Outcome of refractory to conventional and/or biologic treatment adult Still's disease following canakinumab treatment: Countrywide data in 50 patients

Autor: Clio P. Mavragani, D. Dimopoulou, Katerina Laskari, Theodoros Dimitroulas, E. Tsiakou, I. Kallitsakis, D. Pikazis, C. Gerodimos, Paraskevi V. Voulgari, Charalampos Papagoras, Panagiotis Athanassiou, A. Georgiadis, G. Gkoni, Maria G Tektonidou, D. Soukera, Dimitrios Vassilopoulos, Nikolaos Kougkas, E. Theodorou, C. Salamaliki, T. Sarikoudis, P. G. Vlachoyiannopoulos, J. Raftakis, Eleftheria P. Grika, G. Vosvotekas, P.P. Sfikakis, Christina G. Katsiari, L. Settas, P. Tsatsani, M. Zakalka, D. Daoussis, C. Iliou
Rok vydání: 2021
Předmět:
Zdroj: Seminars in Arthritis and Rheumatism. 51:137-143
ISSN: 0049-0172
DOI: 10.1016/j.semarthrit.2020.10.011
Popis: Objective To assess the efficacy and safety of the IL-1b inhibitor canakinumab in all adults with refractory Still's disease identified from the National Organization For Medicines for off-label drug use. Methods : In a retrospective longitudinal multicenter cohort of 50 patients (median age 39 years) with active Still's disease despite treatment with corticosteroids (n = 11), conventional and synthetic (n = 34) and/or biologic disease modifying anti-rheumatic drugs (n = 30), we assessed the efficacy of canakinumab 150–300 mg administered every 4 (n = 47) or 8 weeks (n = 3) as combination therapy or monotherapy (n = 7) during a median follow-up of 27 (3–84) months. Results Α complete response was initially observed in 78% of patients within 3 months (median), irrespective of age at disease onset. A partial response was evident in 20%. One patient had resistant disease. Treatment de-escalation was attempted in 15 of 39 complete responders and a complete drug discontinuation in 21 patients for 8 months (median). Eleven patients (22%) relapsed during treatment, one during de-escalation process, and 11 after treatment discontinuation. Overall, 9 of 11 relapses were successfully treated with canakinumab treatment intensification or re-introduction. At last visit, 18% of patients were off treatment due to remission and 26% due to disease activity. Canakinumab had a significant corticosteroid sparing effect allowing weaning in 21 of 41 cases. Infections (20%, severe 4%) and leucopenia (6%) led to treatment cessation in one patient. Conclusion High rates of sustained remission were observed in this, largest so far, real-life cohort of adult patients with refractory Still's disease treated with canakinumab.
Databáze: OpenAIRE
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