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

Gaggiano C.;Maselli A.;Sfikakis P. P.;Laskari K.;Ragab G.;Hegazy M. T.;Laymouna A. H.;Lopalco G.;Almaghlouth I. A.;Asfina K. N.;Alahmed O.;Giardini Mayrink H. A.;Parente de Brito Antonelli I.;Cattalini M.;Piga M.;Sota J.;Gentileschi S.;Maggio M. C.;Opris-Belinski D.;Hatemi G.;Insalaco A.;Olivieri A. N.;Tufan A.;Karadeniz H.;Kardas R. C.;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.;Akkoc N.;Hinojosa-Azaola A.;Tharwat S.;Hernandez-Rodriguez J.;Espinosa G.;Conti G.;Del Giudice E.;Govoni M.;Emmi G.;Fabiani C.;Balistreri A.;Frediani B.;Rigante D.;Cantarini L.

2023-01-01

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Abstract

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).