Browsing by Author "Maselli, A"

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    Musculoskeletal manifestations in children with Behcet's syndrome: data from the AIDA Network Behcet's Syndrome Registry
    Gaggiano, C; Maselli, A; Sfikakis, PP; Laskari, K; Ragab, G; Hegazy, MT; Laymouna, AH; Lopalco, G; Almaghlouth, IA; Asfina, KN; Alahmed, O; Mayrink, HAG; Antonelli, IPD; Cattalini, M; Piga, M; Sota, J; Gentileschi, S; Maggio, MC; Opris-Belinski, D; Hatemi, G; Insalaco, A; Olivieri, AN; Tufan, A; Karadeniz, H; Kardas, RC; 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; Sahin, 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 Behcet's syndrome (BS), their association with other disease manifestations, response to therapy, and long-term prognosis. Data were retrieved from the AIDA Network Behcet'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 Behcet'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).