Browsing by Author "Di Cola I."

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    Development and Implementation of the AIDA International Registry for Patients With VEXAS Syndrome
    (Frontiers Media S.A., 2022) 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 R.M.R.; Yildirim D.; Cattalini M.; Marino A.; Giani T.; La Torre F.; Ruscitti P.; Aragona E.; Wiesik-Szewczyk E.; Del Giudice E.; Sfikakis P.P.; Govoni M.; Emmi G.; Maggio M.C.; Giacomelli R.; Ciccia F.; Conti G.; 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 G.D.; Insalaco A.; Shahram F.; Sfriso P.; Minoia F.; Alessio M.; Makowska J.; Hatemi G.; Akkoç N.; Li Gobbi F.; Gidaro A.; Olivieri A.N.; Al-Mayouf S.M.; Erten S.; Gentileschi S.; Vasi I.; Tarsia M.; Mahmoud A.A.-M.A.; Frediani B.; Fares Alzahrani M.; Laymouna A.H.; Ricci F.; Cardinale F.; Jahnz-Rózyk K.; Tosi G.M.; Crisafulli F.; Balistreri A.; Dagostin M.A.; Ghanema M.; Gaggiano C.; Sota J.; Di Cola I.; Fabiani C.; Giardini H.A.M.; Renieri A.; Fabbiani A.; Carrer A.; Bocchia M.; Caroni F.; Rigante D.; Cantarini L.
    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: This 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, thus enhancing international collaboration and data sharing for research purposes. The Registry is practical enough to be easily modified to meet future needs regarding VEXAS syndrome. Results: To date (April 22nd, 2022), 113 Centers from 23 Countries in 4 continents have been involved; 324 users (114 Principal Investigators, 205 Site Investigators, 2 Lead Investigators, and 3 data managers) are currently able to access the registry for data entry (or data sharing) and collection. The Registry includes 4,952 fields organized 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. Conclusion: This international Registry for patients with VEXAS syndrome will allow the achievement of a comprehensive knowledge about this new disease, with the final goal to obtain real-world evidence for daily clinical practice, especially in relation to the comprehension of this disease about the natural history and the possible therapeutic approaches. This Project can be found on https://clinicaltrials.gov NCT05200715. Copyright © 2022 Vitale, Caggiano, Della Casa, Hernández-Rodríguez, Frassi, Monti, Tufan, Telesca, Conticini, Ragab, Lopalco, Almaghlouth, Pereira, Yildirim, Cattalini, Marino, Giani, La Torre, Ruscitti, Aragona, Wiesik-Szewczyk, Del Giudice, Sfikakis, Govoni, Emmi, Maggio, Giacomelli, Ciccia, Conti, Ait-Idir, Lomater, Sabato, Piga, Sahin, Opris-Belinski, Ionescu, Bartoloni, Franceschini, Parronchi, de Paulis, Espinosa, Maier, Sebastiani, Insalaco, Shahram, Sfriso, Minoia, Alessio, Makowska, Hatemi, Akkoç, Li Gobbi, Gidaro, Olivieri, Al-Mayouf, Erten, Gentileschi, Vasi, Tarsia, Mahmoud, Frediani, Fares Alzahrani, Laymouna, Ricci, Cardinale, Jahnz-Rózyk, Tosi, Crisafulli, Balistreri, Dagostin, Ghanema, Gaggiano, Sota, Di Cola, Fabiani, Giardini, Renieri, Fabbiani, Carrer, Bocchia, Caroni, Rigante and Cantarini.
