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1. Safety and Efficacy of Long-Term Tocilizumab in a Cohort of Patients with Giant Cell Arteritis: An Italian Monocentric Retrospective Study

Author

Terribili R, Grazzini S, Conticini E, Falsetti P, Biasi G, Fabiani C, Cantarini L, and Frediani B

Subjects
giant cell arteritis, vasculitis, tocilizumab, biological therapy, safety, Medicine (General), R5-920
Abstract

Riccardo Terribili,1 Silvia Grazzini,1 Edoardo Conticini,1 Paolo Falsetti,1 Giovanni Biasi,1 Claudia Fabiani,2 Luca Cantarini,1 Bruno Frediani1 1Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy; 2Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, ItalyCorrespondence: Edoardo Conticini, Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy, Email conticini.edoardo@gmail.comObjective: Tocilizumab (TCZ) is the only biologic drug approved for the treatment of giant cell arteritis (GCA), having clinical trials and real-life studies proved its efficacy and safety. However, the optimal duration of the treatment has yet to be determined, being its early interruption associated with an increased risk of relapse. Conversely, prolonged schemes of therapy may rise safety concerns. The aim of the study was to evaluate the incidence of adverse events (AEs) and remission/relapse rate in a cohort of GCA patients treated with TCZ and an accelerated steroid tapering scheme, followed for 24 months.Methods: We retrospectively included patients referring to our clinic from January 2019 to November 2021 who were diagnosed with GCA and started subcutaneous TCZ treatment (162 mg/week). They also received up to 62,5 mg of prednisone (PDN), tapered following an accelerated six-month scheme.Results: We collected 38 patients, with a mean age of 76,4 years, treated with TCZ for an average of 22,3 months. AEs occurred in 11 (29%) subjects, and only one serious AE was reported; 7 (18%) patients permanently discontinued TCZ. At the end of the follow-up, all the patients continuing treatment showed clinical remission, with a PDN dosage < 5mg. We registered 3 (8%) minor relapses under TCZ, after an average of 15 months.Conclusion: Our data support the evidence of a safe and effective long-term use of TCZ in GCA patients, especially when combined with moderate GCs doses for the shortest possible duration.Keywords: giant cell arteritis, vasculitis, tocilizumab, biological therapy, safety

Published
2024

2. Tocilizumab Vs Methotrexate in a Cohort of Patients Affected by Active GCA: A Comparative Clinical and Ultrasonographic Study

Author

Grazzini S, Conticini E, Falsetti P, D'Alessandro M, Sota J, Terribili R, Baldi C, Fabiani C, Bargagli E, Cantarini L, and Frediani B

Subjects
gca, giant cell arteritis, methotrexate, tocilizumab, ultraonography, vasculitis., Medicine (General), R5-920
Abstract

Silvia Grazzini,1,&ast; Edoardo Conticini,1,&ast; Paolo Falsetti,1 Miriana D’Alessandro,2 Jurgen Sota,1 Riccardo Terribili,1 Caterina Baldi,1 Claudia Fabiani,3 Elena Bargagli,2 Luca Cantarini,1 Bruno Frediani1 1Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy; 2Respiratory Diseases and Lung Transplant Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy; 3Ophthalmology Unit, Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy&ast;These authors contributed equally to this workCorrespondence: Edoardo Conticini, Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, AOUS Le Scotte, viale Mario Bracci 14, Siena, 53100, Italy, Email conticini.edoardo@gmail.comIntroduction: No head-to-head study has assessed the superiority of tocilizumab versus methotrexate in giant cell arteritis (GCA), and few studies have demonstrated its effectiveness in terms of ultrasonographic findings, but without a control group. The primary endpoint was to assess whether tocilizumab was superior to methotrexate in inducing normalization of US findings, whereas the secondary endpoint was to assess the effectiveness of precocious withdrawal of glucocorticoids.Methods: We prospectively enrolled all the patients with active GCA at our clinic. The inclusion criteria were clinical diagnosis of GCA; active disease; and clinical, laboratory, and US data, evaluated using the halo count (HC) and OMERACT GCA Ultrasonography Score (OGUS). Evaluations were repeated at 3, 6, and 12 months.Results: Twenty patients were treated with Tocilizumab and 9 with Methotrexate. All but three tocilizumab-treated patients achieved remission at six months, whereas at 12 months, all patients were in glucocorticoid-free remission. Up to three of the nine methotrexate patients experienced a lack of efficacy or minor relapses. Tocilizumab-treated patients showed a statistically significant difference between baseline and all follow-ups in terms of OGUS and HC, whereas the difference in the Methotrexate group was significant after 1 year. The mean glucocorticoid dosage significantly decreased in both groups. No severe adverse events or major relapses were reported.Conclusion: Our study demonstrates the superiority in terms of rapidity of a tocilizumab-based scheme over a methotrexate-based scheme in inducing clinical and US remission. Precocious withdrawal of glucocorticoids did not increase the risk of relapse.Keywords: GCA, giant cell arteritis, methotrexate, tocilizumab, ultrasonography, vasculitis

