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

C Gaggiano; A Maselli; Pp Sfikakis; K Laskari; G Ragab; Mt Hegazy; Ah Laymouna; G Lopalco; Ia Almaghlouth; Kn Asfina; O Alahmed; Ha Giardini Mayrink; I Parente de Brito Antonelli; M Cattalini; M Piga; J Sota; S Gentileschi; Mc Maggio; D Opris-Belinski; G Hatemi; A Insalaco; An Olivieri; A Tufan; H Karadeniz; Rc Kardaş; F La Torre; F Cardinale; A Marino; S Guerriero; P Ruscitti; M Tarsia; A Vitale; V Caggiano; S Telesca; F Iannone; V Parretti; M Frassi; E Aragona; F Ciccia; E Wiesik-Szewczyk; R Ionescu; A Şahin; N Akkoç; A Hinojosa-Azaola; S Tharwat; J Hernández-Rodríguez; G Espinosa; G Conti; E Del Giudice; M Govoni; G Emmi; C Fabiani; A Balistreri; B Frediani; Donato Rigante; L Cantarini

doi:10.1007/s11739-023-03215-w

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

C Gaggiano, A Maselli, Pp Sfikakis, K Laskari, G Ragab, Mt Hegazy, Ah Laymouna, G Lopalco, Ia Almaghlouth, Kn Asfina, O Alahmed, Ha Giardini Mayrink, I Parente de Brito Antonelli, M Cattalini, M Piga, J Sota, S Gentileschi, Mc Maggio, D Opris-Belinski, G HatemiA Insalaco, An Olivieri, A Tufan, H Karadeniz, Rc Kardaş, F La Torre, F Cardinale, A Marino, S Guerriero, P Ruscitti, M Tarsia, A Vitale, V Caggiano, S Telesca, F Iannone, V Parretti, M Frassi, E Aragona, F Ciccia, E Wiesik-Szewczyk, R Ionescu, A Şahin, N Akkoç, A Hinojosa-Azaola, S Tharwat, J Hernández-Rodríguez, G Espinosa, G Conti, E Del Giudice, M Govoni, G Emmi, C Fabiani, A Balistreri, B Frediani, Donato Rigante, L Cantarini

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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 inefcacious 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 inefcacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefcacy (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 afects response to biologic therapies.

Original language	English
Pages (from-to)	743-754
Number of pages	12
Journal	Internal and Emergency Medicine
Volume	2023
DOIs	https://doi.org/10.1007/s11739-023-03215-w
Publication status	Published - 2023

Keywords

Autoinflammation
Behçet's disease

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10.1007/s11739-023-03215-w

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Gaggiano, C., Maselli, A., Sfikakis, P., Laskari, K., Ragab, G., Hegazy, M., Laymouna, A., Lopalco, G., Almaghlouth, I., Asfina, K., Alahmed, O., Giardini Mayrink, H., Parente de Brito Antonelli, I., Cattalini, M., Piga, M., Sota, J., Gentileschi, S., Maggio, M., Opris-Belinski, D., ... Cantarini, L. (2023). Musculoskeletal manifestations in children with Behçet's syndrome: data from the AIDA Network Behçet's Syndrome Registry. Internal and Emergency Medicine, 2023, 743-754. https://doi.org/10.1007/s11739-023-03215-w

@article{f2e03c13ec374f9588347244cf82a82b,

title = "Musculoskeletal manifestations in children with Beh{\c c}et's syndrome: data from the AIDA Network Beh{\c c}et's Syndrome Registry",

abstract = "This study aims to describe musculoskeletal manifestations (MSM) in children with Beh{\c c}et{\textquoteright}s syndrome (BS), their association with other disease manifestations, response to therapy, and long-term prognosis. Data were retrieved from the AIDA Network Beh{\c c}et{\textquoteright}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 Beh{\c c}et's Syndrome Overall Damage Index was 0 (range 0–4). Colchicine was inefcacious 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 inefcacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefcacy (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 afects response to biologic therapies.",

keywords = "Autoinflammation, Beh{\c c}et's disease, Autoinflammation, Beh{\c c}et's disease",

