Author: "Sfikakis PP" - Searchworks@Jio Institute Digital Library Search Results

Your search keyword '"Sfikakis PP"' showing total 700 results

Start Over Author "Sfikakis PP"

700 results on '"Sfikakis PP"'

1. Effect of Biologics on Cardiovascular Inflammation: Mechanistic Insights and Risk Reduction

Author: Fragoulis GE, Soulaidopoulos S, Sfikakis PP, Dimitroulas T, and Kitas GD
Subjects: atherosclerosis, cardiovascular disease, biologic agents, interleukins, inflammatory disorders, Pathology, RB1-214, Therapeutics. Pharmacology, RM1-950
Abstract: George E Fragoulis,1,* Stergios Soulaidopoulos,2,* Petros P Sfikakis,1 Theodoros Dimitroulas,3 George D Kitas4,5 1Rheumatology Unit, Joint Rheumatology Program, Medical School, First Department of Propaedeutic Internal Medicine, National and Kapodistrian University of Athens, “Laiko” General Hospital, Athens, 115 27, Greece; 2First Department of Cardiology, National and Kapodistrian University of Athens, Hippokration General Hospital, Athens, 115 27, Greece; 3Fourth Department of Internal Medicine, Hippokration Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, 546 41, Greece; 4Department of Rheumatology, Russells Hall Hospital, Dudley Group NHS FT, Dudley, DY1 2HQ, UK; 5Arthritis Research UK Epidemiology Unit, University of Manchester, Manchester, M13 9PT, UK*These authors contributed equally to this workCorrespondence: Stergios SoulaidopoulosFirst Department of Cardiology, National and Kapodistrian University of Athens, 114 Vasilissis Sofias av., Athens, 11527, GreeceTel +306932528561Fax +302132088676Email soulaidopoulos@hotmail.comAbstract: It is increasingly recognized that atherosclerosis and consequently cardiovascular disease (CVD) are closely linked with inflammatory processes. The latter is in the center of the pathogenic mechanism underlying autoimmune rheumatic diseases (ARD). It follows then, that optimal control of inflammation in ARDs may lead to a decrease of the accompanied CVD risk. Major trials (eg, CANTOS, CIRT), aimed at examining the possible benefits of immunomodulatory treatments in CVD, demonstrated conflicting results. On the other hand, substantial evidence is accumulating about the possible beneficial effects of biologic disease modifying antirheumatic drugs (bDMARDs) in patients with ARDs, particularly those with rheumatoid arthritis (RA). It seems that bDMARDs (some more than others) alter the lipid profile in RA patients but do not adversely affect, in most cases, the TC/HDL ratio. Favorable effects are noted for arterial stiffness and endothelial function. This is reflected in the lower risk for CVD events, seen in observational studies of RA patients treated with bDMARDs. It should be stressed that more data exist for the TNF-inhibitors than for other bDMARDs, such as tocilizumab, abatacept and rituximab. As regards the spondyloarthropathies (SpA), data are less robust. For TNF-inhibitors, effects appear to be on par with those seen in RA but no conclusions can be drawn for newer biologic drugs used in SpA (eg, IL-17 blockers). Finally, there is accumulating evidence for a beneficial effect of immunosuppressive treatment in cardiac inflammation and function in several ARDs. Introduction of newer therapeutic options in clinical practice seem to have a positive impact on CVD in the setting of ARD. This is probably due to better control of inflammation, but direct improvement in vascular pathology is also a valid hypothesis. Most data are derived from observational studies and, therefore, randomized controlled trials are needed to assess the possible favorable effect of bDMARDs on CVD outcomes.Keywords: atherosclerosis, cardiovascular disease, biologic agents, interleukins, inflammatory disorders
Published: 2021

2. 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

3. 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

4. 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

5. 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

6. 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

7. 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

8. 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

9. 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

10. 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

11. 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

12. 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

13. 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

14. Correction: Chromatin structure, transcriptional activity and DNA repair efficiency affect the outcome of chemotherapy in multiple myeloma

Author: Gkotzamanidou, M Sfikakis, PP Kyrtopoulos, SA Bamia, C Dimopoulos, MA Souliotis, VL
Subjects: Health Sciences, Επιστήμες Υγείας
Published: 2020

