Browsing by Author "Giardini, HAM"

Now showing 1 - 6 of 6
  • No Thumbnail Available
    Item
    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.
  • No Thumbnail Available
    Item
    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.
  • No Thumbnail Available
    Item
    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.
  • No Thumbnail Available
    Item
    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.
  • No Thumbnail Available
    Item
    A patient-driven registry on Behcet's disease: the AIDA for patients pilot project
    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, D; 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; Wiesik-Szewczyk, E; Akkoç, N; Tufan, A; Sahin, A; Erten, S; Ozen, S; Batu, ED; Frediani, B; Balistreri, A; Cantarini, L
    IntroductionThis 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 Behcet's disease (BD). MethodsThe project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behcet), 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. ResultsRespondents 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 Behcet'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 & PLUSMN; 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). DiscussionPreliminary results from the AIDA for Patients BD registry were consistent with data available in the literature, confirming that PROs and PREs could be easily provided by the patient remotely to integrate physician-driven registries with complementary and reliable information.
  • No Thumbnail Available
    Item
    Influence of gender on Behcet's disease phenotype and irreversible organ damage: Data from the International AIDA Network Behcet's Disease Registry
    Sota, J; Ragab, G; AlMaglouth, I; Lopalco, G; Tufan, A; Direskeneli, H; Hinojosa-Azaola, A; Giardini, HAM; Guerriero, S; Triggianese, P; Sfikakis, PP; Piga, M; Ruscitti, P; Govoni, M; Iagnocco, A; Carubbi, F; Hernández-Rodríguez, J; Laymouna, AH; Mahmoud, AAMA; Ghanema, M; Aboabat, AA; Asfina, KN; Alanazi, F; Morrone, M; Spedicato, V; Kucuk, H; Kardas, R; Öner, FA; Sevik, G; Torres-Ruiz, J; Kawakami-Campos, PA; Antonelli, IPDB; Dammacco, R; Chimenti, MS; Arida, K; Floris, A; Gentile, M; Ruffilli, F; Bellis, E; Alunno, A; Espinosa, G; Gentileschi, S; Gaggiano, C; Vitale, A; Caggiano, V; Lopez, R; Tarsia, M; Montis, S; Hatemi, G; Karakoc, A; Frassi, M; Giacomelli, R; Tharwat, S; Thabet, M; Ciccia, F; Emmi, G; Viapiana, O; Sahin, A; Sebastiani, GD; Batu, ED; Ozen, S; Sener, S; Opris-Belinski, D; Costi, S; Conforti, A; Cattalini, M; Bartoloni, E; Akkoç, N; Gunduz, OS; Conti, G; Maier, A; Giardina, A; Li Gobbi, F; Parronchi, P; Puttini, PS; Breda, L; De Paulis, A; Carreño, E; La Torre, F; Wiesik-Scewczyk, E; de-la Torre, A; Mejía-Salgado, G; Shahram, F; Guiducci, S; Maggio, MC; Aragona, E; Rigante, D; Ciavarro, A; Önen, F; Erten, U; Insalaco, A; Del Giudice, E; Barone, P; Gicchino, F; Brucato, A; Lo Gullo, A; Mauro, A; Karamanakos, A; Balistreri, A; Mazzei, MA; Frediani, B; Fabiani, C; Cantarini, L
    Objectives. - Gender impact on phenotypical expression of Behcet's disease (BD) has been specifically investigated only in a few large-scale studies. The main goal of the study was to examine gender differences in a large cohort of patients affected by BD. Methods. - Data were retrieved from the International AIDA Network Registry for BD. We assessed differences between males and females in terms of Behcet's syndrome Overall Damage Index (BODI), differences in the disease manifestations at onset and in the cumulative manifestations throughout diseasecourse, as well as differences in the cardiovascular risk. Finally, predictive factors leading to major organ involvement were investigated. Results. - In total, 1024 BD patients (567 males, 457 females) were enrolled in the study, with a male-to-female ratio of 1.24/1. Males displayed a significantly higher mean +/- SD BODI (1.92 +/- 2.09) at the last follow-up, compared to female patients (1.25 +/- 1.87) (P < 0.0001). Uveitis (P < 0.0001) and vascular involvement (P = 0.0076) were significantly more frequent among males whereas female patients were significantly over-represented in arthralgia (P < 0.0001), arthritis (P = 0.00025), isolated headache (P < 0.0001), central nervous system (CNS) involvement (P = 0.040), and gastrointestinal involvement (P = 0.00046). Regarding cardiovascular risk, no differences between the two groups emerged (P = 0.617). Four variables were associated with the development of major organ involvement: male gender (OR = 2.104, P = 0.001), current treatment with biologic agents (OR = 2.257, P = 0.0003), origin from endemic countries (OR = 2.661, P = 0.0009), and disease duration (OR = 1.002, P = 0.024). Conclusion. - BD displays a more severe course among males. This subgroup develops more irreversible damage and presents more frequently ocular and vascular involvement during disease course. On the other hand, female patients are prone to experience articular involvement, headache, CNS and gastrointestinal involvement. These data suggest the existence of a gender-driven disease expression. (c) 2024 Les Auteurs. Publie par Elsevier Masson SAS au nom de Socioto Franoaise de Rhumatologie. Cet article est publie en Open Access sous licence CC BY (http://creativecommons.org/licenses/by/4.0/).