Published
2023

7. Modelling urban-scale occupant behaviour, mobility, and energy in buildings: A survey

Author

Salim, FD, Dong, B, Ouf, M, Wang, Q, Pigliautile, I, Kang, X, Hong, T, Wu, W, Liu, Y, Rumi, SK, Rahaman, MS, An, J, Deng, H, Shao, W, Dziedzic, J, Sangogboye, FC, Kjærgaard, MB, Kong, M, Fabiani, C, Pisello, AL, and Yan, D

Subjects
Environmental Science and Management, Architecture, Building, Building & Construction
Abstract

The proliferation of urban sensing, IoT, and big data in cities provides unprecedented opportunities for a deeper understanding of occupant behaviour and energy usage patterns at the urban scale. This enables data-driven building and energy models to capture the urban dynamics, specifically the intrinsic occupant and energy use behavioural profiles that are not usually considered in traditional models. Although there are related reviews, none investigated urban data for use in modelling occupant behaviour and energy use at multiple scales, from buildings to neighbourhood to city. This survey paper aims to fill this gap by providing a critical summary and analysis of the works reported in the literature. We present the different sources of occupant-centric urban data that are useful for data-driven modelling and categorise the range of applications and recent data-driven modelling techniques for urban behaviour and energy modelling, along with the traditional stochastic and simulation-based approaches. Finally, we present a set of recommendations for future directions in data-driven modelling of occupant behaviour and energy in buildings at the urban scale.

Published
2020

13. Uterine schwannoma during pregnancy: a rare case report

Author

Cignini, P., primary, Spina, V., additional, Bisogni, F., additional, Fabiani, C., additional, Schiavi, M.C., additional, Grilli, D., additional, Campanella, L., additional, Etrusco, A., additional, Rago, R., additional, and Palazzetti, P., additional

Published
2024
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14. 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

18. 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

19. 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

25. 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

26. Microclimate Assessment at Real Experimental Conditions for Green Energy Urban Policy

Author

Piselli, C., Balocco, C., Pigliautile, I., Fabiani, C., Cureau, R. J., Sciurpi, F., Carletti, C., Pisello, A. L., and Cotana, F.

Subjects
Renewable Energy, Sustainability and the Environment, Geography, Planning and Development, Management, Monitoring, Policy and Law
Abstract

The incoming transformation of urban built-up areas, surroundings morphology, and local climate due to global warming connected to the necessity of renewable energy use maximization is the fundamental to the main aim of the present research. Indeed, urban policies can benefit from the accurate monitoring of microclimate variables in order to ensure fine living standards in cities, while improving the energy performance of the built environment. In this view, a systemic experimental approach was implemented. A monitoring campaign using dynamic experimental measurements under real conditions was carried out at an inter-urban scale taking into account different building-plant systems forms and urban configuration. In detail, two innovative portable monitoring systems were used for monitoring key multi-domain parameters at hyperlocal, urban, and intra-urban scale. The monitoring campaign was carried out in summer and winter in the city center of Florence, Italy. Research findings highlighted that urban microclimate control can be a potential factor for urban heat island (UHI) mitigation and sustainable green energy solutions, which involve social, economic, and energy policy beyond environment. The analysis of real microclimate conditions may support the green energy urban policy development in terms of renewable energy integration and urban areas design and management.

Published
2022
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27. 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

28. 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

29. 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

30. 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

33. POS0756 EFFICACY AND SAFETY OF ADALIMUMAB IN PEDIATRIC NON-INFECTIOUS NON-ANTERIOR UVEITIS: REAL-LIFE EXPERIENCE FROM AIDA INTERNATIONAL REGISTRY

Author

Fabiani, C., primary, Vitale, A., additional, Della Casa, F., additional, Guerriero, S., additional, Ragab, G., additional, Brucato, A., additional, Cattalini, M., additional, Del Giudice, E., additional, Marino, A., additional, Paroli, M. P., additional, Gaggiano, C., additional, Sota, J., additional, Caggiano, V., additional, Tarsia, M., additional, Frediani, B., additional, and Cantarini, L., additional

Published
2023
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34. 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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35. 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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36. 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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39. Environmental assessment of four waste cooking oil valorization pathways

Author

FROTA DE ALBUQUERQUE LANDI, Fabiana, Fabiani, C., Castellani, B., Cotana, F., and Pisello, A. L.