15. European Headache Federation recommendations for placebo and nocebo terminology

Author: Mitsikostas, DD, Blease, C, Carlino, E, Colloca, L, Geers, AL, Howick, J, Evers, AWM, Flaten, MA, Kelley, JM, Kirsch, I, Klinger, R, Maassen van den Brink, Antoinette, Moerman, DE, Sfikakis, PP, Vase, L, Wager, TD, Benedetti, F, Mitsikostas, DD, Blease, C, Carlino, E, Colloca, L, Geers, AL, Howick, J, Evers, AWM, Flaten, MA, Kelley, JM, Kirsch, I, Klinger, R, Maassen van den Brink, Antoinette, Moerman, DE, Sfikakis, PP, Vase, L, Wager, TD, and Benedetti, F
Published: 2020

16. Transient Encephalopathy Followed by Seizures after Docetaxel Infusion

Author: Polyzos, A, Daskalopoulou, E, Giannitsiotis, E, Gatzonis, S, and Sfikakis, PP
Published: 1999

17. Prolonged Survival in Metastatic Adenocarcinoma of the Small Intestine Treated with Irinotecan

Author: Polyzos, Aristidis, Bramis, J, Delladetsima, JK, and Sfikakis, PP
Published: 1999

18. Double Biochemical Modulation of 5-Fluorouracil by Methotrexate and Folinic Acid for the Treatment of Advanced Colorectal Cancer

Author: Polyzos, A, Tsavaris, N, Kosmas, C, Petrikos, G, Stamatiadis, D, Kalahanis, N, Giannopoulos, A, Karatzas, G, and Sfikakis, PP
Published: 1999

19. PS4:68 Carotid and femoral atherosclerosis in antiphospholipid syndrome: equivalent risk with diabetes mellitus in a case-control study

Author: Tektonidou, M, primary, Kravvariti, E, additional, Konstantonis, G, additional, Tentolouris, N, additional, and Sfikakis, PP, additional
Published: 2018
Full Text: View/download PDF

20. Secukinumab after anti-tumour necrosis factor-α therapy: a phase III study in active rheumatoid arthritis

Author: Dokoupilová, E, primary, Aelion, J, additional, Takeuchi, T, additional, Malavolta, N, additional, Sfikakis, PP, additional, Wang, Y, additional, Rohrer, S, additional, and Richards, HB, additional
Published: 2018
Full Text: View/download PDF

21. Behcet's disease: a new target for anti-tumour necrosis factor treatment. (Report)

Author: Sfikakis, PP
Subjects: Behcet's disease -- Care and treatment, Tumor necrosis factor -- Physiological aspects, Health, Care and treatment, Physiological aspects
Abstract: Behcet's disease is a multisystemic, chronic relapsing inflammatory disease classified among the vasculitides. Recurrent mucocutaneous lesions may be the only symptoms in mild cases, but articular, ocular, vascular, and/or gastrointestinal [...]
Published: 2002

22. FRI0306 Long term drug-free remission is feasible in severe behcet's disease after cessation of successful anti-tnf treatment: a single center, retrospective longitudinal outcome study

Author: Sfikakis, PP, primary, Arida, A, additional, Panopoulos, S, additional, Fragiadaki, K, additional, Pentazos, G, additional, Laskari, A, additional, Tektonidou, M, additional, and Markomichelakis, N, additional
Published: 2017
Full Text: View/download PDF

23. SAT0176 Patterns of biologic dmard monotherapy in a large nationwide rheumatoid arthritis cohort: data from 1036 patients

Author: Thomas, K, primary, Kaltsonoudis, E, additional, Drosos, A, additional, Papalopoulos, I, additional, Sidiropoulos, P, additional, Katsimbri, P, additional, Boumpas, D, additional, Tsatsani, P, additional, Gazi, S, additional, Grika, EP, additional, Vlachoyiannopoulos, PG, additional, Fragiadaki, K, additional, Tektonidou, M, additional, Sfikakis, PP, additional, Karagianni, K, additional, Sakkas, LI, additional, Pantazi, L, additional, Boki, K, additional, Dimitroulas, T, additional, Garyfallos, A, additional, Kasimos, D, additional, Evangelatos, G, additional, Iliopoulos, A, additional, Georganas, C, additional, Vounotrypidis, P, additional, Areti, M, additional, Georgiou, P, additional, Delis, K, additional, Mavragani, K, additional, Bournazos, I, additional, Katsifis, G, additional, Mavromatis, C, additional, Kitas, GD, additional, and Vassilopoulos, D, additional
Published: 2017
Full Text: View/download PDF

24. SAT0361 Chest computed tomography as an alternative to high resolution manometry for the diagnosis of scleroderma esophagus

