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Anakinra and canakinumab for patients with R92Q-associated autoinflammatory syndrome: a multicenter observational study from the AIDA Network
- Source :
- Therapeutic Advances in Musculoskeletal Disease, Vol 13 (2021)
- Publication Year :
- 2021
- Publisher :
- SAGE Publishing, 2021.
-
Abstract
- 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
- Subjects :
- Diseases of the musculoskeletal system
RC925-935
Subjects
Details
- Language :
- English
- ISSN :
- 17597218 and 1759720X
- Volume :
- 13
- Database :
- Directory of Open Access Journals
- Journal :
- Therapeutic Advances in Musculoskeletal Disease
- Publication Type :
- Academic Journal
- Accession number :
- edsdoj.54af1a1b25604d13ad924b07ce6756ae
- Document Type :
- article
- Full Text :
- https://doi.org/10.1177/1759720X211037178