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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
    (John Wiley and Sons Inc, 2024) Ruscitti P.; Masedu F.; Vitale A.; Caggiano V.; Di Cola I.; Cipriani P.; Valenti M.; Mayrink Giardini H.A.; de Brito Antonelli I.P.; Dagostin M.A.; Lopalco G.; Iannone F.; Maria M.; Almaghlouth I.A.; Asfina K.N.; Ali H.H.; Ciccia F.; Iacono D.; Pantano I.; Mauro D.; Sfikakis P.P.; Tektonidou M.; Laskari K.; Berardicurti O.; Dagna L.; Tomelleri A.; Tufan A.; Can Kardas R.; Hinojosa-Azaola A.; Martín-Nares E.; Kawakami-Campos P.A.; Ragab G.; Hegazy M.T.; Direskeneli H.; Alibaz-Oner F.; Fotis L.; Sfriso P.; Govoni M.; La Torre F.; Cristina Maggio M.; Montecucco C.; De Stefano L.; Bugatti S.; Rossi S.; Makowska J.; Del Giudice E.; Emmi G.; Bartoloni E.; Hernández-Rodríguez J.; Conti G.; Nunzia Olivieri A.; 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 G.D.; Gidaro A.; Brucato A.L.; 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. (Figure presented.). © 2024 American College of Rheumatology.
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    Evaluation of Myocarditis in Patients With Still Disease: Clinical Findings From the Multicenter International AIDA Network Still Disease Registry
    (Journal of Rheumatology, 2025) Ruscitti P.; Di Cola I.; Vitale A.; Caggiano V.; Palumbo P.; Di Cesare E.; Torres-Ruiz J.; Guaracha-Basañez G.A.; Martín-Nares E.; Ciccia F.; Iacono D.; Riccio F.; Maggio M.C.; Tharwat S.; Hashad S.; Rigante D.; Ortolan A.; Mayrink Giardini H.A.; de Brito Antonelli I.P.; Cordeiro R.A.; Giacomelli R.; Navarini L.; Berardicurti O.; Conforti A.; Opris-Belinski D.; Sota J.; Gaggiano C.; Lopalco G.; Iannone F.; La Torre F.; Mastrorilli V.; Govoni M.; Ruffilli F.; Emmi G.; Biancalana E.; Sfikakis P.P.; Tektonidou M.; Hernández-Rodríguez J.; Gómez-Caverzaschi V.; Gündüz Ö.S.; Conti G.; Patroniti S.; Gidaro A.; Bartoli A.; Olivieri A.N.; Gicchino M.F.; Brucato A.L.; Dagna L.; Tomelleri A.; Campochiaro C.; De Paulis A.; Mormile I.; Casa F.D.; Direskeneli H.; Alibaz-Oner F.; Karamanakos A.; Dimouli A.; Ragab G.; Ahmed Mahmoud A.A.; Tufan A.; Kucuk H.; Kardas R.; Batu E.D.; Ozen S.; Wiesik-Szewczyk E.; Hinojosa-Azaola A.; Balistreri A.; Fabiani C.; Frediani B.; Cantarini L.
    Objective. We aimed to (1) evaluate the cardiac involvement, with a focus on myocarditis, in patients with Still disease included in the multicenter Autoinflammatory Disease Alliance (AIDA) Network Still disease registry; and (2) assess the predictive factors for myocarditis by deriving a clinical risk patient profile for this severe manifestation. Methods. A multicenter observational study was established, in which consecutive patients with Still disease in the AIDA Network Still disease registry were characterized by cardiac involvement. Cardiac involvement was defined according to the presence of pericarditis, tamponade, myocarditis, and/or aseptic endocarditis. Results. In total, 73 patients with Still disease and cardiac involvement were assessed (mean age 36.3 [SD 19.9] years; male sex, 42.5%), out of which 21.9% were children. The most common cardiac manifestation was pericarditis, occurring in 90.4% of patients; patients also presented with myocarditis (26%), and less frequently endocarditis (2.7%) and tamponade (1.4%). In comparing clinical features of patients with myocarditis to those without, significantly increased frequencies of skin rash and pleuritis, as well as higher systemic scores, were seen. Further, a higher mortality rate was shown in patients with myocarditis. In regression models, skin rash and the systemic score independently predicted the myocarditis. Conclusion. The characteristics of patients with Still disease and cardiac involvement were assessed in the AIDA Network. The most common feature was the pericarditis, but a more severe clinical picture was also reported in patients with myocarditis. The latter was associated with increased mortality rate and higher systemic score, identifying patients who should be carefully managed. © 2025 The Journal of Rheumatology.