Author: Panopoulos, S, primary, Bournia, VK, additional, Karamanolis, G, additional, Denaxas, K, additional, Zorbala, A, additional, and Sfikakis, PP, additional
Published: 2017
Full Text: View/download PDF

25. FRI0410 Predictors of morbidity and mortality in patients with early systemic sclerosis: long-term follow-up from a single centre inception cohort study

Author: Panopoulos, S, primary, Bournia, V-K, additional, Konstantonis, G, additional, Fragiadiaki, K, additional, Sfikakis, PP, additional, and Tektonidou, MG, additional
Published: 2017
Full Text: View/download PDF

26. AB0117 CADHERIN-11 MRNA expression is increased in the peripheral blood of patients with active spondylarthropathy: a pilot study

Author: Vlachogiannis, NI, primary, Christopoulos, PF, additional, Bamias, G, additional, Fragiadaki, K, additional, Tektonidou, M, additional, and Sfikakis, PP, additional
Published: 2017
Full Text: View/download PDF

27. SAT0415 High prevalance of hip arthritis in patients with ankylosing spondylitis treated with tnf inhibitors

Author: Konsta, M, primary, Nurmohamed, MT, additional, Iliopoulos, A, additional, Denderen, JC van, additional, Visman, I, additional, Sfikakis, PP, additional, and van der Horst-Bruinsma, IE, additional
Published: 2017
Full Text: View/download PDF

28. THU0028 Type i interferon signature in the peripheral blood and CXCL4 plasma levels in patients with systemic sclerosis

Author: Vlachogiannis, NI, primary, Bournia, V-K, additional, Nezos, A, additional, Christopoulos, PF, additional, Patsouras, M, additional, Vlachoyiannopoulos, PG, additional, Mavragani, CP, additional, and Sfikakis, PP, additional
Published: 2017
Full Text: View/download PDF

29. AB0244 Microwave radiometry-derived thermal changes of small joints as potential additional biomarker in rheumatoid arthritis: a prospective study

Author: Laskari, K, primary, Pentazos, G, additional, Prekas, K, additional, Raftakis, I, additional, Siores, E, additional, and Sfikakis, PP, additional
Published: 2017
Full Text: View/download PDF

30. FRI0414 Can echocardiography replace follow-up cardiac catheterization in re-evaluation of pulmonary arterial hypertension? a longitudinal single-center study of 30 connective tissue disease patients

Author: Bournia, V-K, primary, Tsangaris, I, additional, Rallidis, L, additional, Konstantonis, D, additional, Anthi, A, additional, Orfanos, S, additional, Panopoulos, S, additional, and Sfikakis, PP, additional
Published: 2017
Full Text: View/download PDF

31. FRI0455 Radiographic progression of hip arthritis in patients with ankylosing spondylitis treated with tnf inhibitors

Author: Konsta, M, primary, Nurmohamed, MT, additional, Iliopoulos, A, additional, Denderen, JC van, additional, Visman, I, additional, Sfikakis, PP, additional, and van der Horst-Bruinsma, IE, additional
Published: 2017
Full Text: View/download PDF

32. CENTRAL AORTIC SYSTOLIC BLOOD PRESSURE MEASUREMENTS IN RHEUMATOID ARTHRITIS: TOWARDS THE OPTIMIZATION OF THE BLOOD PRESSURE-ASSOCIATED CARDIOVASCULAR RISK ASSESSMENT

Author: Protogerou, A. D. Konstantonis, G. Aisopou, E. Arida, A. and Zampeli, E. Kitas, G. D. Sfikakis, Pp.
Published: 2015

33. Chromatin structure, transcriptional activity and DNA repair efficiency affect the outcome of chemotherapy in multiple myeloma

Author: Gkotzamanidou, M Sfikakis, PP Kyrtopoulos, SA Bamia, C Dimopoulos, MA Souliotis, VL
Subjects: immune system diseases, hemic and lymphatic diseases, Health Sciences, Επιστήμες Υγείας
Abstract: Background: Melphalan is one of the most active chemotherapeutic agents in the treatment of multiple myeloma (MM). However, the mechanism underlying differential patient responses to melphalan therapy is unknown. Methods: Chromatin structure, transcriptional activity and DNA damage response signals were examined following ex vivo treatment with melphalan of both malignant bone marrow plasma cells (BMPCs) and peripheral blood mononuclear cells (PBMCs) of MM patients, responders (n = 57) or non-responders (n = 28) to melphalan therapy. PBMCs from healthy controls (n = 25) were also included in the study. Results: In both BMPCs and PBMCs, the local chromatin looseness, transcriptional activity and repair efficiency of the transcribed strand (TS) were significantly higher in non-responders than in responders and lowest in healthy controls (all P
Published: 2014

