Musculoskeletal manifestations in children with Behçet’s syndrome: data from the AIDA Network Behçet’s Syndrome Registry

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dc.contributor.authorGaggiano C.
dc.contributor.authorMaselli A.
dc.contributor.authorSfikakis P.P.
dc.contributor.authorLaskari K.
dc.contributor.authorRagab G.
dc.contributor.authorHegazy M.T.
dc.contributor.authorLaymouna A.H.
dc.contributor.authorLopalco G.
dc.contributor.authorAlmaghlouth I.A.
dc.contributor.authorAsfina K.N.
dc.contributor.authorAlahmed O.
dc.contributor.authorGiardini Mayrink H.A.
dc.contributor.authorParente de Brito Antonelli I.
dc.contributor.authorCattalini M.
dc.contributor.authorPiga M.
dc.contributor.authorSota J.
dc.contributor.authorGentileschi S.
dc.contributor.authorMaggio M.C.
dc.contributor.authorOpris-Belinski D.
dc.contributor.authorHatemi G.
dc.contributor.authorInsalaco A.
dc.contributor.authorOlivieri A.N.
dc.contributor.authorTufan A.
dc.contributor.authorKaradeniz H.
dc.contributor.authorKardaş R.C.
dc.contributor.authorLa Torre F.
dc.contributor.authorCardinale F.
dc.contributor.authorMarino A.
dc.contributor.authorGuerriero S.
dc.contributor.authorRuscitti P.
dc.contributor.authorTarsia M.
dc.contributor.authorVitale A.
dc.contributor.authorCaggiano V.
dc.contributor.authorTelesca S.
dc.contributor.authorIannone F.
dc.contributor.authorParretti V.
dc.contributor.authorFrassi M.
dc.contributor.authorAragona E.
dc.contributor.authorCiccia F.
dc.contributor.authorWiesik-Szewczyk E.
dc.contributor.authorIonescu R.
dc.contributor.authorŞahin A.
dc.contributor.authorAkkoç N.
dc.contributor.authorHinojosa-Azaola A.
dc.contributor.authorTharwat S.
dc.contributor.authorHernández-Rodríguez J.
dc.contributor.authorEspinosa G.
dc.contributor.authorConti G.
dc.contributor.authorDel Giudice E.
dc.contributor.authorGovoni M.
dc.contributor.authorEmmi G.
dc.contributor.authorFabiani C.
dc.contributor.authorBalistreri A.
dc.contributor.authorFrediani B.
dc.contributor.authorRigante D.
dc.contributor.authorCantarini L.
dc.date.accessioned2024-07-22T08:02:54Z
dc.date.available2024-07-22T08:02:54Z
dc.date.issued2023
dc.description.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).
dc.identifier.DOI-ID10.1007/s11739-023-03215-w
dc.identifier.issn18280447
dc.identifier.urihttp://akademikarsiv.cbu.edu.tr:4000/handle/123456789/12048
dc.language.isoEnglish
dc.publisherSpringer Science and Business Media Deutschland GmbH
dc.rightsAll Open Access; Hybrid Gold Open Access
dc.subjectArthritis
dc.subjectBehcet Syndrome
dc.subjectChild
dc.subjectHumans
dc.subjectMyalgia
dc.subjectRegistries
dc.subjectUlcer
dc.subjectarthritis
dc.subjectBehcet disease
dc.subjectchild
dc.subjectcomplication
dc.subjecthuman
dc.subjectmyalgia
dc.subjectregister
dc.subjectulcer
dc.titleMusculoskeletal manifestations in children with Behçet’s syndrome: data from the AIDA Network Behçet’s Syndrome Registry
dc.typeArticle

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