Browsing by Author "Cipriani, P"

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    The Systemic Score May Identify Life-Threatening Evolution in Still Disease: Data from the GIRRCS AOSD-Study Group and the AIDA Network Still Disease Registry
    Ruscitti, P; Masedu, F; Vitale, A; Caggiano, V; Di Cola, I; Cipriani, P; Valenti, M; Giardini, HAM; Antonelli, IPDB; Dagostin, MA; Lopalco, G; Iannone, F; Maria, M; Almaghlouth, IA; Asfina, KN; Ali, HH; Ciccia, F; Iacono, D; Pantano, I; Mauro, D; Sfikakis, PP; Tektonidou, M; Laskari, K; Berardicurti, O; Dagna, L; Tomelleri, A; Tufan, A; Kardas, RC; Hinojosa-Azaola, A; Martín-Nares, E; Kawakami-Campos, PA; Ragab, G; Hegazy, MT; Direskeneli, H; Alibaz-Oner, F; Fotis, L; Sfriso, P; Govoni, M; La Torre, F; Maggio, MC; Montecucco, C; De Stefano, L; Bugatti, S; Rossi, S; Makowska, J; Del Giudice, E; Emmi, G; Bartoloni, E; Hernández-Rodríguez, J; Conti, G; Olivieri, AN; Lo Gullo, A; Simonini, G; Viapiana, O; Wiesik-Szewczyk, E; Erten, S; Carubbi, F; De Paulis, A; Maier, A; Tharwat, S; Costi, S; Iagnocco, A; Sebastiani, GD; Gidaro, A; Brucato, AL; Karamanakos, A; Akkoç, N; Caso, F; Costa, L; Prete, M; Perosa, F; Atzeni, F; Guggino, G; Fabiani, C; Frediani, B; Giacomelli, R; Cantarini, L
    Objective. We aimed to evaluate the clinical usefulness of the systemic score in the prediction of life-threatening evolution in Still disease. We also aimed to assess the clinical relevance of each component of the systemic score in predicting life-threatening evolution and to derive patient subsets accordingly. Methods. A multicenter, observational, prospective study was designed including patients included in the Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale Adult-Onset Still Disease Study Group and the Autoinflammatory Disease Alliance Network Still Disease Registry. Patients were assessed to see if the variables to derive the systemic score were available. The life-threatening evolution was defined as mortality, whatever the clinical course, and/or macrophage activation syndrome, a secondary hemophagocytic lymphohistiocytosis associated with a poor prognosis. Results. A total of 597 patients with Still disease were assessed (mean +/- SD age 36.6 +/- 17.3 years; male 44.4%). The systemic score, assessed as a continuous variable, significantly predicted the life-threatening evolution (odds ratio [OR] 1.24; 95% confidence interval [CI] 1.07-1.42; P = 0.004). A systemic score >= 7 also significantly predicted the likelihood of a patient experiencing life-threatening evolution (OR 3.36; 95% CI 1.81-6.25; P < 0.001). Assessing the clinical relevance of each component of the systemic score, liver involvement (OR 1.68; 95% CI 1.48-2.67; P = 0.031) and lung disease (OR 2.12; 95% CI 1.14-4.49; P = 0.042) both significantly predicted life-threatening evolution. The clinical characteristics of patients with liver involvement and lung disease were derived, highlighting their relevance in multiorgan disease manifestations. Conclusion. The clinical utility of the systemic score was shown in identifying Still disease at a higher risk of life-threatening evolution in a large cohort. Furthermore, the clinical relevance of liver involvement and lung disease was highlighted.
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    Development and implementation of the AIDA International Registry for patients with Behcet's disease
    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; Karkas, B; Sebastiani, GD; Bartoloni, E; Akkoç, N; Cattalini, M; Conti, G; 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; 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, D; Wiesik-Szewczyk, E; Frassi, M; Gentileschi, S; Tosi, GM; Dagostin, MA; Mahmoud, AAMA; 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
    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 Behcet's disease (BD). The Registry is a clinical physician-driven non-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. Starting from January 31st, 2021, to February 7th, 2022, 110 centres 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 5993 fields organised into 16 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. 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. Trial registration NCT05200715 in 21/01/2022.