34. Progressive changes in chromatin structure and DNA damage response signals in bone marrow and peripheral blood during myelomagenesis

Author: Gkotzamanidou, M Terpos, E Bamia, C Kyrtopoulos, SA Sfikakis, PP Dimopoulos, MA Souliotis, VL
Subjects: Health Sciences, Επιστήμες Υγείας
Abstract: The molecular pathways implicated in multiple myeloma (MM) development are rather unknown. We studied epigenetic and DNA damage response (DDR) signals at selected model loci (N-ras, p53, d-globin) in bone marrow plasma cells and peripheral blood mononuclear cells (PBMCs) from patients with monoclonal gammopathy of undetermined significance (MGUS; n=20), smoldering/asymptomatic MM (SMM; n=29) and MM (n=18), as well as in healthy control-derived PBMCs (n=20). In both tissues analyzed, a progressive, significant increase in the looseness of local chromatin structure, gene expression levels and DNA repair efficiency from MGUS to SMM and finally to MM was observed (all P
Published: 2014

35. The IL-1 inhibitor Canakinumab for Familial Mediterranean Fever: the Greek experience in 12 patients

Author: Laskari, K, primary, Boura, P, additional, Dalekos, GN, additional, Garyfallos, A, additional, Karokis, D, additional, Pikazis, D, additional, Settas, L, additional, Skarantavos, G, additional, Tsitsami, E, additional, and Sfikakis, PP, additional
Published: 2015
Full Text: View/download PDF

36. Lyn dericiency in B cells from patients with systemic lupus erythematosus: comment on the article by Flores-Borja et al

Author: Liossis, SNC Solomou, EE Sfikakis, PP
Published: 2006

37. Regression of neovascular age-related macular degeneration following infliximab therapy

Author: Markomichelakis, NN Theodossiadis, PG Sfikakis, PP
Subjects: genetic structures, sense organs, eye diseases
Abstract: PURPOSE: To describe the effects of the antitumor necrosis factor (TNF) monoclonal antibody Infliximab systemic therapy on choroidal neovacularisation (CNV) secondary to age-related macular degeneration (AMD). DESIGN: Prospective, noncomparative series of three patients. METHODS: A subretinal membrane secondary to AMD was documented by fluoroangiography at baseline in three elderly patients scheduled to receive Infliximab therapy for inflammatory arthritis (infusions of 5 mg/kg at weeks 0, 2, 6, and every 8 weeks thereafter). Follow-up was performed at three months post-baseline, as well as during 18 months of continuing treatment in the first patient. RESULTS: CNV regressed partially at three months and resolved at six months in the first patient. Best-corrected visual acuity (BCVA) increased from 0.05 to 0.2; this effect was sustained at 18 months. Regression of subretinal membrane and increase of BCVA was also documented in the other patients. No ocular or extra-ocular side effects were noted. CONCLUSIONS: These findings suggest a plausible pathogenetic role of TNF in CNV secondary to AMD. Additional patients should he studied to confirm the promising clinical results. (c) 2005 by Elsevier Inc. All rights reserved.
Published: 2005

38. Extent of damage and repair in the p53 tumor-suppressor gene after treatment of myeloma patients with high-dose melphalan and autologous blood stem-cell transplantation is individualized and may predict clinical outcome

Author: Dimopoulos, MA Souliotis, VL Anagnostopoulos, A and Papadimitriou, C Sfikakis, PP
Abstract: Purpose To quantitate the individual levels of melphalan-induced DNA damage formation and repair in vivo and to search for possible correlations with clinical outcome in patients with multiple myeloma (MM). Patients and Methods The formation and subsequent repair of DNA damage (monoadducts and interstrand cross-links) in the p53 tumor-suppressor gene, the proto-oncogene N-ras, and the housekeeping gene beta-actin during the first 24 hours after treatment with high-dose melphalan (HDM; 200 mg/m(2)) supported by autologous blood stem-cell transplantation (ABSCT) was measured in blood leukocytes of 26 patients with MM. The peak DNA adduct levels, the total amount of adducts over time, and the rate of adducts repair in each gene were correlated with response and time to progression after HDM, Results The levels of gene-specific DNA damage formation and the individual repairing capacity varied up to 16-fold among patients, indicating that the melphalan-induced biologic effect in vivo is highly individualized. A significantly greater DNA damage and a slower rate of repair in p53 for all end points under study were found in patients who achieved tumor reduction compared with nonresponding patients. Furthermore, longer progression-free survival correlated with increased peak monoadduct levels in the p53 gene (P = .032). Conclusion Increased DNA damage and slower repairing capacity in the p53 gene from blood leukocytes after HDM correlate with improved outcome of patients with MM who undergo ABSCT. These results suggest that quantitation of such biologic end points may identify patients who are more likely to benefit from this procedure. (c) 2005 by American Society of Clinical Oncology.
Published: 2005

