Anakinra and canakinumab for patients with R92Q-associated autoinflammatory syndrome: a multicenter observational study from the AIDA Network
| Autor: | Carla Gaggiano, Donato Rigante, José Hernández-Rodríguez, Antonio Vitale, Maria Tarsia, Alessandra Soriano, Giuseppe Lopalco, Florenzo Iannone, Masen Abdel Jaber, Roberto Giacomelli, Ewa Wiȩsik-Szewczyk, Marco Cattalini, Micol Frassi, Matteo Piga, Gaafar Ragab, Jurgen Sota, Fiammetta Zunica, Alberto Floris, Vito Sabato, Mohamed Tharwat Hegazy, Olga Araújo, Laura Pelegrín, Alessandra Fabbiani, Alessandra Renieri, Salvatore Grosso, Claudia Fabiani, Bruno Frediani, Luca Cantarini |
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| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: | |
| Zdroj: | Therapeutic Advances in Musculoskeletal Disease, Vol 13 (2021) |
| Druh dokumentu: | article |
| ISSN: | 1759-7218 1759720X |
| DOI: | 10.1177/1759720X211037178 |
| Popis: | Background: This study aims at describing the therapeutic outcome of patients carrying the R92Q variant in the TNFRSF1A gene treated with anakinra (ANA) or canakinumab (CAN) and identifying any factors predictive of complete response to IL-1 inhibition. Methods: Clinical data of patients treated with ANA or CAN for recurrent inflammatory attacks due to the presence of the R92Q variant were retrospectively collected and analysed. Results: Data about 20 treatment courses with IL-1 inhibitors (16 with ANA and 4 with CAN) from 19 patients were collected. Mean age at disease onset was 20.2 ± 14.8 years. In 5 cases (26%) the R92Q variant was found in a family member affected by recurrent fever. The therapeutic response was complete in 13(68%) and partial in 2 patients (11%); treatment failure was observed in 4 cases (21%). Median AIDAI decreased from 10 (interquartile range [IQR] = 28) to 0 (IQR = 1) at the 12-month follow-up visit ( p |
| Databáze: | Directory of Open Access Journals |
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