Browsing by Author "Almaghlouth I.A."

Now showing 1 - 3 of 3
  • No Thumbnail Available
    Item
    Development and implementation of the AIDA International Registry for patients with Behçet’s disease
    (Springer Science and Business Media Deutschland GmbH, 2022) Vitale A.; Della Casa F.; Ragab G.; Almaghlouth I.A.; Lopalco G.; Pereira R.M.; Guerriero S.; Govoni M.; Sfikakis P.P.; 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 G.D.; Bartoloni E.; Akkoç N.; Gündüz Ö.S.; Cattalini M.; Conti G.; Hatemi G.; Maier A.; Parronchi P.; Del Giudice E.; Erten S.; Insalaco A.; Li Gobbi F.; Maggio M.C.; Shahram F.; Caggiano V.; Hegazy M.T.; Asfina K.N.; Morrone M.; Prado L.L.; Dammacco R.; Ruffilli F.; Arida A.; Navarini L.; Pantano I.; Cavagna L.; Conforti A.; Cauli A.; Marucco E.M.; Kucuk H.; Ionescu R.; Mattioli I.; Espinosa G.; Araújo O.; Karkaş B.; Canofari C.; Sota J.; Laymouna A.H.; Bedaiwi A.A.; Colella S.; Giardini H.A.M.; Albano V.; Lo Monaco A.; Fragoulis G.E.; Kardas R.C.; Berlengiero V.; Hussein M.A.; Ricci F.; La Torre F.; Rigante D.; Więsik-Szewczyk E.; Frassi M.; Gentileschi S.; Tosi G.M.; Dagostin M.A.; Mahmoud A.A.-M.A.; 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 Behçet’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. © 2022, The Author(s).
  • No Thumbnail Available
    Item
    Musculoskeletal manifestations in children with Behçet’s syndrome: data from the AIDA Network Behçet’s Syndrome Registry
    (Springer Science and Business Media Deutschland GmbH, 2023) Gaggiano C.; Maselli A.; Sfikakis P.P.; Laskari K.; Ragab G.; Hegazy M.T.; Laymouna A.H.; Lopalco G.; Almaghlouth I.A.; Asfina K.N.; Alahmed O.; Giardini Mayrink H.A.; Parente de Brito Antonelli I.; Cattalini M.; Piga M.; Sota J.; Gentileschi S.; Maggio M.C.; Opris-Belinski D.; Hatemi G.; Insalaco A.; Olivieri A.N.; Tufan A.; Karadeniz H.; Kardaş R.C.; 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 D.; Cantarini L.
    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 inefficacious 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 inefficacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefficacy (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 affects response to biologic therapies. ClinicalTrials.gov Identifier: NCT05200715 (registered on December 18, 2021). © 2023, The Author(s).
  • 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
    (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.