author = "C Gaggiano and A Maselli and Pp Sfikakis and K Laskari and G Ragab and Mt Hegazy and Ah Laymouna and G Lopalco and Ia Almaghlouth and Kn Asfina and O Alahmed and {Giardini Mayrink}, Ha and {Parente de Brito Antonelli}, I and M Cattalini and M Piga and J Sota and S Gentileschi and Mc Maggio and D Opris-Belinski and G Hatemi and A Insalaco and An Olivieri and A Tufan and H Karadeniz and Rc Karda{\c s} and {La Torre}, F and F Cardinale and A Marino and S Guerriero and P Ruscitti and M Tarsia and A Vitale and V Caggiano and S Telesca and F Iannone and V Parretti and M Frassi and E Aragona and F Ciccia and E Wiesik-Szewczyk and R Ionescu and A {\c S}ahin and N Akko{\c c} and A Hinojosa-Azaola and S Tharwat and J Hern{\'a}ndez-Rodr{\'i}guez and G Espinosa and G Conti and {Del Giudice}, E and M Govoni and G Emmi and C Fabiani and A Balistreri and B Frediani and Donato Rigante and L Cantarini",

year = "2023",

doi = "10.1007/s11739-023-03215-w",

language = "English",

volume = "2023",

pages = "743--754",

journal = "Internal and Emergency Medicine",

issn = "1828-0447",

publisher = "Milano : Springer Verlag Italia Roma : CEPI",

}

Gaggiano, C, Maselli, A, Sfikakis, P, Laskari, K, Ragab, G, Hegazy, M, Laymouna, A, Lopalco, G, Almaghlouth, I, Asfina, K, Alahmed, O, Giardini Mayrink, H, Parente de Brito Antonelli, I, Cattalini, M, Piga, M, Sota, J, Gentileschi, S, Maggio, M, Opris-Belinski, D, Hatemi, G, Insalaco, A, Olivieri, A, Tufan, A, Karadeniz, H, Kardaş, R, 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, Şahin, A, Akkoç, N, Hinojosa-Azaola, A, Tharwat, S, Hernández-Rodríguez, J, Espinosa, G, Conti, G, Del Giudice, E, Govoni, M, Emmi, G, Fabiani, C, Balistreri, A, Frediani, B, Rigante, D & Cantarini, L 2023, 'Musculoskeletal manifestations in children with Behçet's syndrome: data from the AIDA Network Behçet's Syndrome Registry', Internal and Emergency Medicine, vol. 2023, pp. 743-754. https://doi.org/10.1007/s11739-023-03215-w

TY - JOUR

T1 - Musculoskeletal manifestations in children with Behçet's syndrome: data from the AIDA Network Behçet's Syndrome Registry

AU - Gaggiano, C

AU - Maselli, A

AU - Sfikakis, Pp

AU - Laskari, K

AU - Ragab, G

AU - Hegazy, Mt

AU - Laymouna, Ah

AU - Lopalco, G

AU - Almaghlouth, Ia

AU - Asfina, Kn

AU - Alahmed, O

AU - Giardini Mayrink, Ha

AU - Parente de Brito Antonelli, I

AU - Cattalini, M

AU - Piga, M

AU - Sota, J

AU - Gentileschi, S

AU - Maggio, Mc

AU - Opris-Belinski, D

AU - Hatemi, G

AU - Insalaco, A

AU - Olivieri, An

AU - Tufan, A

AU - Karadeniz, H

AU - Kardaş, Rc

AU - La Torre, F

AU - Cardinale, F

AU - Marino, A

AU - Guerriero, S

AU - Ruscitti, P

AU - Tarsia, M

AU - Vitale, A

AU - Caggiano, V

AU - Telesca, S

AU - Iannone, F

AU - Parretti, V

AU - Frassi, M

AU - Aragona, E

AU - Ciccia, F

AU - Wiesik-Szewczyk, E

AU - Ionescu, R

AU - Şahin, A

AU - Akkoç, N

AU - Hinojosa-Azaola, A

AU - Tharwat, S

AU - Hernández-Rodríguez, J

AU - Espinosa, G

AU - Conti, G

AU - Del Giudice, E

AU - Govoni, M

AU - Emmi, G

AU - Fabiani, C

AU - Balistreri, A

AU - Frediani, B

AU - Rigante, Donato

AU - Cantarini, L

PY - 2023

Y1 - 2023

N2 - 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 inefcacious 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 inefcacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefcacy (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 afects response to biologic therapies.

AB - 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 inefcacious 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 inefcacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefcacy (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 afects response to biologic therapies.

KW - Autoinflammation

KW - Behçet's disease

KW - Autoinflammation

KW - Behçet's disease

UR - http://hdl.handle.net/10807/232827

U2 - 10.1007/s11739-023-03215-w

DO - 10.1007/s11739-023-03215-w

M3 - Article

SN - 1828-0447

VL - 2023

SP - 743

EP - 754

JO - Internal and Emergency Medicine

JF - Internal and Emergency Medicine

ER -

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

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Keywords

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