Musculoskeletal manifestations in children with Behçet's syndrome: data from the AIDA Network Behçet's Syndrome Registry.
Клиническое исследованиеСтатьяНазвание и аннотация: Перевод в очереди
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Gaggiano Carla, Maselli Anna, Sfikakis Petros P, Laskari Katerina, Ragab Gaafar, Hegazy Mohamed Tharwat, Laymouna Ahmed Hatem, Lopalco Giuseppe, Almaghlouth Ibrahim A, Asfina Kazi Nur, Alahmed Ohoud, Giardini Mayrink Henrique Ayres, Parente de Brito Antonelli Isabele, Cattalini Marco, Piga Matteo, Sota Jurgen, Gentileschi Stefano, Maggio Maria Cristina, Opris-Belinski Daniela, Hatemi Gülen, Insalaco Antonella, Olivieri Alma Nunzia, Tufan Abdurrahman, Karadeniz Hazan, Kardaş Riza Can, La Torre Francesco, Cardinale Fabio, Marino Achille, Guerriero Silvana, Ruscitti Piero, Tarsia Maria, Vitale Antonio, Caggiano Valeria, Telesca Salvatore, Iannone Florenzo, Parretti Veronica, Frassi Micol, Aragona Emma, Ciccia Francesco, Wiesik-Szewczyk Ewa, Ionescu Ruxandra, Şahin Ali, Akkoç Nurullah, Hinojosa-Azaola Andrea, Tharwat Samar, Hernández-Rodríguez José, Espinosa Gerard, Conti Giovanni, Del Giudice Emanuela, Govoni Marcello, Emmi Giacomo, Fabiani Claudia, Balistreri Alberto, Frediani Bruno, Rigante Donato, Cantarini Luca, AIDA Network
Аннотация
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).