Publications

Authors:

Vitale, Antonio; Caggiano, Valeria; Leone, Flavia; Hinojosa-Azaola, Andrea; Martín-Nares, Eduardo; Guaracha-Basañez, Guillermo Arturo; Torres-Ruiz, Jiram; Kawakami-Campos, Perla Ayumi; Hissaria, Pravin; Callisto, Alicia; Beecher, Mark; Dagna, Lorenzo; Campochiaro, Corrado; Tomelleri, Alessandro; Frassi, Micol; Franceschini, Franco; Crisafulli, Francesca; Hernández-Rodríguez, José; Gómez-Caverzaschi, Verónica; Araújo, Olga; Sfriso, Paolo; Bindoli, Sara; Baggio, Chiara; Sota, Jurgen; Tufan, Abdurrahman; Kucuk, Hamit; Piga, Matteo; Cauli, Alberto; D'Agostino, Maria Antonietta; De Paulis, Amato; Mormile, Ilaria; Giardini, Henrique A Mayrink; Cordeiro, Rafael Alves; Lopalco, Giuseppe; Iannone, Florenzo; Monti, Sara; Montecucco, Carlomaurizio; Ruiz-Irastorza, Guillermo; Soto-Peleteiro, Adriana; Triggianese, Paola; Gurnari, Carmelo; Viapiana, Ombretta; Bixio, Riccardo; Vitetta, Rosetta; Rovera, Guido; Conticini, Edoardo; La Torre, Francesco; Portincasa, Piero; Jaber, Nour; Ragab, Gaafar; Maher, Amina; Batu, Ezgi Deniz; Ozen, Seza; Wiesik-Szewczyk, Ewa; de-la-Torre, Alejandra; Balistreri, Alberto; Frediani, Bruno; Fabiani, Claudia; Cantarini, Luca

Title:

Efficacy and safety profile of biotechnological agents and Janus kinase inhibitors in VEXAS syndrome: data from the international AIDA Network VEXAS registry

Year:

2025

Type of item:

Articolo in Rivista

Tipologia ANVUR:

Articolo su rivista

Language:

Inglese

Referee:

No

Name of journal:

FRONTIERS IN PHARMACOLOGY

ISSN of journal:

1663-9812

N° Volume:

116

Number or Folder:

1

Page numbers:

42-43

Keyword:

JAK inhibitors; anakinra; anti-TNF; canakinumab; tocilizumab; treatment

Short description of contents:

Background VEXAS syndrome, a recently identified systemic autoinflammatory disorder, poses new diagnostic and management challenges. Based on experience with other autoinflammatory diseases, anti-interleukin (IL)-1, anti-IL-6, anti-tumor necrosis factor (TNF) biotechnological agents, and Janus kinase inhibitors (JAKis) have been widely employed in VEXAS patients. The aim of this study is to evaluate the global effectiveness and safety of biotechnological agents and JAKis using data from the real-world context.Methods Clinical, laboratory, and therapeutic data from VEXAS patients were obtained from the international AIDA Network VEXAS registry.Results In total, 69 VEXAS patients were enrolled in the study. Among them, 12 patients (13 treatment courses) received IL-1 inhibitors, 12 patients (13 treatment courses) were administered anti-IL-6 agents, 8 patients (9 treatment courses) were treated with anti-TNF agents, and 16 patients (17 treatment courses) were treated with JAKis. A complete response was observed in 3 patients (23%) treated with anti-IL-1 agents, 2 patients (15%) receiving IL-6 inhibitors, 1 patient (11%) receiving TNF inhibitors, and 4 patients (23.5%) treated with JAKis. The mean prednisone (or equivalent) dosage significantly decreased during anti-IL-1 treatment (p = 0.01), while glucocorticoids changed during anti-IL-6, anti-TNF, and JAKi treatment in a non-significant fashion. A total of 21 patients experienced adverse events, 3 of which led to death (gut perforation, Legionnaires' disease, and infectious pneumonia) while on JAKis; treatment withdrawal was required for 8 out of 21 patients.Conclusion IL-1 and IL-6 inhibitors, along with JAKis, represent promising therapeutic options for VEXAS patients, albeit careful monitoring is mandatory to control disease activity and ensure safety.

Product ID:

145544

Handle IRIS:

11562/1161351

Last Modified:

May 8, 2025

Bibliographic citation:

Vitale, Antonio; Caggiano, Valeria; Leone, Flavia; Hinojosa-Azaola, Andrea; Martín-Nares, Eduardo; Guaracha-Basañez, Guillermo Arturo; Torres-Ruiz, Jiram; Kawakami-Campos, Perla Ayumi; Hissaria, Pravin; Callisto, Alicia; Beecher, Mark; Dagna, Lorenzo; Campochiaro, Corrado; Tomelleri, Alessandro; Frassi, Micol; Franceschini, Franco; Crisafulli, Francesca; Hernández-Rodríguez, José; Gómez-Caverzaschi, Verónica; Araújo, Olga; Sfriso, Paolo; Bindoli, Sara; Baggio, Chiara; Sota, Jurgen; Tufan, Abdurrahman; Kucuk, Hamit; Piga, Matteo; Cauli, Alberto; D'Agostino, Maria Antonietta; De Paulis, Amato; Mormile, Ilaria; Giardini, Henrique A Mayrink; Cordeiro, Rafael Alves; Lopalco, Giuseppe; Iannone, Florenzo; Monti, Sara; Montecucco, Carlomaurizio; Ruiz-Irastorza, Guillermo; Soto-Peleteiro, Adriana; Triggianese, Paola; Gurnari, Carmelo; Viapiana, Ombretta; Bixio, Riccardo; Vitetta, Rosetta; Rovera, Guido; Conticini, Edoardo; La Torre, Francesco; Portincasa, Piero; Jaber, Nour; Ragab, Gaafar; Maher, Amina; Batu, Ezgi Deniz; Ozen, Seza; Wiesik-Szewczyk, Ewa; de-la-Torre, Alejandra; Balistreri, Alberto; Frediani, Bruno; Fabiani, Claudia; Cantarini, Luca, Efficacy and safety profile of biotechnological agents and Janus kinase inhibitors in VEXAS syndrome: data from the international AIDA Network VEXAS registry «FRONTIERS IN PHARMACOLOGY» , vol. 116 , n. 1 , 2025 , pp. 42-43

Consulta la scheda completa presente nel repository istituzionale della Ricerca di Ateneo