6533b850fe1ef96bd12a83be
RESEARCH PRODUCT
Musculoskeletal manifestations in children with Behçet's syndrome: data from the AIDA Network Behçet's Syndrome Registry
Carla GaggianoAnna MaselliPetros P SfikakisKaterina LaskariGaafar RagabMohamed Tharwat HegazyAhmed Hatem LaymounaGiuseppe LopalcoIbrahim A AlmaghlouthKazi Nur AsfinaOhoud AlahmedHenrique Ayres Giardini MayrinkIsabele Parente De Brito AntonelliMarco CattaliniMatteo PigaJurgen SotaStefano GentileschiMaria Cristina MaggioDaniela Opris-belinskiGülen HatemiAntonella InsalacoAlma Nunzia OlivieriAbdurrahman TufanHazan KaradenizRiza Can KardaşFrancesco La TorreFabio CardinaleAchille MarinoSilvana GuerrieroPiero RuscittiMaria TarsiaAntonio VitaleValeria CaggianoSalvatore TelescaFlorenzo IannoneVeronica ParrettiMicol FrassiEmma AragonaFrancesco CicciaEwa Wiesik-szewczykRuxandra IonescuAli ŞAhinNurullah AkkoçAndrea Hinojosa-azaolaSamar TharwatJosé Hernández-rodríguezGerard EspinosaGiovanni ContiEmanuela Del GiudiceMarcello GovoniGiacomo EmmiClaudia FabianiAlberto BalistreriBruno FredianiDonato RiganteLuca Cantarinisubject
Settore MED/16 - REUMATOLOGIABehçet's diseaseBehçet’s syndromeArthritisArthritis; Behçet’s syndrome; International registry; Pediatric rheumatology; Rare diseasesRare diseasesSettore MED/38 - Pediatria Generale E SpecialisticaArthritis Behçet’s syndrome International registry Pediatric rheumatology Rare diseasesInternational registryEmergency MedicineInternal MedicineAutoinflammationPediatric rheumatologyArthritidescription
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).
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2023-03-07 |