani, C, Frediani, B, Lamacchia, V, Renieri, A, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Manna R (ORCID:0000-0003-1560-3907), Sicignano LL, Verrecchia E, Sota, J, Rigante, D, Cimaz, R, Cattalini, M, Frassi, M, Manna, R, Sicignano, Ll, Verrecchia, E, Aragona, E, Maggio, Mc, Lopalco, G, Emmi, G, Parronchi, P, Cauli, A, Wiesik-Szewczyk, E, Hernández-Rodríguez, J, Gaggiano, C, Tarsia, M, Mourabi, M, Ragab, G, Vitale, A, Fabiani, C, Frediani, B, Lamacchia, V, Renieri, A, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Manna R (ORCID:0000-0003-1560-3907), Sicignano LL, and Verrecchia E

Abstract

Objectives: To investigate survival of interleukin (IL)-1 inhibitors in monogenic autoinflammatory disorders (mAID) through drug retention rate (DRR) and identify potential predictive factors of drug survival from a real-life perspective. Patients and methods: Multicentre retrospective study analyzing patients affected by the most common mAID treated with anakinra or canakinumab. Survival curves were analyzed with the Kaplan-Meier method. Statistical analysis included a Cox-proportional hazard model to detect factors responsible for drug discontinuation. Results: Seventy-eight patients for a total of 102 treatment regimens were enrolled. The mean treatment duration was 29.59 months. The estimated DRR of IL-1 inhibitors at 12, 24, and 48 months of follow-up was 75.8%, 69.7% and 51.1%, respectively. Patients experiencing an adverse event had a significantly lower DRR (p = 0.019). In contrast, no significant differences were observed between biologic-naïve patients and those previously treated with biologic drugs (p = 0.985) Patients carrying high-penetrance mutations exhibited a significantly higher DRR compared with those with low-penetrance variants (p = 0.015). Adverse events were the only variable associated with a higher hazard of treatment withdrawal (HR 2.573 [CI: 1.223-5.411], p = 0.013) on regression analysis. A significant glucorticoid-sparing effect was observed (p < 0.0001). Conclusions: IL-1 inhibitors display an excellent long-term effectiveness in terms of DRR, and their survival is not influenced by the biologic line of treatment. They display a favorable safety profile, that deserves however a close monitoring given its impact on treatment continuation. Special attention should be paid to molecular diagnosis and mutation penetrance, as patients carrying low-penetrance variants are more likely to interrupt treatment.

Published
2021

48. Clinical profile and evolution of patients with juvenile-onset Behçet’s syndrome over a 25-year period: insights from the AIDA network

Author

Sota, J, Rigante, Donato, Lopalco, G, Emmi, G, Gentileschi, S, Gaggiano, C, Ciarcia, L, Berlengiero, V, Mourabi, M, Ricco, N, Barneschi, S, Tosi, Gm, Frediani, B, Tarsia, M, di Scala, G, Vitale, A, Iannone, F, Fabiani, C, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Sota, J, Rigante, Donato, Lopalco, G, Emmi, G, Gentileschi, S, Gaggiano, C, Ciarcia, L, Berlengiero, V, Mourabi, M, Ricco, N, Barneschi, S, Tosi, Gm, Frediani, B, Tarsia, M, di Scala, G, Vitale, A, Iannone, F, Fabiani, C, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Behçet’s syndrome (BS) represents an understudied topic in pediatrics: the main aims of our study were to characterize demographic and clinical features of a cohort of BS patients with juvenile-onset managed in three tertiary referral centers in Italy, evaluate their evolution in the long-term, and detect any potential differences with BS patients having an adult-onset. Medical records of 64 juvenile-onset and 332 adult-onset BS followed-up over a 2-year period were retrospectively analyzed and compared. Mean age ± SD of first symptom-appearance was 10.92 ± 4.34 years with a female-to-male ratio of 1.06:1. Mucocutaneous signs were the most frequent initial manifestations, followed by uveitis. Throughout the disease course, genital aphthae (76.56%) and pseudofolliculitis (40.63%) prevailed among the mucocutaneous signs, while major organ involvement was represented by gastrointestinal and ocular involvement (43.75 and 34.38%, respectively). No significant differences emerged for both mucocutaneous signs and specific major organ involvement between juvenile-onset and adult BS patients. After excluding nonspecific abdominal pain, juvenile-onset BS patients were less frequently characterized by the development of major organ involvement (p = 0.027). Logistic regression detected the juvenile-onset as a variable associated with reduced risk of long-term major organ involvement (OR 0.495 [0.263–0.932], p = 0.029). In our cohort, juvenile-onset BS resembled the clinical spectrum of adult-onset patients. Pediatric patients with a full-blown disease at onset showed a more frequent mucocutaneous involvement. In addition, patients with juvenile-onset seemed to develop less frequently major organ involvement and had an overall less severe disease course.

Published
2021

50. OP0093 RETENTION RATE OF IL-1 INHIBITORS IN PATIENTS WITH SCHNITZLER’S SYNDROME

Author

Crisafulli, F., primary, Vitale, A., additional, Gaggiano, C., additional, Dagna, L., additional, Cavalli, G., additional, Cimaz, R., additional, Viapiana, O., additional, Iannone, F., additional, Lopalco, G., additional, Bortolotti, R., additional, Abdel Jaber, M., additional, Montecucco, C., additional, Monti, S., additional, Balduzzi, S., additional, Emmi, G., additional, Airò, P., additional, Franceschini, F., additional, Cantarini, L., additional, and Frassi, M., additional

Published
2021
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