39. Regression of sight-threatening macular edema in type 2 diabetes following treatment with the anti-tumor necrosis factor monoclonal antibody infliximab

Author: Sfikakis, PP Markomichelakis, N Theodossiadis, GP and Grigoropoulos, V Katsilambros, N Theodossiadis, PG
Published: 2005

40. Aortic stiffness in systemic sclerosis is increased independently of the extent of skin involvement

Author: Moyssakis, I Gialafos, E Vassiliou, V Taktikou, E and Katsiari, C Papadopoulos, DP Sfikakis, PP
Subjects: integumentary system, cardiovascular system
Abstract: Objective. To study the stiffness of large arteries in relation to the extent of skin and lung fibrosis, aortic distensibility was examined in patients with diffuse and limited systemic sclerosis (SSc). Methods. Consecutive patients (55 with diffuse and 51 with limited SSc) without signs and symptoms of heart failure or a previous history of arterial hypertension underwent echocardiography and lung function tests. Aortic stiffness was determined non-invasively by aortic distensibility and aortic strain measurements in all patients and in 50 healthy subjects, matched for age and gender. Results. Aortic distensibility in patients with either diffuse (2.03 +/- 0.26 x 10(-6) cm(2) dyn(-1)) or limited SSc (2.12 +/- 0.33) was similarly decreased compared with controls (2.49 +/- 0.36, P
Published: 2005

41. In lupus nephritis, the benefit of rituximab monotherapy, as opposed to rituximab plus cyclophosphamide combination therapy, remains uncertain: comment on the article by Sfikakis et al - Reply

Author: Sfikakis, PP Boletis, JN Moutsopoulos, HA
Published: 2005

42. Remission of proliferative lupus nephritis following B cell depletion therapy is preceded by down-regulation of the T cell costimulatory molecule CD40 ligand - An open-label trial

Author: Sfikakis, PP Boletis, JN Lionaki, S Vigklis, V and Fragiadaki, KG Iniotaki, A Moutsopoulos, HM
Abstract: Objective. Autoreactive B cells play a key role in tissue injury in systemic autoimmune disease, and therefore a treatment resulting in B cell depletion could have benefit. This open-label study was undertaken to evaluate the efficacy of the anti-CD20 monoclonal antibody rituximab in the treatment of lupus nephritis. Methods. Lupus patients with active proliferative nephritis (4 with focal disease and 6 with diffuse disease) received rituximab (4 weekly infusions of 375 mg/m(2)) combined with oral prednisolone. Clinical, laboratory, and immunologic responses, including peripheral lymphocyte subsets measured by flow cytometry, were prospectively assessed at monthly intervals for 12 months. Complete remission of nephritis was defined as normal serum creatinine and albumin levels, inactive urine sediment, and 24-hour urinary protein 50% improvement in all renal parameters that were abnormal at baseline. Results. B cell depletion lasted from I month to 7 months and was well tolerated. Partial remission was achieved in 8 of 10 patients within a median of 2 months (range 1-4 months); in 5 of them, complete remission was subsequently established (at a median of 3 months from baseline), and it was sustained at 12 months in 4.As early as I month from baseline, the expression of the costimulatory molecule CD40 ligand on CD4+ T cells was decreased by 4-fold, and it was almost blocked when partial remission was clinically evident. The expression of T cell activation markers CD69 and HLA-DR was significantly decreased at time points when partial remission was observed, and was further decreased during complete remission. In contrast, in patients who did not exhibit a response or when relapse was detected in patients in whom an initial remission had been achieved, such decreases were not prominent. Serum concentrations of double-stranded DNA autoantibodies were decreased in all patients, regardless of clinical outcome. Conclusion. Following B cell depletion, clinical remission of lupus nephritis is associated with a decrease in T helper cell activation, suggesting an additional role for B cells, independent of autoantibody production, in promoting disease. A controlled trial to confirm these promising clinical results is warranted.
Published: 2005