Subjects
Bio-materials, Life cycle assessment, Circular economy, Sustainability, Waste Management, Biofuels, Plant Oils, Recycling, Waste cooking oil, Cooking, Waste Management and Disposal
Abstract

Global waste is expected to grow substantially by 2050, therefore, defining an effective waste management strategy is a crucial topic for both industry and academia. Nowadays, food and green waste, in particular, represent a large share of the total waste production. All this considered, effectively processing and eventually reusing materials such as waste cooking oil is of paramount importance. This study investigates the potential environmental impact and the primary energy consumption for three waste cooking oil valorization pathways i.e. biodiesel, direct burning fuel, additive for recycling aged-asphalt, as well as a new application, i.e. phase change material, compared to their specific more common alternative based on a cradle-to-gate approach. The aim is to identify and recommend the most advantageous alternative in terms of environmental impact. Results showed that the waste cooking oil has a lower impact in all comparisons made, except as phase change material. The less effective performance in some cases was compensated by the waste oil entry as a burden-free resource under an attributional model. The best profile of the waste cooking oil is as direct burning fuel. However, the binder asphalt substitution is highly recommended due to the nature of the application. The major obstacles to the waste cooking oil usage are the limited stock, composition and quality variability, and the difficulty of proper collection.

Published
2022
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40. Smart-Earth Multifunctional Cement Composites for Sustainable Constructions: Thermal and Sensing Characterization

Author

Meoni, A., Fabiani, C., D'Alessandro, A., Pisello, A. L., and Ubertini, F.

Subjects
Self-sensing construc-tion materials, Structural Health Monitoring, Smart sustainable composites, Carbon microfibers, Smart-earth concrete, Sustainable structures, Carbon microfibers, Environmental testing, Self-sensing construc-tion materials, Smart-earth concrete, Smart sustainable composites, Sustainable structures, Structural Health Monitoring, Environmental testing
Published
2023

43. Impact of different typologies of smoking on ovarian reserve and oocyte quality in women performing ICSI cycles: an observational prospective study.

Author

GALANTI, F., LICATA, E., PACIOTTI, G., GALLO, M., RICCIO, S., MIRIELLO, D., LAGO, A. DAL, MENEGHINI, C., FABIANI, C., ANTONACI, D., SCHIAVI, M. C., SCUDO, M., SALACONE, P., SEBASTIANELLI, A., BATTAGLIA, F. A., and RAGO, R.

Abstract

OBJECTIVE: Within the last few years smoking activities, as well as infertility, have increased in Italy, and so has the consumption of alternative cigarette devices among women of childbearing age. The aim of this observational study was to evaluate the impact of the consumption of cigarettes and alternatives devices, such as electronic cigarettes and heat-notburn (HnB) products, on infertile women performing in vitro fertilization (IVF), in specific on the quality of oocytes retrieved in women performing intracytoplasmic sperm injection (ICSI) cycles. PATIENTS AND METHODS: Prospective observational longitudinal study involving 410 women referring to the Reproductive Physiopathology and Andrology Unit, Sandro Pertini Hospital, Rome, from 2019-2022. All the women enrolled filled out an elaborate questionnaire investigating smoking consumption, before the beginning of ovarian stimulation by antagonist protocol, ovarian pick-up, and subsequent ICSI technique. The outcomes of the study were the evaluation of clinical and ICSI features between the groups of smokers and non-smokers: the number of retrieved oocytes, immature oocytes, and fertilization rate were confronted between the two groups and between cigarette smokers vs. e-cigarette and heat-not-burn (HnB) products smokers. RESULTS: Clinical parameters were comparable between the group of smokers compared to one of the non-smokers, except for anti-Müllerian hormone (AMH), which was statistically lower in smokers (p<0.05). Regarding IVF hormonal stimulations it appears that the total dose of gonadotropin was statistically lower in the non-smoker’s group, compared to smokers (1850±860 UI vs. 1,730±780 p<0.05). Regarding ICSI techniques interestingly the number of oocytes retrieved was lower in the smokers’ group compared to non-smokers (5.21±0.9 vs. 6.55±3.5, p<0.001), and the number of empty zona pellucida oocytes was statistically higher in the smokers’ group (0.51±0.1 vs. 0.2±0.1, p<0.05). On the other hand, the fertilization rate (FR) was statistically higher in non-smokers compared to the smokers’ group (72.16±3.05 vs. 68.12±2.21, p=0.03). Out of the 203 smokers, overall, any statistically significant difference, regarding ICSI results, has been found between the group of cigarette smokers, compared to the group of e-cigarettes plus HnB products smokers. CONCLUSIONS: Smoking negatively impacts human fertility, leading to a reduction of ovarian reserve and ovarian quality, which can negatively impact results in women performing ICSI cycles. Despite the limitation of the study, our results underline that consumption of cigarette alternative devices seems to have a similar negative impact on the quantity and quality of oocytes retrieved in ICSI cycles. Clinicians should emphasize the reduction of exposure to harmful substances derived from the combustion of tobacco smoking, as well as alternative devices, in women of childbearing age. [ABSTRACT FROM AUTHOR]

Published
2023

46. 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

47. 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

48. 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

49. 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

50. 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

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