@article{3120040,
    title = "Outcome of refractory to conventional and/or biologic treatment adult Still's disease following canakinumab treatment: Countrywide data in 50 patients",
    author = "Laskari, K. and Tektonidou, P.M.G. and Katsiari, P.C. and Athanassiou, P. and Dimopoulou, D. and Gerodimos, C. and Salamaliki, C. and Papagoras, P.C. and Settas, P.L. and Vassilopoulos, P.D. and Voulgari, P.P.V. and Zakalka, M. and Georgiadis, A. and Gkoni, G. and Daoussis, P.D. and Dimitroulas, P.T. and Iliou, C. and Kallitsakis, I. and Grika, E.P. and Mavragani, P.C. and Pikazis, P.D. and Raftakis, J. and Sarikoudis, T. and Kougkas, N. and Soukera, D. and Theodorou, E. and Tsatsani, P. and Tsiakou, E. and Vlachoyiannopoulos, P.P. and Vosvotekas, G. and Sfikakis, P.P.P.",
    journal = "Seminars in Arthritis and Rheumatism",
    year = "2021",
    volume = "51",
    number = "1",
    pages = "137-143",
    publisher = "W.B. Saunders",
    issn = "0049-0172",
    doi = "10.1016/j.semarthrit.2020.10.011",
    keywords = "abatacept;  anakinra;  canakinumab;  corticosteroid;  immunoglobulin;  methotrexate;  methylprednisolone;  tocilizumab;  tumor necrosis factor inhibitor;  antirheumatic agent;  biological product;  canakinumab;  monoclonal antibody, adolescent;  adult;  adult onset Still disease;  aged;  Article;  clinical article;  cohort analysis;  disease activity;  disease resistance;  drug dose intensification;  drug efficacy;  drug megadose;  drug safety;  drug withdrawal;  female;  follow up;  genital tract infection;  human;  leukopenia;  longitudinal study;  macrophage activation syndrome;  male;  monotherapy;  mouth infection;  multicenter study;  onset age;  pneumonia;  priority journal;  relapse;  remission;  respiratory tract infection;  retrospective study;  skin infection;  soft tissue infection;  treatment outcome;  treatment response;  urinary tract infection;  clinical trial;  off label drug use;  treatment outcome, Adult;  Antibodies, Monoclonal, Humanized;  Antirheumatic Agents;  Biological Products;  Humans;  Off-Label Use;  Retrospective Studies;  Still's Disease, Adult-Onset;  Treatment Outcome",
    abstract = "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. © 2020"
}