43. Age-related thymic activity in adults following chemotherapy-induced lymphopenia

Author: Sfikakis, PP Gourgoulis, GM Moulopoulos, LA Kouvatseas, G Theofilopoulos, AN Dimopoulos, MA
Subjects: Health Sciences, Επιστήμες Υγείας
Abstract: Background The potential role of the adult thymus in T-cell homeostasis subsequent to lymphopenia remains the subject of debate. We examined whether thymic activity contributes to reconstitution of the peripheral T-cell pool, a critical process for patients recovering from antineoplastic therapy. Methods In selected patients with various neoplastic diseases we assessed peripheral blood lymphocyte subsets by flow-cytometry, including thymus-derived, CD4+ T cells expressing the CD45RA molecule, and thymic size rebound by CT scan before, and 3, 6 and 12 months after completion of cytotoxic therapy. Results Adult patients (n = 21, mean age of 30 years, range 18-49) had higher baseline numbers of B and lower numbers of NK cells than elderly patients (n = 15, mean age of 79 years, range 70-91), while total T-cell numbers did not differ. Despite the reduction of lymphocyte counts being comparable in the adult (mean of 45%) and elderly (mean of 49%) groups, occurring at, or near, completion of treatment, an enlargement of the previously atrophic thymus was evident in 63% of the adult, but in none of the elderly, subjects. In 22 patients who remained active disease-free during the following year, B cells and NK cells recovered to pretreatment levels as soon as at 3 months, whereas overall T-cell recovery occurred at 6 months post-treatment. Thymic rebound, observed in 11 of 22 patients who were of younger age, correlated significantly with a faster and more complete recovery of CD45RA+ CD4+ (mainly helper-naive) T cells. Conclusion The adult thymus appears capable of regeneration, at least up to middle age, contributing significantly to the reconstitution of the peripheral T-cell pool following chemotherapy-induced lymphopenia. In advanced age, however, although peripheral homeostatic pathways appear intact, regeneration of the naive repertoire is incomplete.
Published: 2005

44. Psoriasis induced by anti-tumor necrosis factor therapy - A paradoxical adverse reaction

Author: Sfikakis, PP Iliopoulos, A Elezoglou, A Kittas, C and Stratigos, A
Subjects: integumentary system
Abstract: Administration of anti-tumor necrosis factor (anti-TNF) agents is beneficial in a variety of chronic inflammatory conditions, including psoriasis. We describe 5 patients in whom psoriasiform skin lesions developed 6-9 months after the initiation of anti-TNF therapy for longstanding, seropositive rheumatoid arthritis (etanercept or adalimumab), typical ankylosing spondylitis (infliximab), and Adamantiades-Behcet’s disease (infliximab). In all 5 patients, the underlying disease had responded well to anti-TNF therapy. Four patients developed a striking pustular eruption on the palms and/or soles accompanied by plaque-type psoriasis at other skin sites, while 1 patient developed thick erythematous scaly plaques localized to the scalp. In 3 patients there was nail involvement with onycholysis, yellow discoloration, and subungual keratosis. Histologic findings from skin biopsies were consistent with psoriasis. None of these patients had a personal or family history of psoriasis. In all patients, skin lesions subsided either with topical treatment alone, or after discontinuation of the responsible anti-TNF agent. The interpretation of this paradoxical side effect of anti-TNF therapy remains unclear but may relate to altered immunity induced by the inhibition of TNF activity in predisposed individuals.
Published: 2005

45. Rituximab anti-B-cell therapy in systemic lupus erythematosus: pointing to the future

Author: Sfikakis, PP Boletis, JN Tsokos, GC
Subjects: immune system diseases
Abstract: Purpose of review To discuss the clinical effects and the immunologic consequences of transient B-cell depletion using the anti-CD20 monoclonal antibody rituximab in systemic lupus erythematosus. Recent findings A total of 100 rituximab-treated patients with severe disease, refractory to major immunosuppressive treatment, have been reported so far. Within a median follow-up period of 12 months rituximab was well tolerated, which is compatible with the experience accumulated from its use in more than 500 000 lymphoma patients. About 80% of patients achieved marked and rapid reductions in global disease activity. Because of the clinical heterogeneity, dosing differences, and concomitant treatments, including cyclophosphamide in 35% of patients, a proper evaluation of the clinical efficacy or rituximab is difficult. Variable degrees of clinical benefit have been reported for all clinical systemic lupus erythematosus manifestations, including active proliferative nephritis. Whereas 4-weekly infusions of 375 mg/m(2) of rituximab result in complete B-cell depletion lasting most often from 3 to 8 months, a prolonged depletion does not always correlate with a more favorable clinical response. Total immunoglobulin levels and protective antibodies are preserved, but anti-dsDNA antibody titers decrease, often independently of the clinical response. Summary The findings reviewed point to a growing optimism for targeting B cells in the treatment of systemic lupus erythematosus; therefore double-blind studies comparing rituximab with existing immunosuppressive therapies are needed. Moreover, careful assessments of the effects of transient B-cell depletion on distinct autoimmune pathogenetic processes will enable optimization of therapeutic single or combined therapeutic schemes.
Published: 2005

