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Войти в исследования Recurrent fever and association with severe organ involvement, mortality and treatment outcomes in VEXAS syndrome: data from the AIDA Network.
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Caggiano Valeria, Sbalchiero Jessica, Frassi Micol, Crisafulli Francesca, Cavazzana Ilaria, Hinojosa-Azaola Andrea, Martín-Nares Eduardo, Guaracha-Basañez Guillermo Arturo, Torres-Ruiz Jiram, Wiesik-Szewczyk Ewa, Nowakowska-Plaza Anna, Sfriso Paolo, Bindoli Sara, Rizzo Samuele, Hernández-Rodríguez José, Gómez-Caverzaschi Verónica, Araújo Olga, Mayrink Giardini Henrique A, González-García Andrés, Lopalco Giuseppe, Viapiana Ombretta, Bixio Riccardo, Tufan Abdurrahman, Hissaria Pravin, Beecher Mark, Callisto Alicia, De Paulis Amato, Dagna Lorenzo, Campochiaro Corrado, Tomelleri Alessandro, Bugatti Serena, Milanesi Alessandra, Ruiz-Irastorza Guillermo, Soto-Peleteiro Adriana, Piga Matteo, Cauli Alberto, Conti Fabrizio, Cardamone Chiara, Triggianese Paola, Gurnari Carmelo, Vitetta Rosetta, Prete Marcella, Racanelli Vito, Talarico Rosaria, Ferro Francesco, di Cianni Federica, Cuccaro Maria Chiara, Mayo-Juanatey Adrián, Renieri Alessandra, Sorrentino Vincenzo, Ergun Mehmet Ali, Balistreri Alberto, Frediani Bruno, Bocchia Monica, Fabiani Claudia, Cantarini Luca, Vitale Antonio
Аннотация
METHODS: Data were obtained from the international AutoInflammatory Disease Alliance (AIDA) Network registry and analyzed using a Bayesian statistical approach. Posterior probabilities [P(β)] were calculated to assess the likelihood that fever was associated with clinical, laboratory, genetic, and therapeutic features.
RESULTS: In total, 87 VEXAS patients were enrolled, 65 (74.7%) of whom suffered from recurrent fever episodes. Fever episodes showed a significant association with patients' mortality [P(β): 99.41%], as well as with major inflammatory organ involvement, including cardiac [P(β): 99.99%], lung [P(β): 99.98%], and gastrointestinal [P(β): 97.5%] involvement. The occurrence of recurrent fever episodes was associated with a negligible probability of both complete response and treatment failure [P(β) <2.5%], instead favoring a partial response [P(β) >97.5%] to conventional disease modifying anti-rheumatic drugs, Janus Kinases inhibitors, and tocilizumab. For temperatures exceeding 40 °C, using anti-interleukin-1 agents was associated with a high probability of treatment failure [P(β): 99.3%].
CONCLUSIONS: febrile episodes are associated with more severe pattern of organ involvement and, accordingly, to death. Furthermore, febrile episodes could correlate with differential therapeutic responsiveness, thereby potentially serving as a valuable marker to guide the treatment strategies in VEXAS patients.