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Data were retrieved from the AIDA Network Beh\u00e7et\u2019s 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\u00a0years (IQR 7.7). The median follow-up duration was 21.8\u00a0years (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 Beh\u00e7et's Syndrome Overall Damage Index was 0 (range 0\u20134). Colchicine was inefficacious for MSM in 4\/14 cases (28.6%), independently from the type of MSM (<jats:italic>p<\/jats:italic>\u2009=\u20090.46) or the concomitant therapy (<jats:italic>p<\/jats:italic>\u2009=\u20090.30 for cDMARDs, <jats:italic>p<\/jats:italic>\u2009=\u20091.00 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 (<jats:italic>p<\/jats:italic>\u2009=\u20090.014). To conclude,\u00a0MSM 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).<\/jats:p>","DOI":"10.1007\/s11739-023-03215-w","type":"journal-article","created":{"date-parts":[[2023,3,7]],"date-time":"2023-03-07T12:02:57Z","timestamp":1678190577000},"page":"743-754","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Musculoskeletal manifestations in children with Beh\u00e7et\u2019s syndrome: data from the AIDA Network Beh\u00e7et\u2019s Syndrome Registry"],"prefix":"10.1007","volume":"18","clinical-trial-number":[{"clinical-trial-number":"nct05200715","registry":"10.18810\/clinical-trials-gov"}],"author":[{"given":"Carla","family":"Gaggiano","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Maselli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petros 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