46. Effect of infliximab on sight-threatening panuveitis in Behcet's disease

Author: Sfikakis, PP, Theodossiadis, PG, Katsiari, CG, Kaklamanis, P., and Markomichelakis, NN
Published: 2001

47. Intra- and intercellular variations in the repair efficiency of O-6-methylguanine, and their contribution to kinetic complexity

Author: Souliotis, VL Sfikakis, PP Anderson, LM Kyrtopoulos, SA
Abstract: Following administration to rats of various doses of N-nitrosodimethylamine (NDMA), O-6-methylguanine (O-6-meG) was lost from the DNA of four tissues (liver, white blood cells, lymph nodes, bone marrow) over two, sharply demarcated phases with substantially differing repair rates. Repair during each phase followed approximately first-order kinetics in O-6-meG, even after a high dose of NDMA which caused substantial depletion of O-6-alkylguanine-DNA alkyltransferase (AGT), a suicide repair protein. This is compatible with rate-determining adduct repair being brought about by a distinct, minor pool of AGT molecules which is rapidly replenished by de novo AGT synthesis. Similar biphasic repair kinetics were also observed in HepG2 cells treated in vitro with NDMA. In this case, the first phase of repair was inhibited by alpha-amanitin, an inhibitor of RNA polymerase II-mediated transcription. However, no dependence on transcriptional activity was found when O-6-meG repair in specific gene sequences with different transcriptional status in rat liver was examined, suggesting that the effects of a-amanitin in HepG2 cells did not reflect inhibition of preferential repair of transcribed sequences. Repair was also examined in rat liver hepatocytes and non-parenchymal cells separately after administration of NDMA at non-AGT depleting doses. Within each cell-population, the repair followed single phase, first-order kinetics, with adduct loss from AGT-rich hepatocytes being significantly faster than from the relatively AGT-deficient non-parenchymal cells. In conclusion, differences in the AGT content of different cell subpopulations in the liver (and probably in other tissues), as well as additional cellular factors affecting repair efficiency, appear to determine the observed variation in the kinetics of repair of O-6-meG. The additional cellular factors involved appear not to be related to the transcriptional state of the sequences being repaired, but may reflect different states of chromatin condensation. (C) 2004 Elsevier B.V. All rights reserved.
Published: 2004

48. Attenuation of inflammatory polyarthritis in TNF transgenic mice by diacerein: comparative analysis with dexamethasone, methotrexate and anti-TNF protocols

Author: Douni, E Sfikakis, PP Haralambous, S Fernandes, P and Kollias, G
Abstract: The impact of diacerein, an effective cartilage targeted therapy that is used in patients with osteoarthritis, on the development and progression of chronic inflammatory arthritis was evaluated in a tumor necrosis factor (TNF) transgenic mouse model (Tg197). The response to diacerein at 2, 20, or 60 mg/kg daily, as well as the comparative effects of other antiarthritis drugs including dexamethasone (0.5 mg/kg daily), methotrexate ( 1 mg/kg three times weekly) and an anti-TNF agent ( 5 mg/kg weekly), were assessed in the Tg197 mice. Treatment was initiated before the onset of arthritis and was continued for 5 weeks. A significant improvement in clinical symptoms was found in all three diacerein treated groups in comparison with untreated groups. Confirming these data, semiquantitative histopathologic analysis of the hind paws revealed a significant reduction not only in cartilage destruction but also in the extent of synovitis and bone erosion in diacerein treated groups in comparison with untreated groups. At the most effective dose tested ( 2 mg/kg daily), diacerein inhibited the onset of arthritis in 28% and attenuated the progression of arthritis in 35% of the Tg197 mice. Comparative analyses showed diacerein to be more potent than methotrexate but not as effective as dexamethasone or anti-TNF agents in suppressing the progression of the TNF mediated arthritis in this model. These results indicate that diacerein has a disease modifying effect on the onset and progression of TNF driven chronic inflammatory arthritis, suggesting that the prophylactic or therapeutic potential of diacerein in patients with RA should be further examined.
Published: 2004

