: 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).

Musculoskeletal manifestations in children with Behçet's syndrome: data from the AIDA Network Behçet's Syndrome Registry / Gaggiano, Carla; Maselli, Anna; Sfikakis, Petros P; Laskari, Katerina; Ragab, Gaafar; Hegazy, Mohamed Tharwat; Laymouna, Ahmed Hatem; Lopalco, Giuseppe; Almaghlouth, Ibrahim A; Asfina, Kazi Nur; Alahmed, Ohoud; Giardini Mayrink, Henrique Ayres; Parente de Brito Antonelli, Isabele; Cattalini, Marco; Piga, Matteo; Sota, Jurgen; Gentileschi, Stefano; Maggio, Maria Cristina; Opris-Belinski, Daniela; Hatemi, Gülen; Insalaco, Antonella; Olivieri, Alma Nunzia; Tufan, Abdurrahman; Karadeniz, Hazan; Kardaş, Riza Can; La Torre, Francesco; Cardinale, Fabio; Marino, Achille; Guerriero, Silvana; Ruscitti, Piero; Tarsia, Maria; Vitale, Antonio; Caggiano, Valeria; Telesca, Salvatore; Iannone, Florenzo; Parretti, Veronica; Frassi, Micol; Aragona, Emma; Ciccia, Francesco; Wiesik-Szewczyk, Ewa; Ionescu, Ruxandra; Şahin, Ali; Akkoç, Nurullah; Hinojosa-Azaola, Andrea; Tharwat, Samar; Hernández-Rodríguez, José; Espinosa, Gerard; Conti, Giovanni; Del Giudice, Emanuela; Govoni, Marcello; Emmi, Giacomo; Fabiani, Claudia; Balistreri, Alberto; Frediani, Bruno; Rigante, Donato; Cantarini, Luca. - In: INTERNAL AND EMERGENCY MEDICINE. - ISSN 1970-9366. - ELETTRONICO. - 18:(2023), pp. 743-754. [10.1007/s11739-023-03215-w]

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

Emmi, Giacomo;
2023

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).
2023
18
743
754
Gaggiano, Carla; Maselli, Anna; Sfikakis, Petros P; Laskari, Katerina; Ragab, Gaafar; Hegazy, Mohamed Tharwat; Laymouna, Ahmed Hatem; Lopalco, Giuseppe; Almaghlouth, Ibrahim A; Asfina, Kazi Nur; Alahmed, Ohoud; Giardini Mayrink, Henrique Ayres; Parente de Brito Antonelli, Isabele; Cattalini, Marco; Piga, Matteo; Sota, Jurgen; Gentileschi, Stefano; Maggio, Maria Cristina; Opris-Belinski, Daniela; Hatemi, Gülen; Insalaco, Antonella; Olivieri, Alma Nunzia; Tufan, Abdurrahman; Karadeniz, Hazan; Kardaş, Riza Can; La Torre, Francesco; Cardinale, Fabio; Marino, Achille; Guerriero, Silvana; Ruscitti, Piero; Tarsia, Maria; Vitale, Antonio; Caggiano, Valeria; Telesca, Salvatore; Iannone, Florenzo; Parretti, Veronica; Frassi, Micol; Aragona, Emma; Ciccia, Francesco; Wiesik-Szewczyk, Ewa; Ionescu, Ruxandra; Şahin, Ali; Akkoç, Nurullah; Hinojosa-Azaola, Andrea; Tharwat, Samar; Hernández-Rodríguez, José; Espinosa, Gerard; Conti, Giovanni; Del Giudice, Emanuela; Govoni, Marcello; Emmi, Giacomo; Fabiani, Claudia; Balistreri, Alberto; Frediani, Bruno; Rigante, Donato; Cantarini, Luca
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1308379
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