• Carla Gaggiano, Anna Maselli, Petros P Sfikakis, Katerina Laskari, Gaafar Ragab, Mohamed Tharwat Hegazy, Ahmed Hatem Laymouna, Giuseppe Lopalco, Ibrahim A Almaghlouth, Kazi Nur Asfina, Ohoud Alahmed, Henrique Ayres Giardini Mayrink, Isabele Parente de Brito Antonelli, Marco Cattalini, Matteo Piga, Jurgen Sota, Stefano Gentileschi, Maria Cristina Maggio, Daniela Opris-Belinski, Gülen Hatemi, Antonella Insalaco, Alma Nunzia Olivieri, Abdurrahman Tufan, Hazan Karadeniz, Riza Can Kardaş, Francesco La Torre, Fabio Cardinale, Achille Marino, Silvana Guerriero, Piero Ruscitti, Maria Tarsia, Antonio Vitale, Valeria Caggiano, Salvatore Telesca, Florenzo Iannone, Veronica Parretti, Micol Frassi, Emma Aragona, Francesco Ciccia, Ewa Wiesik-Szewczyk, Ruxandra Ionescu, Ali Şahin, Nurullah Akkoç, Andrea Hinojosa-Azaola, Samar Tharwat, José Hernández-Rodríguez, Gerard Espinosa, Giovanni Conti, Emanuela Del Giudice, Marcello Govoni, Giacomo Emmi, Claudia Fabiani, Alberto Balistreri, Bruno Frediani, Donato Rigante, Luca Cantarini, and AIDA Network.
• Department of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
• Intern Emerg Med. 2023 Apr 1; 18 (3): 743754743-754.

AbstractThis 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).

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