49. Costimulation blockade in the treatment of rheumatic diseases

Author: Liossis, SNC Sfikakis, PP
Subjects: chemical and pharmacologic phenomena
Abstract: The autoimmune response is executed via cognate interactions between effector immune cells and antigen presenting cells. Cognate interactions provide the immune effectors with specific signals generated through the antigen receptor as well as with second, non-specific signals, generated from the interaction of pairs of cell-surface molecules (costimulatory molecules) present on their plasma membrane. Disruption of this second, non-specific costimulatory signal results in the interruption of the productive (auto)immune response, leading to anergy, a state of immune unresponsiveness. The CD28:137 families of molecules and the CD40:CD40L pair of molecules are considered as critical costimulatory elements. Disruption of the CD28:137 interaction using a genetically engineered soluble form of the inhibitory molecule CTLA4 in vitro, as well as in experimental models of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), led to the inhibition of the autoimmune response. Similarly, promising data stem from the use of an anti-CD40L monoclonal antibody (mAb) in murine SLE. While such treatments prevent the development of autoimmunity in animal models, this preventive approach is inapplicable to human diseases. However, the rational bench-to-bed side approach led investigators to clinical trials of CTLA4-Ig and of two different humanized anti-human CD40L mAbs in patients with RA and SLE, respectively. Initial experience with the use of CTLA4-Ig in patients with RA is encouraging, since in one short-term trial the construct was well-tolerated and produced clinically meaningful improvement of the disease in a significant proportion of those treated. Surprisingly, the anti-CD40L mAb treatment approach in human lupus was not fruitful, since short-term administration of the anti-CD40L mAb ruplizumab in lupus nephritis was correlated with life-threatening prothrombotic activity despite initial encouraging data in the serology and renal function of the patients. Also, IDEC-131 anti-CD40L mAb treatment did not prove to be clinically effective in human SLE, despite being well tolerated. Precise tailoring of the administration schemes for these novel therapeutic modalities is awaited. Finally, conceptually different approaches to block costimulation by inhibiting the induced expression of costimulatory molecules or the transmission of their specific intracytoplasmic signal have already produced encouraging preliminary results.
Published: 2004

50. Infliximab for chronic cystoid macular edema associated with uveitis

Author: Markomichelakis, NN Theodossiadis, PG Pantelia, E and Papaefthimiou, S Theodossiadis, GP Sfikakis, PP
Subjects: genetic structures, eye diseases
Abstract: PURPOSE: To assess the efficacy of the anti-TNF monoclonal antibody infliximab in uveitis patients without clinically evident ocular inflammation and impaired visual acuity because of chronic cystoid macular edema (CME). DESIGN: Prospective, noncomparative, interventional case series. METHODS: Patients with refractory CME (14 eyes, mean duration of 14 months), associated with intermediate uveitis (n = 6), Adamantiades-Behcet disease (n = 2), adult-type vascular pseudotumor (n = 1), and HLAB27+-related uveitis (n = 1) received an intravenous infliximab infusion (5 mg/kg); five patients were retreated after 1 month. RESULTS: Macular thickness, measured by ocular coherence tomography, was reduced from 428 +/- 138 mum to 219 +/- 51 mum at 2 months postbaseline (P = .0001), while visual acuity increased from 0.41 +/- 0.18 to 0.83 +/- 0.17 (P < .00001). Anatomic and functional improvement was sustained at 6 months in all. No ocular or extra-ocular side effects were noted. CONCLUSION: These promising results suggest that TNF may play an important pathogenetic role in chronic CME, thus, a controlled trial is warranted. (C) 2004 by Elsevier Inc. All rights reserved.
Published: 2004

Catalog

Books, media, physical & digital resources

See catalog results

Searchworks

Select search scope, currently: Articles Catalog books, media & more in Jio Institute collections Articles journal articles & other e-resources

Search

Search Constraints

Refine your results

Search Limiters

Topic

Publication Year Range

Language

Publication Type

Journal

Region

Database

Publisher

700 results on '"Sfikakis PP"'

Search Results

Catalog

Select search scope, currently: Articles

Catalog

books, media & more in Jio Institute collections

Articles

journal articles & other e-resources