Browsing by Author "Di Cola, I"

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    The clinical assessment of lung involvement in patients with Still's disease, results from the multicentre international AIDA Network Still's Disease Registry
    Ruscitti, P; Vitale, A; Di Cola, I; Caggiano, V; Palumbo, P; Di Cesare, E; Hinojosa-Azaola, A; Torres-Ruiz, J; Guaracha-Basañez, GA; Martín-Nares, E; Lopalco, G; Morrone, M; Iannone, F; Giardini, HAM; Cordeiro, RA; Antonelli, IPD; Berardicurti, O; Navarini, L; Ciccia, F; Visconti, MC; Iacono, D; Direskeneli, H; Erten, S; Yao, HH; Thabet, M; Tharwat, S; Ragab, G; Gómez-Caverzaschi, V; Sfikakis, PP; Fotis, L; La Torre, F; Maier, A; Karamanakos, A; Almaghlouth, IA; Frassi, M; Tufan, A; Govoni, M; Sota, J; Simonini, G; Emmi, G; Li Gobbi, F; Parronchi, P; Costi, S; Sarzi-Puttini, P; Opris-Belinski, D; Sfriso, P; Tarsia, M; Maggio, MC; Monti, S; Gündüz, OS; Rigante, D; Bartoloni, E; Verrecchia, E; Iagnocco, A; Viapiana, O; Bargagli, E; Batu, ED; Sebastiani, GD; Del Giudice, E; Conti, G; Breda, L; Gidaro, A; Gicchino, MF; Gaggiano, C; Brucato, AL; Triggianese, P; Makowska, J; Carubbi, F; Farina, N; Guggino, G; De Paulis, A; Mazzei, MA; Di Meglio, N; Lo Gullo, A; Conforti, A; Ogunjimi, B; Calabrese, L; Rubegni, P; Giardina, A; Wiesik-Szewczyk, E; Balistreri, A; Fabiani, C; Frediani, B; Dagna, L; Giacomelli, R; Cantarini, L
    Objectives To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.Methods A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry. Still's disease-lung involvement was defined by the presence of pleuritis, parenchymal features, acute respiratory distress syndrome (ARDS) and/or pulmonary arterial hypertension.Results In total, 90 patients with Still's disease and lung involvement were assessed (mean age 36.3 +/- 17.8 years, 35.6% male sex). Among them, 13.3% of patients were paediatrics. These patients with lung involvement mainly showed pleuritis in 72.2% of cases, parenchymal features in 34.4%, ARDS in 9.5% and pulmonary arterial hypertension in 2.3%. After that we focused on patients characterised by parenchymal lung involvement, which is an emergent issue of clinical concern. These patients with parenchymal lung disease were significantly characterized by sore throat, pericarditis and higher values of systemic score than others. Finally, the administration of both IL-1 or IL-6 inhibitors was not associated with the presence of parenchymal lung involvement.Conclusion The clinical characteristics of patients with Still's disease and lung involvement were described in the AIDA network. We also provided a clinical profile of patients with parenchymal lung involvement considering its prognostic relevance. Although providing a clinical landscape of these patients, further studies are needed to fully clarify this issue.
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    Development and Implementation of the AIDA International Registry for Patients With VEXAS Syndrome
    Vitale, A; Caggiano, V; Della Casa, F; Hernandez-Rodriguez, 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, 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, GD; Insalaco, A; Shahram, F; Sfriso, P; Minoia, F; Alessio, M; Makowska, J; Hatemi, G; Akkoc, N; Li Gobbi, F; Gidaro, A; Olivieri, AN; Al-Mayouf, SM; Erten, S; Gentileschi, S; Vasi, I; Tarsia, M; Mahmoud, AAMA; Frediani, B; Alzahrani, MF; Laymouna, AH; Ricci, F; Cardinale, F; Jahnz-Rozyk, 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, 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 22(nd), 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.
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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.